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Dermpath Meeting Melbourne August

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					Dermpath Meeting Melbourne August 2008

Geoffrey Gottlieb spoke first on topographical dermatopathology. He talked
initially of reactive processes that were site specific particularly lesions with pale
nuclei looking like keratoacanthomas that may be seen in skin tags especially
taken off the eyelid and the neck. They have a cloverleaf type of atypia. He then
looked at neoplastic processes and said that if you see features of a BCC with
some surrounding areas of keloidal collagen that the lesion is usually situated on
the ear. If the BCC is deep enough in the ear then it can form this keloidal
collagen. Also nodular BCC but with an infiltrated base that can be a lot more
extensive than it looks is usually seen on the upper back but there is no stromal
response to the BCC. Remember the shave biopsies may not pick this particular
lesion up. It is one of those lesions that you initially think is a papular BCC and
you excise it and it seems a bit infiltrated in the base and you will often find you
have inadequately excised it. Sometimes old warts can have a solar keratosis or
even an SCC arising in a small proportion of the lesion. This is usually seen on
the dorsum of the hand, the foot or the extensor surfaces of the forearm and
lower leg. Other problem sites include the anterior tibia with shave biopsies
where it may be impossible to tell between lichen planus, lichen simplex,
keratoacanthoma, SCC and pseudo epitheliomatous hyperplasia. He then went
on to talk about melanocytic lesions and the influence of site and the
interpretation of these lesions. With acral nevi there are often scattered cells in
the upper epidermis and even pigment in the keratin layer. The dermal
component is usually discontinuous. Note the presence of pigment in the
cornified layer that may present as a discreet column of both cells and pigment.
If there is a discreet column like this then it is likely to be a benign lesion, but if
the pigment is diffused in the epidermis then it is more likely to be melanoma. He
then looked at lesions occurring in the milk line defining this as including the
breast, the groin and the periumbilical areas. Vulval nevus often has confluent
nests and these nests may go down the adnexa as well. This is not a malignant
pattern it is a benign pattern. Periumbilical lesions can be similar but they often
have a funny fibrosis in the base considering the origin of the umbilicus and
involution of the (vitilome?) umbilical duct. On the thigh the lesion is usually again
benign but it may have peculiar features of a Clark or a spitz nevus particularly in
women and it can be seen on the thigh of the lateral buttock. It may look like a
spitz nevus at the edge but it is not a spitz in the centre. The nevus cells are
often small and banal so remember the spitzoid Clark designation of nevi on the
thigh in young women. On the abdomen pigmented lesions may be quite broad
and because of this even though there is not really any atypia they may be
diagnosed as a dysplastic nevus. Many nevi on the upper back have a
lentiginous or single cell spread at the junctional and may be diagnosed as
lentiginous junctional dysplastic nevi. However these lesions usually have a well
defined edge and again it is usually single melanocytic cells along the retie
ridges. He did acknowledge that the lentiginous junctional dysplastic nevus on
the back is a precursor to melanoma and I must admit that I find it difficult to
reconcile this with his description of some of these other lentiginous spreads of
melanocytic lesions on the upper back. As this lesion ages it tends to get more
nested with bridging of dysplastic nevus like nests but you still have a maturing
dermal nevil component. So you have to watch a shave biopsy of this lesion. You
need to have the dermal component to make it benign. The other type of nevus
on the back is again often quite broad with prominent disparity in the size and the
shape of the nests however in this case there is often fibrosis at the centre of the
lesion and the lesion itself may be papular and it may have dysplastic bridging at
the edges. Nonetheless this is still a benign nevus.

Scalp: The nevus on the scalp of a child

Again it is usually a broad lesion with some degree of epidermal hyperplasia.
There are often spindle nuclei and there is a random nesting pattern. In other
words the nests are not just seen at the retie tips. This is probably a type of
congenital nevus. You may occasionally get a benign nevus of the scalp where
there is as well as the usual nevus another population of melanocytes presenting
slightly differently and this is commonly seen in congenital nevi of the scalp.

Melanoma on the leg

It is usually below the knee in women and there are small cells but occasionally
they are larger with monomorphous melanocytes. Essentially there are too many
cells per unit area and when you look at this with low scanning there are multiple
patterns across a broad front. There is no stromal reaction. Note that atypia and
cells that appear to be above the epidermal junction are not good discriminators
for a melanoma diagnosis. Melanomas on the legs of women may have some
degree of satellite metastasis pattern as well. They appear to have a skip lesion
and then new melanoma. Sometimes nevi in children will cause quite marked
expansion of the epidermis but if you see this with nests or cells and if you see
this in an adult then they shouldn’t occur. So in essence if you have got a
monomorphous melanocytic presentation in an adult it is unusually cellular and
there is no stromal response to the deep component then this may show a
tendency to satellite metastasis locally and it is a melanoma. He also talked
about blue nevi of the scalp in people in the mid 50s. He has seen cases of
melanoma growing in the substance of the blue nevus and it is many years after
the lesion has obviously been there. Many blue nevi are in fact congenital
anyway. He also commented on pilar tumours of the scalp such as epidermoid
cysts recurring as SCC if bits are left and he feels that blue nevus can in fact
subsequently present as melanoma on the scalp. I find the cellular blue nevi are
perhaps more common on the scalp. I do not know if they are more likely to form
melanoma many years later than normal monomorphous blue nevi.
Phil LeBoit then gave a lecture of interface dermatitis and it was an approach
based on epidermal changes. This lecture was well covered in the handout that
came at the conference and it is probably unnecessary for me to summarise this
if you were in fact there. A few clinical points that I gained from it were that if you
have someone with lichenoid interface dermatitis over 60 then look for a lichenoid
drug eruption but if under 60 it is likely to be lichen planus and note also that
lichenoid drug eruptions can take a long while to go away even though the drug
is withdrawn. In a lichenoid drug eruption you ……………………… parakeratosis
with associated eosinophils and plasma cells as part of the cellular infiltrate not
just lymphocytic. He also commented that it is difficult to separate hypertrophic
lichen planus, verrucous lupus and SCC. Mycosis fungoides never presents with
an anogenital lesion initially but may subsequently. He talked also of secondary
syphilis as a mimic of lichen planus and found that immunoperoxidase stains
were better than the Warthin Starry stain for demonstrating the treponemal
organism. Another point was that if an epidermis with a lichenoid eruption is very
thin then it is usually lupus or dermatomyositis rather than lichen planus.
Interface reactions may also be seen in lichen planus like keratosis, poikiloderma
vasculare atrophicans, regression of a melanoma, regression of a BCC and
sometimes of porokeratosis of Mibelli. Occasionally you can even get an MF like
keratosis reaction. He also highlighted the problem of misdiagnosing lupus
erythematosus if a biopsy is taken from the central region of an area of
disseminated superficial actinic porokeratosis.

