Primary Cutaneous Zygomycosis in Two Immunocompromised Children

Abstract

Zygomycosis, often referred to as ''mucormycosis'' or ''phycomycosis,'' is a rapidly progressive fungal infection which usually occurs in immunocompromised individuals, and is characterized by soft tissue destruction and invasion of blood vessels. The rare and easily misdiagnosed primary cutaneous form may present as a superficial erosion with a painless, gradual onset and slow progression of symptoms or a gangrenous, necrotic ulceration due to rapid tissue and vascular invasion. With the latter form, the mortality rate among affected individuals is high even after aggressive surgical debridement and amphotericin B administration, emphasizing the importance of early recognition and proper diagnosis. We present two instances of gangrenous cutaneous zygomycosis in immunocompromised children and review the literature with regard to etiology, diagnosis and treatment, highlighting the pediatric population.

Deep cutaneous fungal infections in immunocompromised children

“…Zygomycoses are rapidly progressing fungal infections and hematogenous dissemination can occur in 70% of cases even after aggressive surgical and systemic antifungal treatment. 32 Deep cutaneous Rhizopus infection was observed in a 14-year-old girl with acute nonlymphoblastic leukemia in the context of severe neutropenia. She survived the infection, but died 4 months later because of the underlying disease.…”

Clinical presentation of zygomycosis

“…, haematogenous dissemination to the skin was rare [43]. On the other hand, primary cutaneous zygomycosis is associated with traumatic inoculation of the skin with zygomycetes in immunocompromised patients, burn victims, and patients with severe soft tissue trauma [16,17,35,50–61]. The clinical symptoms and signs of erythema and induration are non‐specific and indistinguishable from those of any cutaneous or subcutaneous infection.…”

Long-term posaconazole treatment and follow-up of rhino-orbital-cerebral mucormycosis in a diabetic girl

“…The mortality of invasive mucormycosis with standard therapy with amphotericin B in adults is about 40% (24) and in the rare pediatric clinical reports is high (25, 26). The efficacy of POS in pediatric mucormycosis is demonstrated in several reports (11, 13–15, 17, 19–22, 27). The 2‐yr clinical follow‐up of our patient strongly suggests that long‐term therapy with POS is safe and also beneficial in treatment and prevention of relapses of, otherwise fatal, CNS mucormycosis.…”

“…Data on POS efficacy and toxicity in children are still very sparse. In the literature, a few successful pediatric cases are reported, with different localization of the mucormycoses: four cutaneous (13–15), four pulmonary (16, 17), three rhino‐orbital, two with cerebral dissemination (18–20), and one cardiac (21).…”