CONGENITAL CUTANEOUS CANDIDIASIS
A CASE REPORT
PRESENTED DURING THE PSCM CME
AUGUST 2003, MANILA
JOSE BENJAMIN B. UNTALAN, M.D.*
KAREN LEE ALABADO, M.D.**
DEPARTMENT OF DERMATOLOGY
DAVAO MEDICAL CENTER
DAVAO CITY, PHILIPPINES
*Resident , **Consultant
To describe Congenital Cutaneous Candidiasis a rarely reported disorder
acquired by the infant in utero or during delivery from a mother who had vaginal
candidiasis 12 weeks before delivery.
A case report
Davao Medical Center- a tertiary government hospital
A one (1) day old Filipino female
A one day old female newborn presented with generalized pleomorphic
lesions characterize as erythematous erosion, hemorraghic bullae, vesicles, and
plaques with good APGAR score and normal hematologic picture. KOH mount
and histopathology results were positive for hyphal elements consistent with
candidiasis. The patient was started with systemic antifungal fluconozole and
topical nystatin with resolution of lesions.
This case is being reported because fewer than 100 cases were reported in
English literature and 70 cases in the 1990. The pathogenesis of congenital
cutaneous candidiasis is partially understood. Presumably it is an ascending
intrauterine infection with cutaneous manifestation that typically presents right
Furthermore, in contrast to systemic candidiasis, congenital cutaneous
candidiasis has a benign course.
Congenital cutaneous candidiasis is a rare disease believed to be due to the
colonization of the female genital tract by Candida spp. during pregnancy. The
pathogenesis of congenital cutaneous candidiasis is only partially understood.
Candidal infection may be congenital, acquired in utero from an antecedent
chorioamnionitis or may present as neonatal candidiasis, candidiasis presents
shortly after birth.1
Congenital cutaneous candidiasis presents as a generalized, erythematous,
maculopapular or maculovesicular eruption. It is often present without
constitutional signs and symptoms and clears quickly with topical antifungal or
oral plus topical antifungal therapy.2 To the best of our knowledge there has
been no published case reports of such disease in the Philippine setting.
REPORT OF A CASE
This is a case of S.B. 1 day old female neonate, who was referred from the
Neonatal Intensive Care Unit to the Dermatology service because of rash.
The infant was born by normal spontaneous vaginal delivery ( NSVD ) at 37
weeks of gestation to a 22 years old woman G 1P1 with regular prenatal care
since 1 month AOG. The mother received a complete dose of Tetanus toxoid on
the last trimester of pregnancy. She had iron and folic acid preparation during the
gestation. By prenatal history, she denies any history of rashes, fever, use of
illicit drugs,alcohol or tobacco during the period of gestation. However, two
months prior to delivery the mother reported a pruritic whitish vaginal discharge
but no treatment was done. Both parents deny of any history of heredofamilial
illnesses. Mother had varicella in childhood. The father denies of extramarital
After the infant was born by NVSD, the APGAR scores were 8 at 1 minute
and 9 after 5 minutes. Generalized erythemaous rash were noted on
examination, hence was admitted at the Neonatal Intensive Care Unit and was
referred for further evaluation and management to the Department of
On physical examination, the patient was awake, afebrile and not in distress
with the following vital signs HR 140 beats / minute RR 50 cpm T 37. 3 0 C
The rest of the physical examination was normal.
The skin revealed scattered eroded and crusted vesicles atop erythematous
bases (Fig. 1). Erythroplakia was noted on the upper palate (Fig. 2). Soles
revealed the presence of hemorrhagic bullae and vesicles (Fig. 3) later on
evolving to crusted hyperpigmented plaques involving the plantar area and
toenails with associated nail dystrophy (Fig.4). The back area had scattered
erythematous erosions (Fig. 5).
On day 1, TORCH was initially entertained. However, blood exams were
within normal limits. Tzanck’s smear was negative. Skin punch biopsy and
GS/CS of wound discharge were requested. Patient was started with Ampicillin
150 mg. IV q 12 hours, Gentamycin 15 mg IV daily and Mupirocin ointment
applied on the skin lesions BID.