Phil also then went on to present several cases. The first was a neutrophilic
urticaria and early lesions of Sweet’s syndrome may look like neutrophilic
urticaria. You also have to consider early dermatitis herpetiformis, bite reactions,
cellulitis, Still’s disease and erythema annulare centrifugum in your histological
differential diagnosis and very rarely the urticarial like lesion of erythropyetic
protoporphyria. Note that the pre versicular stage of dermatitis or piriformis often
has neutrophils as the infiltrate in the papillary dermis and sometimes you can
even have neutrophilic collections. Treatment is usually Dapsone and perhaps
colchicine for neutrophilic urticaria. Case 2 was the psoriasiform eruption of a
zinc deficiency of acrodermatitis enteropathica. If in an adult it is usually seen in a
patient who has Celiac disease. Also necrotic migratory erythema may look very
similar. The classic histological feature is the paler of the top layer of cells in the
epidermis. Necrolytic acral erythema is another condition that is seen particularly
in the Middle East and is associated with hepatitis C and sometimes with zinc
deficiency. Case 3 was an atrophic lupus erythematosus. Note the thin
epidermis with also an infiltrate around the appendages and quite a marked
lichenoid reaction. He talks about Lamisil and subacute cutaneous LE. There
were also a variety of other drugs that can cause a similar picture. Drugs that
cause subacute cutaneous LE usually have both a positive ANA and a positive
SSA and SSB in their immunofluorescence patterns. Particular drugs that can
cause subacute cutaneous lupus include the calcium channel blockers,
Terbinafine, Ranitidine, Tamoxifen and surprisingly Hydroxychloroquine. Usually
subacute LE has a very dense lichenoid infiltrate as against the lichenoid infiltrate
that is seen in discoid lupus. Case 4 was a case of cutaneous TB. Note that it is
very (pauci?) bacillary and generally the cases that we will tend to see are
hypersensitivity reactions or tuberculids. Case 5 was an acute generalised
eczematous pustulosis mimicking toxic epidermal necrolysis. We tend to think of
AGEP as being a pustular disorder. In other words primarily presenting as
pustules but sometimes it will present as a TEN like syndrome. A majority of
cases are obviously drug related and the other important thing about this
condition is that it occurs very shortly after a drug is introduced. In other words it
would not seem to be almost an allergic reaction. The mechanisms are a bit
obscure. IV immunoglobulin may be used on treatment. Case 6 was a CD32 cell
lymphoma with positive cells only seen in the base of the specimen and the
lesion was very neutrophil rich. Case 7 was a case of lichen striatus that he had
seen just before coming out here to Australia.

Peter Ebeling then spoke on Vitamin D. If your Vitamin D levels go down
parathyroid levels go up and it would appear that Vitamin D toxicity is not
possible due to sun exposure. One study suggested that one MED body
exposure is equivalent to about 15,000 international units of Vitamin D. The
optimal level of Vitamin D that we should have is about 75mili mills per litre.
Insufficiency is where your levels are between 50 and 75, mild deficiency is
between 25 and 50, moderate is between 12.5 and 25 and severe is less than
12.5mili mills per litre. With low levels like this you may get approximal myopathy
and quite marked aches and pains. Other factors, which may affect Vitamin D
levels include the season, the latitude and probably most of all behavioural
factors. Note that certain medications taken for epilepsy will lower Vitamin D
levels and they are also particularly low in patients with celiac disease.

From the recent lecture from Gary Halliday at the epiderm meeting on
immunosuppression from less than 1 MED of ultraviolet light it would appear that
the two things that we really need to take if we want to protect ourselves are
Nicotinamide to prevent ultraviolet induced immunosuppression to the skin and
Vitamin D to help our bones and also perhaps to prevent the development of
other cancers. We should take somewhere between 700 and 800 international
units of Vitamin D per day but the tablets that are available here in Australia are
usually a 1,000 international units per day. In New Zealand you can get tablets
that are 50,000 international units of Vitamin D and it is suggested that you need
one of these a month. The other concept that was raised by Peter Folley in his
talk was that sunlight induced aging is the speeding up of the natural process
with shortening of the telomeres and chromosomes and thought that sun aged
skin shows this but normal aged skin such as the buttock skin does not. It was
also interesting that apparently sub erythemal doses are best at inducing Vitamin
D levels rather than having more than 1 MED because at higher levels the
sunburn induced by these seems to break down the pre Vitamin D3 so sub
erythemal     doses     are     best    for    both     your    production      of
Vitamin D and to minimise your immunosuppression. Vitamin D modification in
the skin from pre Vitamin D to Vitamin D3 is best at the lower UVB levels rather
than the 310 nanometer level that we use in the treatment of psoriasis or atopic
eczema. Make sure you are not getting any extra Vitamin A in with Vitamin D
supplements because it tends to counteract the affect to the Vitamin D. It is also
suggested that if you are deficient in Vitamin D you take a loading dose of
perhaps 5,000 units before taking your 1,000 a day.

Dr Gottlieb then spoke about interesting cases from the Academy and by
Academy he meant the Acumen Academy of Dermatopathology. First case was
of a desmoplastic melanoma. You have to look for a neurotropic component of
this melanoma. Case 2 was a case of herpes zoster with destructive
inflammation of the hair follicles and removal of the hair follicles. Case 3 was a
sebaceous adenoma and he then went on to talk about that and sebaceous
carcinoma, the Muir-Torre syndrome and the increase risk of colon and thyroid
cancer. You may find a keratoacanthoma like lesion with sebaceous carcinoma
at the base. Sebaceous adenoma may not be associated with Muir-Torre
syndrome. Case 4 was a combined congenital nevus and one had to compare it
with melanoma. Case 5 was chemotherapy induced drug reactions, which may
give unexplained diffuse atypia in the epidermis of a skin biopsy. It is due to the
chemotherapy that they take. He also said that ……………. bodies were a clue to
carcinoma and illustrated it with a case in which they were seen when the patient
in fact had breast cancer. Case 7 were linear erythematous bands that are
sometimes seen in patients with rheumatoid arthritis. They look like Monge’s
disease but they are not a thrombosed vein. It presents with a palisaded
interstitial dermatitis often associated with arthritis and it also may be an
indication of other collagen diseases. Case 8 was thyroid carcinoma. Case 9 was
a young person with neutrophils and sebaceous glands and he called it
neutrophilic sebaceous adenitis. He thought it was due to a fat soluble substance
that somehow is absorbed into the sebaceous glands and destroys them. Case
10 was a fibrosing septal panniculitis. It was very like erythema nodosum
histologically but in point of fact it was nephrogenic fibrosing dermopathy. Case
11 was a case of lymphedema secondary to reflex sympathetic dystrophy and a
complex regional pain syndrome. The patient ended up having the hand, which
became functionally useless amputated. Phil LeBoit then spoke of some
emerging conditions in dermatopathology. He spoke about (Fox Fordie ?)
disease with forme histocytes being present. He talked of the tick tack toe sign
with infant (dibular?) hyperplasia and telangiectasia seen also in keratosis
lichenoides chronica. He spoke of some cases of breast metastases being a
hybrid of pyogenic granuloma and metastatic breast tissue. He also showed a
case of the amyloid occurring with some cutaneous bone formation. If you see
this phenomenon of cutaneous bone formation make sure you stain locally for
amyloid because it may be a presentation of systemic amyloidosis. He then
talked of some of the pseudo lymphomas especially when some present as a
solitary lesion of the head and neck. There may be a type of pleomorphic T-cell
lymphoma but there are in fact some cases like this that have no evidence of
…………………….. If you have multiple lesions then it is more likely to be a
significant problem. He also talked about keratoacanthoma like changes
overlying some ductal tumours and Steven Gossards concept of infundibulo
cystic hyperplasia.

