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CASE REPORT
Year : 2011  |  Volume : 56  |  Issue : 1  |  Page : 92-93
Congenital cutaneous candidiasis: A rare and unpredictable disease


Department of Pediatrics, Grant Medical College and J.J. Group of Hospitals, Byculla, Mumbai, Maharashtra, India

Date of Web Publication10-Mar-2011

Correspondence Address:
Sujit A Jagtap
Department of Pediatrics, Room no. 202, CWC Building, Grant Medical College and J.J. Group of Hospitals, Byculla, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.77564

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   Abstract 

Congenital cutaneous candidiasis (CCC) is an extremely rare disorder that presents within the first 6 days of life. The manifestations ranges from diffuse skin eruption without any systemic symptoms to respiratory distress, hepatosplenomegaly, sepsis, and death. We report a neonate who presented with generalized skin eruptions at birth, characterized by erythematous macules and papules. The eruption involved head, face, neck, trunk, and extremities. Candida albicans was demonstrated on direct KOH smear, skin biopsy. The disease implies a congenital intrauterine infection and is different from neonatal candidiasis, which manifests as thrush or diaper dermatitis. The infection is acquired from the maternal genital tract in an ascending fashion. Clinical features, direct smear examination of specimen, and appropriate cultures are useful in differentiating the lesions from other more common dermatoses of the neonatal period. Topical antifungal therapy is sufficient unless systemic candidiasis is present. Prognosis for congenital cutaneous candidiasis is good.


Keywords: Congenital cutaneous candidiasis, Candida albicans, immunocompromised


How to cite this article:
Jagtap SA, Saple PP, Dhaliat SB. Congenital cutaneous candidiasis: A rare and unpredictable disease. Indian J Dermatol 2011;56:92-3

How to cite this URL:
Jagtap SA, Saple PP, Dhaliat SB. Congenital cutaneous candidiasis: A rare and unpredictable disease. Indian J Dermatol [serial online] 2011 [cited 2019 Sep 15];56:92-3. Available from: http://www.e-ijd.org/text.asp?2011/56/1/92/77564



   Introduction Top


Newborn babies commonly develop mucocutaneous candidiasis, which is usually acquired during the process of birth. Congenital cutaneous candidiasis presenting at birth is very uncommon and is due to intrauterine infection by the organism. Up to 25% of pregnant women harbor Candida albicans in their vaginal flora, and hence, mucocutaneous candidiasis is common in their babies. By contrast, congenital cutaneous candidiasis is very uncommon with only about 70 cases reported in the literature over the past 40 years and none in Indian literature.


   Case Report Top


A female child weighing 3.1 kg was born to a 30-year old gravida 3, parity 2, and VDRL negative mother by vaginal breech delivery. Mother did not have any per vaginal leak, bleed, or premature rupture of membrane prior to onset of labor. On examination, the baby was well developed, stable having generalized, erythematous "maculopapular" eruptions over the face trunk, back and extremities [Figure 1]. In many areas, the rash was papulovesicular. The palms, soles, and mucus membrane were spared. Cord, membranes, and placenta appeared normal on gross examination. Her white blood cell count was 17,000/mm 3 with 45% polymorphonuclear cells, 6% band cells, 30% lymphocytes. Chest radiograph was normal. Scrapings of the lesion showed budding yeast cells with chlamydospore formation. Blood and urine culture were obtained on admission were negative. Skin biopsy with Periodic acid-Schiff (PAS) staining revealed the fungus within stratum corneum. Topical antifungal therapy was started. No further complications were observed; vesicles began drying by fourth day and desquamated completely by day 10.
Figure 1: One-day neonate with rash due to C. albicans. The rash was primarily papular involving back and extremities

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   Discussion Top


Congenital candidiasis was first reported in 1960 by Sonnenschein et al. [1] Fewer than 100 cases have been reported since then. Pathogenesis is not completely understood. According to Sonnenschein et al., congenital cutaneous candidiasis (CCC) occurred from a combination of maternal vaginal candidiasis, prolonged rupture of membranes, and administration of antibiotics. Drovak et al.[2] described CCC in an infant born by caesarean section without PROM. Bruner et al. [3] described chorioamnionitis due to candida by amniocentesis. Although 20%-25% of women harbor candida species in their vagina only a few ascending infections are noted. Whether this relative infrequency of CCC is because of asymptomatic infection or resistance to uterine invasion by candida is not known. A factor associated with congenital candidiasis is a foreign body in the uterus or cervix, such as a retained IUCD or sutures placed for cervical encirclage. [4]

CCC presents as a generalized eruption detected at or within 72 h of life. It begins as erythematous macules that may evolve through papular, vesicular, and pustular stages. [5],[6] Bullae have rarely been reported. Rash often involves palms and soles unlike erythema toxicum or milaria. Nail dystrophy may be seen. [7] Prematurity is generally accepted as a factor predisposing to disseminated disease because of their immature, compromised mucocutaneous barrier and systemic host defenses. Those with burn-like dermatitis, respiratory distress, altered liver enzymes as well as laboratory evidence of sepsis should be considered systemically infected until proven otherwise. [5] Differential diagnosis includes listeriosis, syphilis, staphylococcal, and herpes simplex infection as well as erythema toxicum, transient neonatal pustular melanosis, malaria, and drug eruption. Infection may be limited to the skin or may disseminate causing respiratory distress, sepsis, or deranged liver function tests.

