Isolated Cutaneous Cryptococcosis in an Otherwise Healthy Girl

Cryptococcus neoformans is a ubiquitous, encapsulated yeast with high prevalence in soil, especially soil contaminated with pigeon excreta.1 Cryptococcosis mainly involves the central nervous system; the most common extraneural site of cryptococcal infection is the skin. Although 10 to 15% of systemic cryptococcal infections become manifested in the skin, isolated cutaneous cryptococcosis in otherwise healthy patients is rare.1 We describe a young, otherwise healthy girl who developed cutaneous cryptococcosis after varicella. Case report. A 6-year-old girl was admitted to Children's Hospital Medical Center, Cincinnati, OH, in January, 1997, with a 1-day history of erythema and swelling of the upper chest extending to both sides of her neck which developed 1 week after chicken pox. No lymph node enlargement was present. She received one dose of intramuscular ceftriaxone, followed by oral amoxicillin/clavulanate, for the diagnosis of cellulitis. After 5 days of treatment an intense maculopapular rash on her upper chest and neck and an abscess developed. The chest wall abscess was drained, and the antimicrobial regimen was changed to intravenous nafcillin and gentamicin. The abscess material was purulent with fibrinous debris. Bilateral neck incisions revealed two other small abscess cavities in the left suprasternal region along the anterior border of right sternocleidomastoid muscle connecting to a cavity in the presternal region. Hemoglobin, hematocrit and white blood cell values were 12.5 g/dl, 37.9% and 8800/mm3, respectively, with a differential of 28% polymorphonuclear leukocytes, 57% lymphocytes, 7% monocytes, 5% eosinophils and 3% atypical lymphocytes. Platelet count was 379 000/mm3 and erythrocyte sedimentation rate was 18 mm/h. Chest roentgenogram at initial presentation revealed only soft tissue swelling of the upper chest on lateral view. No hilar adenopathy was evident. Blood culture was negative. Cryptococcus neoformans was isolated from the chest wall abscess material after 6 days of incubation in anaerobic culture. Oral fluconazole was begun. Physical examination was normal except for the incision and drainage site wound, which was healing well when the patient was referred to our clinic the next day. Continuation of oral fluconazole was recommended but was actually discontinued after 7 days by the family. The serum immunoglobulin profile was normal as were follow-up complete blood counts. At follow-up 6 months later she had no evidence of systemic disease. The lesions were healed with no local recurrence. Comment. To our knowledge this is the youngest otherwise healthy patient with localized cutaneous cryptococcosis who has been reported.2 Predisposing factors for cryptococcal infections are chronic diseases with cellular immune system dysfunction and lymphoreticular malignancies such as Hodgkin's lymphoma, leukemia, steroid and immunosuppressive therapy, transplantation, hepatitis, sarcoidosis and HIV infection.2, 3 Inoculation of cryptococci into the skin by trauma has also been reported, but the role of the breakdown of cutaneous immunologic barriers, as occurs in varicella infection, has not been investigated as an etiologic or triggering factor.4 A variety of lesions have been described in association with cryptococcal skin infection, including subcutaneous nodules, ulcers, panniculitis, vasculitis, herpetiform lesions, acneiform lesions, palpable purpura, pyoderma gangrenosum-like ulcers, molluscum contagiosum-like lesions and cellulitis as in our patient.5 Many of these lesions have been described in association with an underlying disease or immune dysfunction. Few cases without evidence of systemic involvement have been reported in the literature, and there remains a debate whether primary cutaneous cryptococcosis really exists or is a sentinel of the disseminated disease.6, 7 We hypothesize that the preceding varicella infection provided a portal of entry of the cryptococcal infection in an otherwise healthy host. It is likely that debridement provided the principal therapy. History of varicella lesions in the area where the cellulitis appeared, their limitation to the skin, no evidence of disseminated disease during the follow-up period and healing of the lesions without prolonged antifungal treatment suggest that pathogenesis was local inoculation of C. neoformans. Some authors recommended long term systemic antifungal treatment of localized skin infections to prevent systemic dissemination.8, 9 Avoiding drug treatment in the absence of signs of systemic disease may be warranted in selected cases. The clinical improvement of our patient after 1 week of oral fluconazole treatment with no recurrences underlines the need for further data for the necessity of long term systemic treatment in otherwise healthy hosts with isolated cryptococcal infection. Guliz Erdem, M.D.* Beverly L. Connelly, M.D.