CaNe r e D o r | s Congenital cutaneous candidiasis T h o m a s A. Chapel, M . D . , Carl Gagliardi, M . D . , and Wallace Nichols, M.D. Detroit a n d Dearborn, MI We report a case of congenital cutaneous candidiasis and review the clinical features and probable pathogenesis of this rarely recognized and infrequently reported condition. The symptoms, physical findings, laboratory results, and risk factors that influence prognosis and choice of therapy are discussed. (J AM ACAD DERMATOL 6:926-928, 1982.)
Cutaneous candidiasis in the n e w b o r n exists in a congenital f o r m with skin lesions present at birth, or shortly thereafter, and in a neonatal form that is c o m m o n l y seen after the first w e e k o f life. Neonatal candidal infection is manifested most often in the f o r m of oral thrush and less often as lesions confined to the diaper area. The infection is presumed to be acquired b y the infant during passage through the m o t h e r ' s infected birth canal. On the other hand, a generalized candidal dermatitis at birth implies an intrauterine infection, and there have been few authentic reports o f this condition. This paper adds an additional case, and it discusses the clinical and laboratory features of congenital cutaneous candidiasis that permit its differentiation f r o m other papular and vesicopustular dermatoses occurring during the first few days o f life.
CASE REPORT A 3,270-gm full-term female infant was born to a 20-year-old primipara by normal spontaneous delivery from a breech presentation. Her membranes had ruptured 8 hours prior to delivery. The mother had a history of recurrent candidal vaginitis, with the last episode having occurred 6 months bet'ore her pregnancy. Fromthe Departmentof Dermatologyand Syphilology,WayneState University, Detroit, and the Department of Pediatrics, Oakwood Hospital, Dearborn. Reprint requests to: Dr. Thomas A. Chapel, Department of Dermatology and Syphilology, Wayne State University School of Medicine, 540 E. Canfield, Detroit, MI 48201.
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The infant's Apgar score was 8 and 8 at 1 and 5 minutes, respectively. At birth, the infant was active and in no distress. Abnormal physical findings consisted of scattered erythematous papules and vesicopustules on the cheeks, trunk, buttocks, and legs. Forty-eight hours after delivery, vesicles and pustules appeared on the palms, soles, and scalp, (Figs. I and 2), and at day 6 the patient developed a diaper dermatitis. The infant was afebrile throughout and manifested no constitutional signs of systemic disease. The mother's postpartum course was uneventful. Candida albicans was identified by culture of the mother's vaginal secretions. The infant was blood type group O, Rh-positive, and the direct antiglobulin test was reactive +4. The mother was blood type group A, Rh-negative, and an antibody screen was positive with the antibody identified as anti-D. The direct bilirubin value was 0.5 mg/ dl, and bilirubin total, 2.6 mg/dl on the day of birth. These values increased to 0.7 mg/dl and 10.9 mg/dl by day 3. The hemoglobin level was 22.0 gm. At 1 and 7 days of age, a direct smear from the pustules showed budding yeast forms that were identified as C. albicans by culture. Treatment consisted of local applications of 2% miconazole nitrate cream, applied three times daily for l0 days, and 200,000 U of nystatin oral suspension every 6 hours for 1 week. The eruption cleared after 1 week of treatment. The sites of heaviest involvement had transient postinflammatory desquamation. COMMENT Vaginal candidiasis has been estimated to occur in 20% to 25% of all pregnant w o m e n . 1-3 Kozinn et al 1 recovered Candida in 50% o f the infants born to such mothers, and it is presumed that the 0190-9622/82/050926+03500.30/0 © 1982 Am Acad Dermatol
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Fig. 1. Congenital cutaneous candidiasis of the palm. Fig. 2. Congenital cutaneous candidiasis of the scalp.
