Chronic Mucocutaneous Candidiasis Case Report

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Chronic Mucocutaneous Candidiasis: A Case Report.

ABSTRACT                                                                            can be made based on clinical findings. Microscopy and
Chronic mucocutaneous candidiasis is a immuno deficiency disorder                   culture of skin swabs and scrapings confirms the presence
primarily due to T cell dysfunction characterized by persistent
                                                                                    of microorganisms. Serum values for various endocrinopathies
candidal infection of mucous membrane, skin, scalp and nails.                                                                           8
Chronic mucous membrane candidiasis has an onset in infancy or                      formulate an important key to diagnosis .
childhood; the primary affected site is the oral cavity; however, lesions
may occur on trunk, hands, feet and scalp. This paper describes a 12-               CASE REPORT
year-old girl with candidial infection of the oral mucosa and extra oral
involvement of fingers, nails, toes and intertragus area.
                                                                                    A 12-year-old female patient reported to the Department of
                                                                                    Pedodontics Govt. Dental College Raipur with the complaint
Keywords: Autoimmune, Moniliasis, Polyendocrinopathy,
Thrush.                                                                             of broken maxillary anterior tooth. She wanted to replace the
                                                                                    same with an artificial tooth. Intra oral examination revealed
INTRODUCTION                                                                        carious maxillary anteriors and premolars, bilaterally. Soft
                                                                                    tissues examination revealed white curd like lesions scattered
Chronic mucocutaneous candidiasis (CMC) is the term used                            all over the buccal mucosa (Fig 1), upper and lower labial
to describe a rare, heterogeneous group of syndromes in                             mucosa (Fig 2), palatal mucosa (Fig 3) and dorsal surface
which affected individuals with congenital, endocrinological                        of tongue with loss of papillae and prominent central groove
or immunological disorders develop recurrent or persistent                          (Fig 4). The hands and feet showed scaly lesions with
mucosal, cutaneous or nail infections with Candida albicans.                        involvement of intertrigenus area and nails (Fig 5 and 6). Nails
Chronic mucocutaneous candidiasis is associated with                                were markedly thickened, fragmented and discolored with
endocrine conditions like hypoparathyroidism,                                       significant edema. Erythema of the surrounding periungal
hypothyroidism, hypoadrenalism and diabetes mellitus .                              tissue simulating clubbing was also present. Scrapping from
Immune defects i.e. malfunctioning of lymphocytes, low levels                       the lesion revealed the presence of Candida albicans.
of immunoglobulin is also a contributory factor . Rarely chronic                    Laboratory investigations revealed Hb 8gm%, total leukocyte
mucocutaneous candidiasis develops in adult life. This is                           count 3500 / cu mm, ESR 35mm at the end of one hour
often as a result of a thymoma (tumor of thymus gland) and                          (Westergen method). T3, T4, TSH and liver function tests
is associated with internal disease such as myasthenia gravis,                      were within normal limits. Patient was medicated with
myositis, aplastic anaemia and neutropenia. Patient presents                        ketoconazole 150 mg systemically along with topical
with a history of recurrent or persistent superficial infections                    application for 12 weeks. Carious broken maxillary teeth and
of the oral cavity (thrush), intertriginous or perioro-facial                       root pieces were extracted and replaced with a removable
structures. Infants often present with candidial diaper                             space maintainer. Patient responded well to the therapy and
dermatitis . These infections can be followed by more                               oral lesions disappeared completely after six weeks. Finger
extensive scaling of skin lesions, as well as thickened nails                       nails and toe lesions started healing after 10 weeks of therapy.
and red swollen periungal tissues . The scalp may be involved                       After 12 weeks, patient was asked to continue with
with similar hyperkeratotic plaques, which can result in                            ketoconazole once in a week for next six months. The patient
scarring and alopecia .                                                             was followed upto two years without recurrence.
Some patients with chronic mucocutaneous candidiasis have
an associated abnormality of iron metabolism demonstrated                           DISCUSSION
by low serum iron and decreased iron stores . These
abnormalities may be caused by decreased iron absorption.                           Chronic mucocutaneous candidiasis is a heterogeneous
Provisional diagnosis of chronic mucocutaneous candidiasis                          disorder of immune system characterized by infection of
                                                                                    mucous membrane, scalp, skin and nails. Familial CMC may
a.Principal, b.Assistant Professor, Dept. of Pedodontics & Pre-                     be associated with autoimmune polyendocrinopathy . Most
ventive Dentistry, Govt. Dental College and Hospital, Raipur,                       patients with CMC have a selective defect of cell-mediated
                                        Chronic Mucocutaneous Candidiasis

Fig 1: Clinical photograph showing candidial lesions       Fig 2: Clinical photograph showing candidial lesions
      on atrophic buccal mucosa.                                  on labial mucosa.