The Brennan Prize presentations were on bacillary angiomatosis and on the
different types of melanoma and particularly a patient who presents with diffuse
melanosis cutis presenting as diffuse hyperpigmentation. Some usual variants of
melanoma were also described including oral melanoma seen in less than 4% of
malignant lesions of the mouth. The third presentation was on crypticoccal
meningitis in a patient who was on Infliximab and previously on another
monoclonal for pustular psoriasis. The Geoffrey Hunter oration was given by Phil
LeBoit on dysplastic nevi thesis, antithesis and synthesis. This was a very
scholarly lecture on the evolution of the dysplastic nevus entity in term that
pointed out some of the fallacies of the concept but in the end go down to the
genetic level and showed that in some of these lesions there are in fact genetic
abnormalities which are also seen in early melanoma. Mind you as he pointed
out there is still a lot of work to be done in analysing the instance of these
apparent genetic anomalies in what we would regard morphologically as benign
simple nevi. The histological features of dysplastic nevi include fused retie ridges
with fibroplasia beneath the retie ridges. There is evidence of a nevus seen in up
to 20% of melanomas. If you have 10 or more dysplastic nevi then there is a 12
times increased risk of developing melanoma. If you have one dysplastic nevus
or atypical or Clark nevus call it what you will there is two times risk of melanoma.
If you have increased numbers of just small and large nevi which are neither
clinically atypical or dermatoscopically dysplastic then there is a four times
increased risk of melanoma. He also spoke a bit about specialised sites of the
body and the morphology and histology of lesions that are seen at these sites.
There are the diverse eclipse nevi, which are seen particularly on the scalps of
children. They have a dark periphery to the lesion and a clearer centre. You also
can have what have been described as spitzoid Clarks nevi on the outer aspect
of the thigh. Phil also noted to go back to the genetic features of nevi that B RAF
mutations are present in 60% of common nevi. The major factor that appears to
be related to melanoma is just density of nevi or the total number of nevus cells
that an individual has. So in the area of dysplastic or Clark nevi the clinical
………………type correlates with melanoma risk. In these lesions a degree of
what people regard as atypia really means nothing. If an individual is
immunosuppressed then this appears to lead to many of these dysplastic nevi
erupting and tumour ………………..mutations are also increased in dysplastic or
Clark nevi. To go back to immunosuppression with the increase in dysplastic or
Clark nevi this is usually a dead end phenomenon. Only some of these lesions
ever develop into melanoma so that some individuals with multiple lesions may
lack something that keeps their normal nevi under control and they grow and
develop into these dysplastic or Clark nevi. So it is the cell number and surface
density of these cells that is important and it is acknowledged that they can be
mildly genetically unstable. Another way of looking at a dysplastic or Clark nevi is
that the nevi in a prolonged state of adolescence.

An important take home area of Phil’s lecture on when to re-excise dysplastic
nevi. He felt that they should be re-excised if they are growing firstly on sun
damaged skin where single melanocytes predominate in the histology and he
said particularly to beware of diagnosis of dysplastic nevus on the face that has
not been fully excised. He felt that many of these were probably melanoma in situ
especially if the predominant histology was junctional. The second reason to re-
excise dysplastic nevi is there is marked confluence of the nests. Third reason
was if there was pagetoid scatter but no evidence of the nevus having been
irritated. Fourthly if there were large melanocytes in the lesion and the lesion was
not in a special site or it was not spitzoid. Fifthly if a partial biopsy had been taken
of a lesion and it was in a special site and sixthly if there was regression at the
edges of the lesion particularly or even elsewhere in the lesion. In summary it
was felt that if a dysplastic or Clarks nevus had been biopsied or taken with
narrow margins that irrespective of the degree of atypia and in point in fact he did
not comment on the level of atypia in these lesions but if there were a lot of single
cells with lots of associated elastosis particularly on the face then the lesion
should be excised. It was interesting that he did not feel that the degree of atypia
in these lesions should be described. Apparently even amongst Pathologists this
is a very subjective decision.

Day 2 of the Conference began with discussion on cutaneous lymphoma by Phil
LeBoit. He initially began with a philosophical discussion on pseudo lymphoma or
cutaneous lymphoid hyperplasia where he felt that the histopathology was often
just a reiteration of the lymph node and its structure with a formation of lymphoid
follicles in the skin. The neoplastic counterpart of this was cutaneous follicular
cell lymphoma. The lymphoid follicles in the latter have thinner mantle cell areas
around them and less mitosis in the centre of the follicle than say a normal
lymphoid follicle and these cells would stain for BCL 2, KI 67 and CE 10. Then
there was the marginal zone lymphoma. One often may see anetoderma within
these lesions clinically when they present. A key feature is the presence of
lympho plasmacytoid lymphocytes looking like plasma cells along the adnexal
structures. He then talked about the diffuse large B cell lymphoma. It is usually
seen on the lower leg in the elderly. It is BCL2 positive and sometimes these
lesions may be misdiagnosed as sarcomas because of the cell structure and
shape. The next common lymphoma that clinicians such as Dermatologists will
see will be the angiotrophic lymphomas. These generally present as bruise like
lesions where the blood vessel lumens are obscured by cells in the vessels and
these cells are usually B cells. A simulant is intravascular histiocytosis, which is
seen in some patients with rheumatoid arthritis. He then went from the B cell
lymphomas onto the T cell lymphomas and commented that the patch stage can
last for years especially if less than 10% of the body surface area is involved.
Some time was spent discussing digitate dermatosis or whether this in fact
should be described as patch stage mycosis fungoides. It was felt that it was best
not to include it as such as a lymphoma because of the concern that this arises in
a patients mind. As T cell lymphomas develop and as they develop from a patch
to a plaque and a nodular stage then patients with these later stages actually die
not from the lymphoma but from immune collapse associated with lymphoma. In
other words they have no functional T cell even though they have lots of T cells.
It was felt that various clinical …………tapes of mycosis fungoides including
verrucous, atrophic and pigmented purpuric types are in fact due to the actions of
cytokines in the epidermis.