infant becomes infected during passage through the infected vagina. Neonatal infections with Candida appear after the first week of life and usually present as oral thrush or, less commonly, perianal lesions. C. albicans frequently can be isolated from the stool of these patients, and contaminated feces is thought to be the source of infection in candidal diaper dermatitis. Congenital cutaneous candidiasis is distinctly different from neonatal cutaneous candidiasis. In most cases the eruption is noted at birth or within 12 hours after delivery, although in two cases with evidence of intrauterine infection of the fetal cutaneum the eruption appeared at 4 and 6 days of age. 4 Lesions of congenital cutaneous candidiasis are scattered diffusely over the trunk, neck, and head, but the nails, palms, and soles also have been involved. 5-:~ Oral lesions usually are absent, and the diaper area is spared. Intertriginous areas, the back, and extensor surfaces of the extremities may be severely affected. Individual lesions begin as intensely erythematous macules and papulovesicles that become pustular and dry within 4 to 7 days, producing a prominent postinflammatory desquamation. Stool cultures at birth are sterile and oral lesions are usually not observed, although oral and throat cultures may grow C. albiccms. ~ Infants with candidal infections that are acquired prior to delivery can have either local or
systemic disease. Infections limited to the skin or the placenta and/or umbiiical cord usually have favorable outcomes. However, disseminated congenital candidal infection, a condition that usually presents without cutaneous lesions, can lead to intrauterine death or death in the immediate neonatal period. The most reliable indicators for predicting the extent and outcome of congenital candidiasis are the infant's birth weight and the symptomatology.t° Infants with birth weights less than 1,500 gm and infants with early onset of severe respiratory symptoms have all died with autopsy-proved disseminated candidiasis or candidal pneumonia. Most newborns with cutaneous candidiasis have an absence of constitutional signs and localized benign disease, but fatal outcomes have been reported in infected neonates with low birth weight and neonates with respiratory distress. The white blood cell and platelet counts in benign cases have been normal, as have the total lymphocyte count and lymphocyte stimulation studies with C. albicans and phytohemagglutinin. Negative blood cultures and the absence of precipitating antibodies to C. albicans suggest that there is no hematogenous spread; however, Candida has been cultured from the urine by suprapubic puncture, suggesting that asymptomatic systemic dissemination of the organism may occurJ ~ Johnson et al 1~ advocated repeated cultures of
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the urine, cerebrospinal fluid (CSF), and blood of patients with congenital candidiasis to determine if candidemia or systemic involvement is present. Transient benign candidemia or candiduria may occur, but consistently positive blood, urine, or CSF cultures indicate disseminated disease and the need for systemic antifungal therapy. Despite spontaneous involution of lesions in most cases of benign congenital cutaneous candidiasis, Johnson and colleauges TM feel that treatment with topical and oral preparations of nystatin lowers the risk of systemic invasion by decreasing the number of organisms on the skin and the gastrointestinal tract. Congenital cutaneous candidiasis does not appear to be related to maternal age, parity, type of delivery, duration o f labor, or gestational age. Moreover, in contrast with neonatal bacterial infection, congenital cutaneous candidiasis is not related to duration of rupture of membranes. Lesions appear 36 to 72 hours after experimental infection of the skin with C. albicans. H Therefore, cutaneous candidiasis present at birth indicates that intrauterine infection must have occurred within the week prior to labor. Intrapartum infection of the skin by inoculation from an infected vagina or infection transmitted by infected nursery personnel results in the appearance of an eruption at a later age. Flamm ~ demonstrated that Candida cannot pass the placental barrier; therefore, ascending infection is believed to be the route of invasion in cases of congenital cutaneous candidiasis. Subclinical, self-healing ruptures in amniotic membranes may facilitate passage of C. albicans into the amniotic sac. However, Gotz et aP a showed that C. albicans can infect and penetrate through chick chorioallantoic membrane and kill the chick embryo. Dvorak and Gavaller14 reported congenital candidiasis in an infant born by cesarean section without rupture of membranes, suggesting that the organisms also can penetrate intact membranes in humans. Amniotic fluid supports the growth ofC. albicans in vitro, and the organism in the infected amniotic fluid can invade the fetal adnexa, placenta, or umbilical cord. 7
Congenital cutaneous candidiasis is an infrequently reported condition that is probably more common than the literature would suggest. Conditions that enter into a differential diagnosis of congenital cutaneous candidiasis include neonatal bacterial infections, particularly superficial staphylococcal pustulosis, transient neonatal pustular melanosis, and erythema toxicum. However, the clinical features of congenital cutaneous candidiasis, coupled with the presence of spores and pseudohyphae in scrapings of skin lesions, permit easy differentiation of this condition from these other dermatoses that occur during the first few days of life. REFERENCES
1. KozinnPJ, Taschdjian CL, Dragutsky D, et al: Cutaneous candidiasis in early infancy and childhood. Pediatrics 20:827-834, 1957, 2. Nagesha EN, Ananthakrishna NC: Clinical and laboratory study of monilial vaginitis. Am J Obstet Gynecol 107:1267-1268, 1970. 3. Oriel JD, Patridge BM, Denny MJ, et al: Genital yeast infections. Br Med J 4:761-764, 1972. 4. SchirarA, Rendu C, VielhJP, et al: Congenitalmycosis. Biol Neonate 24:273-278, 1974. 5. Kam LA, GiacoiaGP: Congenitalcutaneous candidiasis. Am J Dis Child 129:1215-1218, 1975. 6. RudolphN, Tariq AA, Reale MR, et al: Congenital cutaneous candidiasis.Arch Dermatol 113:1101-1103, 1977. 7. SonnenscheinH, Taschdjian CL, Clark IlL: Congenital cutaneous candidiasis. Am J Dis Child 107:260-266, 1964. 8. SonnenscheinH, Clark HL, Taschdjian CL: Congenital cutaneous candidiasis in a premature infant. Am J Dis Child 99:81-85, 1960. 9. Rhatigan RM: Congenitalcutaneous candidiasis. Am J Dis Child 116:545-546, 1968. 10. JohnsonDE, ThompsonTR, Ferrieri P: Congenital candidiasis. Am J Dis Child 135:273-275, 1981. 11. MaibachHI, KligmanAM: The biologyof experimental human cutaneous moniliasis (Candida albicans). Arch Dermatol 85:113-137, 1962. 12. Flamm H: Die pranatalen infektionen des menscher. Stuttgart, Germany, 1959, George Thieme, p. 70. 13. Gotz H, Nasemann T, Sturde HC: Weitere untersuchungen fiber die praktische verwendung des chorionallantoistestszur hefedifferenzierung.Arch Klin Exp Dermatol 203:582-597, 1956. 14. DvorakAM, Gavaller B: Congenitalsystemic candidiasis. N Engl J Med 274:540-543, 1966.