On the 3rd hospital day no new lesions were observed except for
desquamations on the resolving lesions. KOH mount of skin scrapings was done
and revealed peudohyphae. Histopath result showed subcorneal neutrophilc
pustules, papillomatosis with superficial layers of the epithelium infiltrated by
large number of neutrophils. There were cellular infiltrates of
lymphocytes,neutrophils and eosinophils in the upper dermis (Fig 6). PAS stain
showed pseudohyphae on the subcorneal pustules in the upper and lower
dermis. Histopathologic diagnosis was consistent with Candidiasis (Fig 7).
Flouconozole was started 5 mg./k/d, dissolving 50 mg in 5 ml. of water, 2ml.
given OD. Ampicillin was discontinued.
On the 7th hospital day 30% of lesions improved with some
hypopigmentations. Blood culture and sensitivity were negative. Gentamycin
On the 9th hospital day 50 % of lesions improvedTopical Nystatin was started
applied on the affected areas BID. Ultrasound of the abdomen was normal.
On the 14th hospital day the patient was discharged in good condition.
Fluconozole was discontinued. Nystatin was continued at home and was advised
for follow up after 7 days at Davao Medical Center outpatient department (Fig.
A one day old female presented with generalized pleomorphic lesions
characterized as erythematous erosions, hemorrhagic bullae, vesicles and
plaques. The initial impression was TORCH on the basis of the above
manifestations that were present right after birth. The cutaneous lesions were not
consistent with what have been described as the typical lesion “ blueberry muffin
spots” consisting of petechiae and purpura of congenital toxoplasmosis. Other
findings include central nervous system malformations with ocular and neurologic
findings, myocarditis, pneumonitis, hepatosplenomegaly, lymphadenopathy and
gastrointestinal problems, were not present in this case. Other disease in the
syndrome like congenital Rubella typically presents with the triad of cataract,
deafness and heart malformation in a child who had primary rubella in
pregnancy. The cutaneous lesions of congenital Rubella are similar to those
associated with congenital Toxoplasmosis. Other findings include central
nervous system abnormalities, ophthalmologic abnormalities, lymphadenopathy
and thymic aplasia that were absent in the patient. Vesicular lesions do not
occur. The absence of maternal rubella provides a strong evidence against a
diagnosis of congenital rubella in this case. 90 % of Cytomegalovirus infected
neonates are asymptomatic and symptomatic disease presents with petechiae,
jaundice or hepatosplenomegaly. Sixty percent of symptomatic neonates have
clinical neurologic abnormalities. The absence of neurologic signs and the rarity
of vesicles ruled out congenital cytomegalovirus infection. Congenital herpes
simplex infection consist of vesicles, bullae and scars. The negative Tzanck’s
smear ruled out this diagnosis.
Another condition which was considered was Varicella infection because of
the widespread vesicular eruption The negative Tzanck’s smear and history of
maternal varicella ruled out this diagnoisis.
Two vesicular and pustular eruptions occur in otherwise healthy neonates.
Erythema toxicum neonatorum develops in approximately 50 % of full term
neonates presents with erythematous macules containing central vesicles or
pustules. The process resolves entirely in two weeks. Milaria results in the
obstruction of eccrine glands. The lesions are not present at birth but may
develop days afterwards without any sequelae of the disease.
