Adrian Florens, MD; Medha Kamat, MD; Tessy Joseph, MD; and Suma Pyati, MD, are with the Division of Neonatal-Perinatal Medicine, John H. Stroger Hospital of Cook County, Chicago. Warren Piette, MD, and Giacomo Maggiolino, MD, are with the Division of Dermatology, John H. Stroger Hospital of Cook County.
Dr. Florens; Dr. Kamat; Dr. Joseph; Dr. Piette; Dr. Maggiolino; and Dr. Pyati have disclosed no relevant financial relationships.
The authors would like to thank Cory Olans for his collaboration with the picture material.
A full-term, 3,700-g male infant was born via vaginal delivery to a 26-year-old gravida 2 para 1001 Hispanic mother, who had good prenatal care. Prenatal laboratory evaluations documented that she was rapid plasma reagin-nonreactive; rubella-immune; HIV negative, hepatitis B surface antigen negative, Neisseria Gonorrhoeae negative; and Chlamydia trachomatis negative. There was no known maternal history of herpes simplex infection, candidal vaginitis, intrauterine device (IUD) usage, cervical cerclage, or amniocentesis. The mother tested positive for group B Streptococcus and received antibiotics before delivery. The mother had artificial rupture of membranes 2 hours before delivery, and there were no signs of chorioamnionitis.
On examination, the infant was vigorous, afebrile, with only mild grunting, although gas exchange was good. On his skin were diffusely distributed, erythematous papules coalescing into plaques with overlying pinpoint vesiculopustules on the face, neck, trunk, and extremities, including palms and soles (see Figure 1, page 553; Figure 2; and Figure 3). The back, diaper area, buttocks, and mucous membranes were spared. The umbilical cord was of normal appearance. There were no signs of hepatosplenomegaly. The remainder of the physical exam was normal.
Figure 1. Erythematous Papules Coalescing into Plaques with Overlying Pinpoint Vesiculopustules in Face, Neck, and Trunk. These Lesions Were Apparent at Birth.
Figure 2. Similar Lesions Are Seen in the Lower Extremities.
Figure 3. Presence of Lesions in Plantar Surfaces. Note that Similar Lesions Were also Seen in Palmar Surfaces.
For diagnosis, see page 553.
Laboratory examination showed a total leukocyte count of 15,000/mm3, 66% neutrophils, 8% bands, 16% lymphocytes, 7% monocytes, and 3% eosinophils. Chest radiograph was normal, and his grunting resolved shortly after admission.
Metabolic profile, liver enzymes, and culture of blood, urine, and CSF were negative for bacteria, virus and fungi. A Wright’s stain result of the smear of the fluid from the vesicles lacked eosinophils. A potassium hydroxide preparation confirmed the diagnosis (see Figure 4).
Figure 4. Potassium Hydroxide Preparation Shows Two Pseudoyphae and Budding Yeast, Yielding the Diagnosis.
Congenital Cutaneous Candidiasis
The infant was treated empirically with ampicillin, gentamicin, and acyclovir for 48 hours. No topical or systemic antifungal therapy was given. The rash gradually resolved over several days, followed by diffuse superficial desquamation of the skin. The infant had no systemic signs of infection. He was discharged home from the hospital on the fourth day of life and had no recurrence of the rash. A potassium hydroxide preparation showed pseudohyphae and budding yeasts, which confirmed the diagnosis of congenital cutaneous candidiasis (CCC).
CCC is a rare condition that results from infection from Candida sp. acquired in utero. The infection is thought to occur by the ascension of organisms from candidial vulvovaginitis, which is present in 20% to 25% of pregnant women.1–3 Risk factors for CCC include a history of maternal candidal vulvovaginitis; intrauterine foreign body (such as an IUD or cervical cerclage); diagnostic amniocentesis; infant prematurity less than 27 weeks gestational age; and extremely low birth weight less than 1,000 g.4,5 There have been no reported associations between either systemic antibiotic or corticosteroid administration and the development of CCC.6 The preterm infant’s immature and compromised mucocutaneous barriers, as well as systemic host defenses, are thought to be predisposing factors.5,6
It is important to recognize that CCC is acquired in utero, whereas neonatal candidiasis is acquired during the passage through an infected birth canal.7
Prenatal infection may range from skin eruption without systemic disease to severe systemic involvement. The infection is characterized by a generalized eruption of various morphologies representing different stages of evolution and includes erythematous macules, papules, vesicles, and pustules. The back, extensor surfaces of extremities and skin folds are usually most involved. Pustules are usually seen on the palms and soles. Nail dystrophy has also been documented. Thrush on the oral mucosa is not commonly seen. The eruption usually presents within the first 6 days of life, although most present at birth.7,8
CCC is diagnosed by presence of spores and pseudohyphae in skin scrapings and culture of the organism from lesions. The differential diagnosis of CCC includes neonatal candidiasis, which differs from CCC by appearing after the first week of life and manifesting as thrush or diaper dermatitis. Some other conditions that may resemble CCC include erythema toxicum neonatorum, transient neonatal pustular melanosis, miliaria, group B streptococcal infection, and herpes simplex/varicella infection. Transient neonatal pustular melanosis and miliaria crystallina present without any erythema. Miliaria rubra does not typically present at birth. Herpes simplex infection would present with more erosive disease and fever. Listeria monocytogenes may also present with a rash at birth and very characteristic white plaques in the umbilical cord (funisitis) and placenta.