Johnson et al.[8] studied 31 case reports of newborns with CCC and showed that 15 responded to topical therapy alone. They suggested that the risk of dissemination of CCC is increased if: (1) there is an evidence of respiratory distress or other signs of sepsis in immediate neonatal period; (2) birth weight is <1500 gm; (3) received treatment with broad spectrum antibiotics; (4) extensive instrumentation during delivery and (5) positive blood, urine, or Cerebrospinal fluid (CSF) culture. A potassium hydroxide preparation of the skin scrapings will demonstrate budding yeast and pseudohyphae. Skin biopsy with PAS staining will reveal the fungus within the stratum corneum. Blood, urine, and CSF cultures should be obtained to rule out systemic involvement.

Treatment is in the form of topical antifungal therapy given the benign nature of the illness. [9] Oral nystatin suspension is often added to decrease the number of organisms in the gastrointestinal tract. [8] Systemic antifungal therapy is recommended for neonates with burn-like dermatitis attributable to Candida species, or positive blood, urine, and/or cerebrospinal fluid cultures. Systemic treatment also should be considered for all infants with CCC who have respiratory distress in the immediate neonatal period and/or laboratory signs of sepsis such as an elevated leukocyte count with an increase in immature forms or persistent hyperglycemia and glycosuria [5] .Amphotericin B (0.5-1 mg/kg/day) is the first line agent for treatment of systemic disease. [10] Lipid-associated amphotericin B preparations (35 mg/kg/day) are preferred in those cases with invasive candidiasis and severe preexisting renal insufficiency. Fluconazole (6-12mg/kg/day) can be used as alternative therapy to amphotericin B, if the Candida species is identified and the susceptibility is shown or toxicity of Amphotericin B is prohibiting its use. Flucytosine (50-100 mg/kg/day) is used in combination with amphotericin B for central nervous system infection. There are no controlled clinical trials to provide the optimal length of therapy. The topical antifungal agent must be used until the lesions resolve, and the systemic therapy must be used a minimum of 21-28 days. Prompt initiation of antifungal therapy appears to be the most important associated with survival in systemic infection. We emphasize the self-limited character of this disease, although preterm infants may be at risk of systemic spread.



 
   References Top

1.Sonnerschein H, Taschdijan CL, Clark DH. Congenital cutaneous candidiasis. Am J Dis Child 1964;107:260-6.  Back to cited text no. 1
    
2.Dvorak AM, Gavallu B. Congenital systemic candidiasis. NEJM 1966;274:540-3.  Back to cited text no. 2
    
3.Bruner JP, Eliott JP, Kilbride HW, Garite TJ, Knox GE. Candidial chorioamnionitis diagnosed by amniocentesis with subsequent fetal infection. Am J Perinat 1986;3:213-8  Back to cited text no. 3
    
4.BF Cosgrove, K Reeves, D Mullins. Congenital Cutaneous candidiasis associated with respiratory distress and elevation of liver enzymes; a case report and review of literature. J Am Acad Derm 1997;37:818-23.  Back to cited text no. 4
    
5.Darmstardt G, Dinulos J, Miller Z. Congenital Cutaneous candidiasis- Clinical presentation, pathogenesis and management guidelines. Pediatrics. 1996.  Back to cited text no. 5
    
6.White RK, Hussain Z, Desa D. Antenatal infection with candida species. Arch Dis Child 1982;57:528-35.  Back to cited text no. 6
    
7.Raval DS, Barton LL, Hansen RC. Congenital cutaneous candidiasis; Case report and review. Ped Dermatol 1995:12.  Back to cited text no. 7
    
8.Johnson DE Thompson TR, Ferreiri P. Congenital candidiasis. Am J Dis Child 1981;135:273-5.  Back to cited text no. 8
    
9.Hebert AA, Easterly NB. Bacterial and candidal cutaneous infection in the neonate. Dermatol Clin 1986;4:3-21.  Back to cited text no. 9
    
10.Rowel JL, Tate JM. Management of neonatal candidiasis. Ped Inf Dis J 1998;17:1007-11.  Back to cited text no. 10
    


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    Abstract
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