The patient was further evaluated by doing KOH and PAS further confirmed
the presence of hyphae and so our final impression was Congenital Cutaneous
CCC is a rare clinical entity in which intrauterine Candida infection becomes
manifest at birth.1,5 The disease was first described by Sonnenschien et. al. in
1960 and fewer than 100 cases were reported in English literature 2,4 and 70
cases in the 1990’s.3 According to Almeida and colleagues reported 15 cases of
CCC during the period 1968 –1986. Few cases has documented in the literature
in Medline during the period of 1990-June 1998.12
The pathogenesis of CCC is not fully understood. Several pathogenic
mechanism have been considered. Neonates may acquire Candida by vertical
or horizontal (nosocomial) transmission.6,12,13,14 In this case the mother had a
history of vaginal candidiasis 2 months prior delivery that resulted in vertical
transmission. Martinez et.al and Gonzalez et.al reported CCC born to a mother
with severe vaginal candidiasis one week before delivery.2,12 The likelihood of
colonization increases in pregnancy to 25-20% few ascending infection are
reported. Pregnancy and oral contraceptive affect the carbohydrate content of
the vagina and its pH which leads to overgrowth of Candida in the vagina.4
Sonnenshien et al. thought that CCC might result from the combination of
vaginal candidal infection, prolonged rupture of membranes, and intensive
adminstration of antibiotics6, all of their first patient had.2,4 Flamm demonstrated
that Candida cannot pass the placental barrier; therefore the presence of
candidal infectrion at birth, even in the absence of ruptured membranes, suggest
an ascending infection. It seems that Candida present in the maternal vaginal
flora somehow penetrates through grossly intact fetal membranes to cause
candidal chorioamnionitis, so that infected amniotic fluid baths the entire
cutaneous surface of the fetus.
Factors associated with CCC include foreign body in the uterus, retained IUD,
sutures of cervical cerclage. Factors that don’t seem to increase the risk factors
include the duration of rupture of membrane, labor type of delivery, history of
maternal antibiotic, steroid treatment, maternal age and parity.4,9,11,16
CCC presents with extensive rash that becomes manifest within the first
hours of life. Macular erythema may evolve from pustular, popular or vesicular
phase, bullae then extensive desquamation.6,7.8,9 Most commonly affected are
the trunk, neck, face and extremities.2 CCC involves the palms and soles.
Palmar and plantar pustules are considered hallmark of CCC.4 Paronychia and
dystrophy are sometimes present.10 CCC is usually a benign infection 3,7,8
resolves in 1-2 weeks of life. 6
The presence of vesiculobullous or pustular lesion in the neonatal period and
infancy may represent benign non-infectious, transient neonatal disorders, mild
infections, serious infections or potentially serious non-infectious disorders.
Neonatal sepsis is an important consideration in this neonate. The
manifestation of neonatal sepsis is non-specific, with fever, respiratory distress,
lethargy, petechiae, purpura and shock. In this case the patient did not manifest
any fever. Although antibiotics was started in this patient it was important to treat
the neonate for possible bacterial sepsis.
KOH preparation of skin scrappings from the involved skin should readily
demonstrate a budding yeast and pseudohyphae. 3 4 KOH is still the easiest and
most cost effective method for diagnosing CCC.3 If still in doubt, a biopsy of the
specimen with PAS should reveal the fungal elements within the stratum
If CCC is suspected early, it is important that placenta and cord be examined
for gross lesions and cultures of the tissues including the amniotic fluid.
Complete blood count with differentials are normal with only cutaneous
involvement. The majority of infants with CCC have disease limited to the skin,
umbilical cord and placenta, and they lack constitutional symptoms.4
The treatment of CCC in its purely cutaneous form consists of topical
anticandidal agents alone. Oral Nystatin suspension is added to decrease the
number of organisms in the gastrointestinal tract.3,4 In this case oral fluconozole
was given, a new triazole antifungal agent with activity against many common
fungal pathogens. Although not commonly used, fluconozole has a favorable
pharmacokinetic profile that includes a long serum half-life, making once daily
administration possible, more consistent absorption than ketoconozole.
Flouconozole is currently approved for the treatment of Candida and
Our case of CCC resulted from vertical transmission, the first to be
documented in our setting. The patient had a favorable outcome with oral and
topical antifungal agents.
It is important that CCC is a significant differential diagnosis in a patient with
vesiculobullous and pustular lesions. The proper diagnosis should be established
with the help of laboratory parameters so that prompt and proper treatment be
Fig . 1 Scattered eroded and crusted vesicles with erythematous bases.
Fig . 2 Erythroplakia of the upper palate.
Fig. 3 Hemorrhagic bullae and vesicles of the soles.
Fig. 4 Crusted hyperpigmented plaques involving the toenail with associated nail
Fig. 5 Erythematous erosions of the back.