In almost all full-term infants, CCC tends to follow a self-limited, benign course. Skin lesions typically resolve within 1 to 2 weeks with desquamation. Neonates less than 27 weeks and less than 1,000 g at birth are at greatest risk for systemic infection and death. Neonates with burn-like lesions are at particular risk for systemic infection, with death occurring in 55% of such neonates weighing less than 1,000 g.7,9,10
It is not known if medical intervention affects outcome, given the benign course of the disease. However, some authors, based on anecdotal experience, have recommended topical and/or systemic therapy. Infants with respiratory distress or signs of systemic infection, especially burn-like lesions, should be treated with systemic antifungal therapy. Amphotericin B has been documented to be the first line of therapy for at-risk infants.7,10
Candida sp. manifests in a variety of ways in humans, from a benign colonizing agent to a major pathogen. It is a well-recognized problem in the high-risk neonate that requires longterm central intravenous lines, so much so that prophylaxis with fluconazole is common practice in the neonatal intensive care unit for the extremely low-birth weight infant. However, we encountered an uncommon presentation of Candida sp. We believe it is important for the pediatric practitioner to consider the possibility of this diagnosis in an otherwise healthy newborn that presents with a rash at birth and to recognize the risk factors that would increase morbidity and mortality and that may require systemic treatment. Most of these infants do not need antifungal treatment; however, those with respiratory distress or other systemic features should receive immediate anti-fungal treatment. Maternal history of intrauterine foreign device may be key to the diagnosis.
- Rudolph N, Tariq AA, Reale MR, Goldberg PK, Kozinn PJ. Congenital cutaneous candidiasis. Arch Dermatol. 1977;113(8):1101–1103. doi:10.1001/archderm.113.8.1101 [CrossRef]
- Hernandez-Machin B, Reyes CS, Vargas MM, Hernando LB. Picture of the month. Arch Pediatr Adolesc Med. 2007;161(9):908.
- Oriel JD, Petridge BM, Denny MJ, Coleman JC. Genital yeast infections. Br Med J. 1972;4(5843):761–764. doi:10.1136/bmj.4.5843.761 [CrossRef]
- Ioppi M, Tapparelli E, Aldovini D. Case of chorioamnionitis due to Candida in a IUD user. Clin Exp Obstet Gynecol. 1981;8:34–37.
- Sinha AK, Hird MF. Congenital cutaneous candidiasis with funisitis. Arch Dis Child. 2005;90(4):425. doi:10.1136/adc.2004.059394 [CrossRef]
- Whyte R, Hussain Z, DeSa D. Antenatal infections with Candida species. Arch Dis Child. 1982;57(7):528–535. doi:10.1136/adc.57.7.528 [CrossRef]
- Darmstadt GL, Dinulos JG, Miller Z. Congenital cutaneous candidiasis: clinical presentation, pathogenesis, and management guidelines. Pediatrics. 2000:105(2):438–444. doi:10.1542/peds.105.2.438 [CrossRef]
- Cosgrove BF, Reeves K, Mullins D, Ford MJ, Ramos-Caro FA. Congenital cutaneous candidiasis associated with respiratory distress and elevation of liver function tests: a case report and review of the literature. J Am Acad Derm. 1997;37(5 Pt 2):817–823. doi:10.1016/S0190-9622(97)80002-3 [CrossRef]
- Baley JE, Silverman RA. Systemic candidiasis: cutaneous manifestations in low birth weight infants. Pediatrics. 1998;82(2):211–215.
- Rowen JL, Tate JM. Management of neonatal candidiasis: Neonatal Candidiasis Study Group. Ped Infect Dis J. 1998;17(11):1007–1011. doi:10.1097/00006454-199811000-00008 [CrossRef]
Each month, this department features a discussion of an unusual diagnosis in genetics, radiology, or dermatology. A description and images are presented, followed by the diagnosis and an explanation of how the diagnosis was determined. As always, your comments are welcome. Please e-mail firstname.lastname@example.org.