Fig 3: Clinical photograph showing candidial lesions        Fig 4: Clinical photograph showing depapillation of
      on palatal mucosa with carious 11 & 21.                      tongue with central groove & candidial lesions.

      Figs 5 and 6: Clinical photograph showing markedly thickened nails, scaly lesions of hand and feet,
                    erythema of surrrounding periungal tissue, simulating clubbing.
                                                 Chronic Mucocutaneous Candidiasis

antifungal agents for repeated or prolonged courses often in        3.   Rothberg M.S., Eisenbud I. and Girboff S.: Chronic
higher doses than is normally necessary for Candida                      mucocutaneous candidiasis thymoma syndrome. A case
infections. Terbinafine has been used effectively in                     report.Oral Srg Oral Med. Oral Pathol.1989; 68(4) : 411 - 3.
                                                              9     4.   Caputo R.V.: Fungal infections in children. Dermatol
Ketoconazole resistant chronic mucocutaneous candidiasis .
                                                                         Clin.1986; 4(1): 137 - 349.
Use of transfer factor, Levamisole, thymosin and lymphocyte
                                                                    5.   Jorizzo J.L.: Chronic mucocutaneous candidiasis. An update
infusion is still under experimental stages. Transfer factor             Arch. Dermatol 1982; 118(12): 963 - 965.
and cimetidine act as immuno modulatory agents. The ultimate        6.   Boni R., Trueb R. M. and Wuthrich B.: Alopecia areata in a
judgment regarding the propriety of any specific procedure               patient with candidiasis-endocrinopathy syndrome
must be made by physician in light of all circumstances                  unsuccessful treatment trial with diphenyl cyclo propenone.
presented by individual patient.                                         Dermatology. 1995; 191(1): 68-71.
Clinical significance of chronic mucocutaneous candidiasis          7.   Masi M., Devinci C. and Baricord O. R.: Transfer factor in
                                                                         chronic mucocutaneous candidiasis. Biotherapy, 1996; 9 (1-
lies in the fact that it may present as an inaugural symptom
                                       1                                 3): 97-103.
of auto immune polyendocrinopathy and should lead to a
                                                                    8.   Weiner M.H. and Coats-stephen M.: Immuno diagnosis of
familial study with an endocrinology work-up to determine                systemic candidiasis mannan antigenemia detected by
any clinical manifestations and biological evidence of auto              Radio Immunoassay in experimental and human infections.
immunity. Care should be taken to screen patients periodically           J Infect Dis 1979; 140 (6): 989 - 993.
for the emergence of endocrinopathy. Parents should be              9.   Hassan C: Terbinafine effectiveness in ketoconozole
alerted regarding chronic recurrent nature of disease and                resistant mucocutaneous candidiasis in polyglandular auto
                                                                         immune syndrome type 1 : J Assoc Physicians India 2003;
current paucity of effective long lasting therapies.
                                                                         51: 323.

1.   Rybojad M., Abimelec P., Feuilhafe M., Morel P. and Bourrat    Reprint Requests to :
     E.: Familial CMC associated with Autoimmune                    Dr. Rahul Bhowate
     Polyendocrinopathy. Treatment with Fluconazole 3 cases.        Principal,
     Ann Dermatol Venerol.1999 ; 126(1): 54 - 56.                   Govt. Dental College & Hospital,
2.   Kalfa V.C., Robert R.L. and Stiehm E.R.: The syndrome of       Raipur, Chattisgarh.
     CMC with selective antibody deficiency. Ann Allergy Asthama
     Immunol 2003; 90(2): 259 - 264.

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