When biopsying an early case of suspected patch type T cell lymphoma it is
important often to get a series of biopsies. Either a six pack in the form of punch
biopsies or sometimes even a shave biopsy to be able to look at a large surface
area of the epidermis where most of the action is. So I think that it is important to
reiterate again that a shave biopsy might be better here in early patch stage
mycosis fungoides. The histological feature that is important in diagnosing this
early patch stage mycosis fungoides is that the lymphocytes in the epidermis are
larger and have darker nuclei than those that remain in the dermis. The
prognosis of patch stage mycosis fungoides does not depend on the
immunohisto chemistry or on gene rearrangement studies but in fact it depends
on the total surface area involved. So when one has less than 10% surface area
involved in patch stage then the long term prognosis is excellent and people will
regularly go 30 years or more without it developing any further. He did spend a
little bit talking of the pigmented purpuric presentation of some cases of mycosis
fungoides particularly the lichenoid purpuric presentation like lichen aureus and
also the true pigmented purpuric eruptions particularly this lichenoid type may
histologically simulate patch stage mycosis fungoides but the things to look for
where the siderophages in the base of the specimen. The critical point in the
neoplastic progression in mycosis fungoides is when plaques form from the
patches. There is an increase in atypia of the lymphocytes invading the
epidermis. These lymphocytes can grow on the connective tissue in the dermis
itself and has a deleterious affect on immune response. There is often the
presence of eosinophils in the cellular infiltrate and you have variable sized
lymphocytes and there is a switch from TH1 to TH2 type immune responses.
Another interesting thing that he pointed out was that most cases of mycosis
fungoides presenting as follicular mucinosis have little early epidermal
involvement. Some cases even can be follicular trophic without mucin formation.
Histologically presentations with granulomatous mycosis fungoides did not have
distinctive clinical lesions and they appear to have the same prognosis.
Granulomatous slack skin diseases are a very distinctive clinical variant of
mycosis fungoides and may have a curious relationship to Hodgkin’s disease as
well than increased incidence of the latter. Patients present with hanging bags of
skin particularly in the axillae and the groin and histologically within the dermis
you will see giant cells that basically are gobbling up elastin and hence giving rise
to the hanging bags of skin. These patients react badly to surgery and if you try
and excise these areas they will just reform. He then spoke of the Worringer
Kollop variant of Pagetoid reticulosis particularly seen in younger people on the
lower limbs. These lesions show marked epiderma trophism and in some way
there seems to be the phenomenon of …………….. up of these large cells in the
epidermis rather than the dermis.

Sezary’s syndrome presents with generalised erythroderma with hyperkeratosis
of the palms and soles and lymphadenopathy. Skin biopsies themselves are not
diagnostic of Sezary’s syndrome and really you have to find Sezary cells in the
peripheral blood. Hall also pointed out that if you look at most people over the
age of 80 5% to 10% of them may in fact have a clonal T cell population in the
blood. One needs to find 1,000 Sezary cells or more per mill of blood to make a
confident diagnosis of Sezary’s syndrome. He also spoke of the HTLD1 virus T
cell lymphoma. It is particularly seen in Southern Japan and other parts of Asia.
The characteristic feature is the cloverleaf lymphocyte in the peripheral blood. He
then talked of CD30+ lymphomas and the one that we are more likely to see in
dermatology is lymphomatoid papulosis. There is often a CD4+ and CD30+ T cell
proliferation in the lymphomatoid papulosis but often the diagnosis can only be
made with certainty when the patients are followed up over a period of time and
the lesions have a finite lifespan and involute. Examples were also shown of
patients with lymphomatoid papulosis occurring on a background of lesions of
more typical patch stage mycosis fungoides. The CD30 stain lights up the large
cells in this condition and he reiterated again the importance of clinical
observation of a patient’s progress in making a confident diagnosis of
lymphomatoid papulosis.

You can get cutaneous Hodgkin’s disease but it is usually skin spread from
systemic disease and it is not common. They also talked about some of the
subcutaneous lymphomas particularly subcutaneous panniculitic like T cell
lymphoma. He said this was a particularly dangerous area because it is usually
initially misdiagnosed as an inflammatory panniculitis particularly lupus profundus
and these patients may have in fact a very high ANA. They often though develop
an erythrophagacytic syndrome and the patient becomes panocytopenic.
Ringing of adiposities may be found in this lymphoma by the involved T cells.
The CD56 positive natural killer cell lymphoma is usually of the nasal type that
may also be seen with disease on the extremities. Epstein Barr virus may also
be seen commonly in these lymphomas.

Dr Deborah Norris then spoke of cutaneous lymphomas highlighting pitfalls in
immuno histochemistry. This was an excellent lecture for the dermatopathologist
present especially those who do a lot of immunoperoxidase staining. She
reiterated that it was important to do targeted staining rather than a whole battery
of these stains and then try to interpret things. This was a difficult lecture for a
Dermatologist to fully appreciate. The main things that I picked up were that
CD30 is a marker of activation of lymphocytes and it can commonly be seen in
conditions such as insect bite reactions, drug reactions, herpes simplex and
zoster infections and other infections particularly scabies, orf and other viral
disorders. Methotrexate induced lymphoproliferative disease often have the EB
virus found as well and 60% of these sort of cases regress when Methotrexate is
withdrawn. She also talked about the immuno queevey website where one can
look up the likely diagnosis for certain combinations of immuno staining results.
Most of Dr Norris’s lecture is in fact contained in an excellent handout, which she
gave in the Conference handbook.

Dr Grant Jenkins an Infectious Diseases Physician than gave an excellent
presentation on microbacterium ulcerans also known as the Bairnsdale ulcer.
Microbacterium ulcerans is the commonest microbacterial disease that we will
see after TB and leprosy. You can get a pre ulcerative nodule with a fair degree
of subcutaneous involvement in this condition but more commonly you see
usually papules, which subsequently progress to ulceration. In the initial histology
of these lesions granulomas aren’t prominent. This is because the
microbacterium ulcerans makes a toxin, which is both cytotoxic and
immunosuppressive and in part is due to the extensive subcutaneous
involvement that you will get with this particular organism. Dr Jenkins also
showed some excellent images of the acute oedematous variant or cellulitic like
variant of this condition which extensively involves the subcutaneous tissue
before much ever happens in the overlying epidermis. Culture of microbacterium
ulcerans often take six to eight weeks and is relatively insensitive. He explained
how important the PCR test is in making a diagnosis and it can be done even on
a swab of an ulcerated lesion rather than on tissue. The PCR is very sensitive
and very specific. The techniques are able to extrapolate on 100 th of an organism
and build it up because of the insertion sequences that are seen in this particular
organism. It will even work on formalised tissue as well. Note that the treatment
of choice in microbacterium ulcerans is in fact excision of the involved area and
either flaps or grafts so it is important to make this diagnosis early. You can in
fact aspirate pre ulcerative lesions if you are looking for tissue in which to do the
PCR. The cost of the PCR is only about $25.00.
The Clinical gems section following with Dr John Snow showing a case of
secondary syphilis his point being that the immuno stains for spirochetes were in
fact much more sensitive than the Warthin Starry stain where it was difficult to
pick up the thread like spirochetes against a black background of other staining
with this stain. A case of chronic cutaneous microbacterium marinum infection
was then presented by Dr Ellen Ma. This patient probably acquired this disease
when he was a boy in Fiji about 50 years ago and he presented now with multiple
pigmented nodules on his left limb. He was treated with oral Clarithromycin and
Vitamin D supplementation and the case represented the longest cutaneous
microbacterium marinum infection known.

