Congenital Cutaneous Candidiasis-Case Report

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             A CASE REPORT
           AUGUST 2003, MANILA



*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

after birth.

     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.


   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

was discontinued.

   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 and Gonzalez 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

cryptococcal infection.17


   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


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