Fig. 6 Subcorneal neutrophilic pustules, papillomatosis with superficial layers of
the epithelium infiltrated by large number of neutrophils. There are cell infiltrates
of lymphocytes,neutrophils and eosinophils in the upper dermis.
Fig. 7 PAS stain showing pseudohyphae on the subcorneal pustules in the
upper and lower dermis. Histopathologic diagnosis was consistent with
Fig. 8 With clearing of cutaneous lesions on the 14th hospital day
1. Dvorak AM, Gavaller B. Congenital Systemic Candidiasis New England
Journal of Medicine 1966. 274:540-543
2. Martinez, Fernandez N, Centeno, Gomez P, Conde Gonzalez MV, et. al.
Candidiasis Cutanea Congenita. A propositode dos casos, An Esp.
3. Lambiase M, Vaughan T. Candidiasis,Cutaneous, eMedicine, Jan. 18,2002:
4. Cosgrove B, Reeves K, Mullin D, et. al. Congenital Cutaneous Candidiasis
associated with respiratory distress and elevation of liver function test a
case: report and review of literature. Journal of American Academy of
Dermatol. November 1997: 817-833
5. Martin AG, Kobayashi GS. Yeast infections: Candidiasis, Pityriasis (Tinea)
Versicolor.In: Freedberg I, Eison A, Wolff K, et.al. 5th ed. Fitzpatrick
Dermatology in General Medicine. New York: Mc Graw-Hill. 1999: Vol. 2
6. Chapman R. Candida Infection in the neonate.Current Opinion in Pediatrics.
Feb. 2003: 97-102
7. Santos A, Beceiro I, Hernandez R, et.al. Congenital Cutaneous Candidiasis:
a report of four cases and review of literature.1991, European Journal Ped.
8. Knoebel R ,Congenital Cutaneous Candidiasis: A case presentation,
Neonatal Network,Sept.- Oct. 2002 Vol.21 No. 6: 1-2.
9. Darmstadt G, Dinulos JG, Miller,Z. Congenital Cutaneous Candidiasis:
Clinical presentation, Pathogenesis and management guidelines, Pediatrics,
Feb. 2000:105(2) 438-44.
10. Arbegast K, Lambery M, Koh J , et.al. Congenital Cutaneous Candidiasis
limited to the Nailplates. Pediatric Dermatology, 1990 7:310 -12.
11. Saiman L., Ludington E., Pfaller M, et.al. Risk factors for candidemia in
Neonatal Intensive Care Unit Patients (original studies), The Pediatric
Infectious Disease Journal, April 2000, vol. 19(4) : 319-24.
12. Gonzalez de Dios J., Moya M , Gonzales R. Candidiasis Cutanea Congenita:
Una ntidad para Recordar. An Esp. Pediatric Journal 1999: 50:499-500.
13. Reef SE, Lasker D., Butcher,DS, et.al. Non-perinatal nosocomial
transmission of Candida Albicans in a neonatal intensive care units.
Prospective Study, Journal Clinical Microbiology, 1998, 36: 1255-1259.
14. Waggoner-Fountain L, Walker MW, Hollis RJ, et.al. Vertical and Horizontal
transmission of the Unique Candidia species to premature newborns.
Clinical Infectious Disease, 1996, 22:803-808.
15. Shoff PS, Parikh DA, Fernandez RJ, et.al. Clinical and Mycological Spectrum
of Cutaneous Candidiasis in Mumbai, Journal of Postgraduate Medicine.
1990: 36 (2) : 83-86.
16. Delprado WJ, Baird PJ, Russel P. Placental Candidiasis: report of 3 cases
with a review of lit. Pathology, 1982, 14: 191-195.
17. Winston DJ, Chandrasekar PH, Pranatharti H, et.al. Fluconozole Prophylaxis
of Fungal Infections in Patients with Acute Leukemia: Results of a
Randomized Placebo-Controlled, Double Blind, Multicenter Trial, Annals of
Internal Medicine, April 1, 1993 : vol. 118 (7): 495-503.