Dr Delwyn Dyall-Smith then presented a case of palisading necrobiotic
granulomas in the HIV+ patient. Clinically these lesions look like rheumatoid
nodules. Histologically they had a granuloma annulare like presentation in a
patient who was not known to be HIV+. She was subsequently found to be HIV+
with a very high fyrol and low CD4 count but the source of her infection was not
known. The skin lesions were responding to HAART therapy.

Dr Myles Prince spoke on the treatment of cutaneous T cell lymphoma. This was
a very detailed lecture looking at a series of investigational drugs that have been
used in the Melbourne Lymphoma Group to treat various types of lymphoma. The
notes that I made were to watch the CD30+ large cell transformation of mycosis
fungoides. If it is localised in a single nodule that develops against a background
of patch stage MF then basically just treat conservatively and either excise the
nodule or treat with radiotherapy. If it is generalised then aggressive
chemotherapy is in fact required. He pointed that there was a distinction between
erythrodermic mycosis fungoides and Sezary’s syndrome and that erythrodermic
mycosis fungoides is less of a problem than actual Sezary’s disease. The risk of
CD30+ large cell transformation for early stage mycosis fungoides is only about
0.4%. He also pointed to the relative immune suppression in some people with
lymphomas and the change in …………….type from TH1 to TH2 responses. He
also advocated Methotrexate for lymphomatoid papulosis but the doses that are
required are not the sort of homeopathic 5mg per week doses but 20mg to 30mg
on a weekly basis. He spoke also of the use of Bexarotene or Targetin this is a
Retinoid and it is useful in some of the T cell lymphomas. The concept also of
having diphtheria toxin linked to an IL2 receptor protein that will attack the IL2
receptor on T cells was also reiterated as a means of targeted destruction of T
cells and the last group of drugs that he talked about were the Histone and
(Diasetilator?) inhibitors which can both switch on and off genes by putting the
DNA material back on the Histones and switching it off by making it difficult to be
read and switching it on by unravelling it again. These drugs may even have a
place in early patch stage mycosis fungoides and we will probably hear more
about them in the hear future because they are pretty safe drugs and they have a
very good side affect profile. Some thalidomide like drugs are also being trialled
in T cell lymphomas.

Phil LeBoit then spoke on genomic insights into the nature and diagnosis of
melanoma. He indicated that many new investigative techniques of a genetic
basis were being used to look at the lymphomas and the one he spoke mainly on
was comparative genome hybridisation where there may be a growth increase or
even loss in parts of chromosomes. A technique of fluorescent in situ
hybridisation is then used to see the number of copy number mutations or
changes that have occurred and where they have occurred. There may be an
amplification in the number of ……………………….of these chromosomes. In
melanoma there are both losses and gains of DNA over various chromosomes
including chromosome 6 and 9. He commented that proliferating nodules in
congenital nevi do not necessary develop as a melanoma even though
histologically they may look like melanomas and often with a five year follow up
of these sort of cases no melanomatous metastases developed if there were a
few mitoses in the lesions. There are usually gains or losses of whole
chromosomes in these lesions rather than just localised amplifications of genes.
They looked at comparative genomic hybridisation in Spitz nevi. In benign nevi
you really find nothing when you look for these but in Spitz nevi there are often
gains on the 11P Chromosome and losses of the 9P chromosome. 20% of Spitz
nevi may show 11P changes but most of these are in fact seen as Spitz nevi that
are not difficult to diagnose anyway. You ask the question of: Do melanomas
arise in Spitz nevi? You generally do not see pagetoid spread in the epidermis in
a pre pubertal child with melanoma. B raf is absent in Spitz nevi.

Geoff Gottlieb then gave an excellent hour long lecture on an algorithmic
approach to the diagnosis of melanocytic lesions or melanoma. There was a
progression from benign to malignant and the four things that they looked for
were the size of the lesion, symmetry of the lesion, circumscription and that there
was no maturation. Generally if a lesion is well circumscribed it will end with a
complete nest rather than tailing off with single cells into the surrounding
epidermis. A major fact he emphasised was that in an adult sheets of
melanocytes tends to suggest melanoma. There are basically too many cells.
Another point was that a thick lesion with some degree of normal elastosis below
it suggests that the lesion has been there for some time, i.e. there has been
filtering of ultraviolet light. If there is downward displacement of upper layer type
solar elastosis into a lower level then this suggests that it is a new lesion that has
pushed the previously damaged elastotic tissue down lower. Small lesions less
than 4mm are almost invariably benign. So are large lesions that are
symmetrical. Large lesions that are asymmetrical and well circumscribed again
are often benign but those that are poorly circumscribed which still show some
degree of maturation may be either a melanoma or a nevus but those with no
maturation at the base they are almost always melanoma. Of course there are
exceptions to all of these and he spent some time looking at these exceptions.
The criteria for exceptions included melanocytes above the derma epidermal
junction, variations in the size and shapes and confluence of nests in atypia.
When they were seen even with small lesions he suggested that the small lesion
might be a melanoma. Certain anatomical sites made it more likely that a
particular lesion that may be large and asymmetrical may in fact be benign.
Mitoses don’t always mean that a lesion is malignant but he number of mitoses is
of some importance. The greater the number the more you take notice that it may
be a malignant lesion. As he had explained in his very first lecture of the
Conference the sites and dermoscopy of a lesion may cause the Pathologist to
reinterpret. If you have a Spitz nevus it negates other worrying features so that
the type of nevus can be an important criteria for exceptions to the algorithm. A
combined congenital nevus can be another exception where a papule that is
biopsied is included with a different cell population. Generally in these
circumstances the other cell population is interacting with the underlying cell
population and not displacing it. If one set of cells displaces another set of cells
then it is more likely to be a melanoma than a combined congenital nevus. He
then talked about a residual or recurrent nevus. In this often the cells are
confined to the scar itself because the scar when its reformed when something
has been partially shaved is in fact repopulated by nevus cells that come from the
base of the shaved lesion. This was a very good lecture in which he showed
several examples to illustrate these criteria for exceptions to the underlying
simple algorithmic features that he described.

Peter Sawyer then gave a talk on the case for a combined diagnostic approach
using both histopathology and dermatoscopic evaluation of lesions. Some
technical points made in his lecture were that the method of cutting acral lesions
should be changed so that one cuts along the line of the dermato glyphics
because often the more typical cut at right angles to this will give the impression
of melanocytic cells in the epidermis and tend to over call these as melanomas.
He talked a bit about tape stripping of lesions that have quite marked central
hyperpigmentation because then you can remove the pigmented parakeratotic
cells that are in the stratum corneum and allow you to see the network underlying
the lesion and the fact that it is generally benign. He talked a bit about confocal
microscopy and the fact that it is like a horizontal section of the skin. It does not
though penetrate terribly deep into the skin only to about 200micrometers and so
it is mainly used for superficial epidermal lesions particularly a lentigo maligna.
Generally you find that cases, which are equivocal dermatoscopically are also
equivocal pathologically so the dermatoscopic picture is a visual representation
of what the Pathologist will see. He ended the lecture with a discussion of a
paper that is due to published which looked at the degree of change of a
Pathologist’s diagnosis related to the amount of additional information that he
was presented with including such factors as the age of the patient, the site of the
lesion, the dermatoscopic image, the clinical image and the finding was that the
more information that was in fact given to some histopathologists especially
those who also had a background in dermoscopy then the more accurate the
diagnosis became, the degree of change though varied from Pathologist to
Pathologist.

Then followed a melanocytic lesion responder session chaired by Martin Haskett
with cases provided by John Kelly from the Melbourne Melanoma Unit. This was
an excellent teaching exercise in which one had to respond fairly quickly with
ones degree of concern for a clinical image, the dermatoscopic image and
subsequently the histological image of a lesion and state whether one thought it
was benign or malignant. The dermatoscopic features often caused
Dermatologists to either consolidate or change their opinion much more than
Pathologists whereas obviously the histopathological image was a major factor in
influencing the final diagnosis of Pathologists. Some dermatoscopic images were
shown with an inverse network, which was quoted as being about 22% specific
for a diagnosis of melanoma but 95% sensitive in the diagnosis of melanoma. A
take home message was given at the end of each of the 12 cases that was
presented and some of them were that any change in a body map image of a
lesion deserves histological assessment of that lesion. Two clinical images that
looked remarkably alike the Reid nevus were presented, one was in fact a Reid
spindle cell nevus but the other was a melanoma although there was some
discussion from the audience on this. In general in melanoma the radial streaking
that you see at the edge will extend back into the body of the lesion rather than
just being at the periphery, which tends to be a feature more seen in the variant
of a Spitz nevus. The ridge pattern of an acral lesion was particularly obvious in
the dermatoscopic image to Dermatologists in allowing them to say that this acral
lesion was a melanoma. A particularly good case of desmoplastic melanoma was
presented where the only dermatoscopic features were some dotted vessels and
smudged pigmentation. The lesion had been biopsied before and reported then
as a sclerotic nevus. The take home message here was that if you have any new
lesion that you think in fact is a dermatofibroma clinically then in the absence of a
history of it being there for quite some time you should biopsy and it needs to be
a good punch biopsy or an excisional biopsy. Desmoplastic melanomas are still
one of those melanomas that we find very late and its evolution and this leads to
these lesions being a lot thicker then they should be before they are picked up.
Not a particularly nice case of nodular melanoma that initially looked like a
hemangioma was presented and was also a good case of superficial spreading
melanoma in the conchal bowl.

The Steven Cossard lecture was presented by Geoff Gottlieb and it was on the
folk law of melanocytic neoplasia. He started off by commenting on excision
margins for pigmented lesions and commented that these were really just
arbitrary numbers that were initially put forward and that there is no evidence
base for them but he spent most of the talk doing an analysis of the theory of
sentinel lymph node biopsy and a particularly good forensic analysis of the paper
published in 2006 in the New England Journal of Medicine by Morton where
subset analysis of the data was done to try and prove a survival benefit for this
procedure. His main criticism was that only half the data was used for a
prognosis analysis of this subset and that various errors could have been present
in this data including wrong diagnosis which would have been balanced out if the
other subset of the data had been included but not when selectively chosen like
this. The purported 5 year difference in survival rate when this half data subset
was looked at was a difference of 72% versus 90%. This was not a significant
enough difference even allowing the analysis for the data to go unchallenged to
make it worthwhile to carry out the procedure. He also raised the issue of
whether these lymph nodes in fact have a preventative function in stopping
metastasis elsewhere in the body and used the data to point out that there does
in fact appear to be a better long term result in those patients who in fact did not
undergo elective lymph node dissection and that this benefit could be as much as
20% relative to the other group who did. The fact that melanocytes are found in
the lymph nodes does not mean metastatic disease. You can often in fact see
cell nests in nodes. This is particularly seen in congenital nevi and also has been
seen in blue nevi on the buttocks. This report does not really give an adequate
description as to how good his forensic examination of this paper was. It is
probably the best exposition I have seen on the futility of sentinel lymph node
biopsy in melanoma. Whether the concept is valid in other tumours such as
Merkel cell and breast cancer remains to be seen.

Dr Phil LeBoit then spoke again on pitfalls in the diagnosis of melanoma with
emphasis on lentigo maligna and nevoid melanoma. He began by saying that the
era of casualness in the approach to lentigo maligna should come to an end. His
major list of pitfalls were:

1.        Not recognising that a lesion is fundamentally melanocytic.
2.        An over diagnosis of pathology due to the increased number or size of
          melanocytes in chronically sun damaged skin. The term actinic
          melanocytosis had been used to describe this phenomenon but he was
          not a great fan of it. He also felt that there was an over diagnosis of
          melanoma in situ if special stains immunoperoxidase stains were in
          fact done. In some of these cases where you are not sure where the
          lesion ends and the background larger melanocytes are seen that you
          should do punch biopsies at either side of the lesion and see if they are
          the same. If they are then that really is just the normal skin for that
          patient.
3.        His third pitfall was enlargement of melanocytes around a lesion that
          had previously been biopsied. These large melanocytes are seen often
          at the edge of the biopsied area and this is a false positive.
4.       His fourth pitfall was recurrence of melanoma and this clinical point
         was that the margins of excision need to be wider if the pigmentation is
         indistinct at the edge of a lesion. And margins also need to be wider if
         inflammation present of a lesion. Acral melanoma can have cells two to
         three retie ridges beyond the obvious margins even microscopically
         when these lesions are then checked with in situ hybridisation study for
         increased copy numbers of chromosome 9 so this is why acral
         melanomas should certainly be excised with reasonable margins to
         pick up these subclinical changed cells and even sub microscopically
         changed cells.
5.       His fifth pitfall was a failure to recognise when too much is too much.
6.       His sixth point was that mitoses do not a melanoma make.
7.       His seventh pitfall was the internet and the ease with which images can
         be sent from one person to another but unless you get a chance to
         look at the original block and the original slides it is often very difficult
         to make an accurate diagnosis.
8.       His eighth pitfall was that inflammation can alter the appearance of a
         benign nevus. The example he quoted was the Myerson nevus where
         there is a dermatitic reaction around an otherwise benign nevus. The
         inflammation may alter epidermal keratinocyte genesis and may give
         the appearance of melanoma cells in the epidermis and hence you can
         overcall the nevus as malignant. In hallo nevi or a ………..hallo nevi
         large outlying nests that are not being attacked by lymphocytes are a
         worrying feature and might make you consider that this could be a
         melanoma that is having a hallo affect. Other inflammatory simulants of
         melanoma include the associations of lichen sclerosis and vulval nevi.
9.       This is where you have a melanoma with small cells at the base of the
         lesion and little epidermal scatter.
10.      Do not get too fancy in making a diagnosis.
David Ellis gave an excellent talk on clues in the diagnosis of cutaneous
lymphoma. Anyone who includes an extract from the Life of Brian in his talk gets
full marks from me. He basically showed the naughty boy scene where Eric Idle
plays the messiahs mother and screams to the waiting car that he is not the
messiah he is just a very naughty boy. With the T cell lymphomas that we see in
practice mycosis fungoides accounts for about 48% of them with a 3D30+
……………..lymphoma the next most prominent group and that includes
lymphomatoid papulosis. David talked about two neurons the Archimedes neuron
where you just look at something blink and say eureka I know what that is and
the holmesian neuron where it takes a series of deductions to make a diagnosis.
Very often with lymphoma it is the holmesian maximum neuron that has to work
because it is only by doing a series of immunoperoxidase stains and looking at
algorithms that you can often put the diagnosis to bed. He commented that
lymphomatoid contact dermatitis and lymphomatoid drug reactions were both
very naughty boys that can simulate a lymphoma. There also may be problems in
differentiating Sezary’s syndrome from a lichenoid drug reaction versus lichenoid
erythroderma. Other points I picked up are that you may get a single clone in a T
cell lymphoma that does not mean too much at all. Gene scan is a technique that
often shows several clones in separate samples but these clones are identical. I
had previously thought that a T cell lymphoma only had one clone of T cells but it
appears as if there can be several clones but they are identical clones. He then
illustrated his algorithm by going through several cases one of which was a
marginal zone lymphoma. In crossties lymphoma inside out follicles may be seen
histologically. Lymphomatoid granulomatosis may also be another simulant of
lymphoma and the CD4+ small pleomorphic T cell lymphoma can be a very
naughty boy. This was an excellent lecture for Pathologists but I have to admit a
lot of the immunoperoxidase stains and the significance was a bit beyond me.
Melanoma Guidelines 2008
These were discussed by Dr John Kelly and Dr Peter Heenan. The overall
lifetime risk of in situ or invasive melanoma in Australia is about 1 in 20. A 70
year old Australian has an 8 times increased risk of getting a melanoma than a
30 year old and the risk ratio is even greater in men than in women. The
estimated risk factors are that a previous melanoma gives you a relative risk
increase of 10. A large number of nevi has a risk factor of 6 to 7. Dysplastic nevi
gives you a risk factor of 6 to 7. Non melanoma skin cancers have a risk factor of
4 and there is a family history where if it is a close relative it will double the risk
and there is a 1.7 relative risk for each affected member. The CDKN2A gene
mutation is one of the commonest gene mutations in melanoma. If three first
degree relatives get melanoma and you examine these families then 5% will
have findings of this gene mutation in Australia. John went through various
recommendations. I have only made a note of some that caught my eye. Total
body photography is recommended for high risk individuals with a six month full
body examination. There was level 3 evidence for this. Level A being the most
secure. The sensitivity of dermoscopy in diagnosing melanoma was 10% higher
than the naked eye but it was not quite as good in the specificity. Hence one of
the new recommendations is that training is recommended in dermoscopy for
clinicians who are practicing in the field and examining skin lesions and a met
analysis of public studies said that there was …………day evidence for this. Most
of the studies on automated diagnosis had verification biases built into them and
hence the evidence was not good. With total body photography no studies
actually had a comparison arm done so this weakened the evidence quite a bit
and it was only Level 4 evidence. On the question of biopsy it was recommended
there be complete excision of pigmented lesions with 2mm margins where there
is a suspicion of melanoma. But it was also commented that partial biopsies are
acceptable where suspicion of melanoma is low. It was also thought good
practice to refer both clinical and dermatoscopic photos to a Pathologist to
increase the information that they have in assessing pigmented lesions.


Alex Chamberlain then gave a talk on nodular melanoma on the clinical and
dermoscopic features. Nodular melanomas have to have a very distinct with
rapid growth and they differ from the superficial spreading in that respect. They
are normally seen in older men particularly the head, neck, lower limb and back.
The way they grow and the rapidity of growth suggests that there may be a
divergent pathway for nodular melanoma and melanoma development. Chronic
UV damage is associated with nodular melanoma whereas superficial spreading
is more associated with intermittent blasts of UV. A nodular melanoma can grow
at 0.49mm a month with vertical growth whereas a superficial melanoma may
only grow at 0.12mm per month. 60% of nodular melanomas are pink or red
rather than pigmented. Only rarely are they crusted. Ignore the presence of
terminal hair in these lesions because it does not signify that the lesion is likely to
be benign. Most nodular melanomas are firm and rapidly growing. The ABCD
criteria do not work and one has to use EF and G. Elevated, firm and growing to
help to pick up nodular melanoma. Alex then went through some of the
dermoscopic features in nodular melanoma including the milky pink background
colour, the polymorphous vessels and polarising dermoscopy to show chrysalids.
Most nodular melanomas do have a small degree of pigment associated with
them even when they appear amelanotic so one should consider non pigmented
nodules very carefully. You do not monitor a nodular lesion, if you have doubts
about a nodular lesion you cut it out.
Dr Richard Scoliar gave a lecture on subungual melanoma. There is often a late
presentation of this condition for various reasons. He was recommending biopsy
at an earlier stage. 23% of Japanese melanomas are in fact acral but that is
because they get fewer of the other types whereas here in Australia at the
Sydney Melanoma Unit the incidence of acral melanomas is 0.4% because there
are so many of the other types. Richard commented that the nail plate is a strong
barrier to UVB but it certainly isn’t to UVA because we get photo onycholysis
which is separation of the nail from the nail bed often due to a photo drug
reaction so UVA certainly is able to penetrate and UVA may well be responsible
for melanomatous change in nevus or melanocytes. The most superficial part of
the nail plate is derived from the most proximal part of the nail matrix. The
deepest part of the nail plate is from the most distal part of the nail matrix. The
proximal nail plate has very little nail actually over it. In fact you only have a
squamous epithelium. There are various other causes of subungual pigmentation
ranging from haemorrhage through drugs and fungal infection. The dermoscopy
of a subungual melanoma shows irregular width and spacing of the bands. There
is often varying colours. If you have had subungual haemorrhage then look at the
proximal area near the lunular as it grows out because this area of nail should not
have any pigment in it. There should be a clear area between the proximal edge
of the cuticle all the time. Subungual melanomas seen in the Sydney Melanoma
Unit have on average a thickness of 3.2mm and 80% are Clark Level 4 or 5.
These figures obviously mean that you are going to get a poor prognosis.
Richard then gave a comparison of the features of acral nevi versus subungual
melanoma. Subungual nevi are seen in about 12% of longitudinal melanonychia
but it is as high as 50% in childhood. They are usually due to nevus cells in the
neo matrix. A subungual melanoma usually has infiltrated lymphocytes present
and the infiltrate is adjacent to the subungual melanoma in direct association with
the cells. This feature is uncommon in acral nevi. Features that favour melanoma
versus nevus are severe atypia, an inflammatory hallo, lymphocytes within the
lesion, architectural disorder, a much greater density of cells and haphazard
pagetory spread which is seen higher in the nail and is a late phenomenon. A
subungual lentigo generally gives a mild increase in melanocytes and an
increase in the pigmentation in keratinocytes. Dermoscopy shows a grey
pigmentation and fine grey stripes that do not change. Note again the greater
density of cells in a subungual melanoma versus the nevus. Complete stretches
of single melanoma cells can be seen like a tombstone. They are often multi
nucleated melanocytes. There may be lichenoid inflammation and atypia. When
gene studies are done on acral melanomas the peripheral edges that may just be
melanoma in situ in fact if you have genetic analysis done you find that it may
extend between 4mm and 6mm beyond the edge of where you think the lesion
ends. This indicates why these lesions have to be taken out with wide margins or
recurrence is likely.
Steven Cossard looked at the similarities between reaction patterns in the skin
and the mucosal surfaces. He particularly looked at the psoriasiform pattern, the
lymphocytic lichenoid pattern and spongiosis. Note that spongiosis can be
induced by both irritation, infection and allergy and is characterised by
neutrophils and eosinophils. Contact dermatitis though in the mouth is a lichenoid
reaction and in the skin is a spongiotic reaction. Eosinophilic and neutrophilic
spongiosis though can occur in both the mouth and the skin. He then looked at
the concentrations of desmal ……………1 and 3 in both mucosal surfaces of the
mouth and in the skin in both pemphigus foliaceus and pemphigus vulgaris.
Pemphigus vulgaris regularly gives ulceration in the mouth whereas pemphigus
foliaceus does not and it is due to the different desmal …………that has
antibodies to it in these conditions. There is also varying levels within the skin in
the situation of the desmal……………..with desmal ……………3 being deeper in
the skin than desma ……………..1. He thought the geographic tongue was not
really an association with psoriasis. The lichenoid pattern was often expressed
both in the mouth and on the skin. Even contact sensitivity reactions in the mouth
tend to be lichenoid for example mercury and amalgam. In the mouth if you have
some associated acantholysis as well as a lichenoid reaction consider that this
might be paraneoplastic pemphigus. In lichenoid reactions the pathology is likely
to be autoimmune. With psoriasiform reactions it is likely to be the
……………….immune system acting and the spongiotic reactions he felt it was
delayed immunity type 4 reactions that were important. Most lichenoid reactions
are type 2 reactions. The oral mucosa is an area for the induction of immune
tolerance suggesting that there is a difference in androgen handling. Toll like
receptors and neutrophilic pattern are associated with the psoriatic reaction
pattern. Toll like receptors can stimulate the acquired immune system as well.
These receptors are generally associated with bacterial infection or a part of the
………… immune system.
The clinical gem section then had several presenters. There was a case of
CK20+ extramammary Paget’s of the urothelial type having a poor prognosis.
The second clinical gem was on VIN. It was commented the warty basaloid VIN
is the commonest type of VIN but it only causes less than 20% of the invasive
SCCs seen in this condition. Adequate biopsies are necessary to be inclusive of
the depth of the lesion to try and pick up any cases of VIN that may be forming
invasive SCCs. Shave biopsies are not acceptable. John Snow presented a case
of an unusual fungal infection showing sporotrichoid spread. Commented that
histologically histiocytes plus small collections of neutrophils basically equals
infection.
There was an excellent talk on scleromyxedema being treated with high dose
intravenous immunoglobulin. The condition began as lichen myxedematosus
and subsequently evolved into scleromyxedema. Other differentials to be
considered in these presentations include nephrogenic fibrosing dermopathy,
system sclerosis and amyloidosis. The intravenous immunoglobulin dose used
was 0.4 grams daily for four weeks. IVIG appears to have anti …………….type
antibodies in it and it may play a part in clearing the abnormal antibody seen in
scleromyxedema. There was an interesting case of a nasal glioma which is
ectopic brain tissue it is firm and non pulsatile. The differential diagnosis includes
other cysts and vascular tumours but particularly an encephalocele. Generally
encephalocele’s will communicate with the skull vault and they will tend to
enlarge with crying. The bone adjacent to the nasal glioma was often very thin
and occasionally a fibrous stalk may extend to the cranial cavity but there does
not appear to be any bony defect.
There was a case of primary cutaneous angioimmunoblastic T cell lymphoma
with no signs of system disease, an initial diagnosis of granuloma faciali was
made because the lesions occurred on the face and had a gren zone. The cell of
origin of this condition appears to be the follicular helper T cell, CXCL13 is a
marker for it. When studies were done on biopsies from different areas there
was the same T cell clone in different sites and times. Low dose Methotrexate
was used at 5mg per week and the patient improved dramatically and cleared.
There was a good case presented of lipo dermatosclerosis having some great
images. The other name for this is sclerosing panniculitis. 68% of cases of
significant venous abnormalities may have had previous DVTs. Histologically the
phases may be divided into an early, intermediate and late phase, the late phase
being characterised by fat microcysts with membranous changes. When
treatment is just with compression stockings, some cases can be due to
disorders of fibrinolysis and there is often a small vessel vasculitis. Stanozolol
can be effective treatment. The last case was one of primary cutaneous angio
plasma cellular hyperplasia. Apparently there has only been one other case
reported in literature. This case presented as a fairly nondescript nodule on the
neck. I have no doubt if I see anything similar in the future I will probably shave it
off as a presumptive SCC and it would probably need a pathologist of David
Weedon’s stature to diagnose this particular angio plasma cellular hyperplasia
condition as well.

				
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