ABSTRACT. A 1-year-old girl presented with acute onset of edematous erythematous plaques associated with bullae on her extremities and accompanied by peripheral eosinophilia. She was afebrile, and the skin lesions were pruritic but not tender. The patient was treated with intravenously administered antibiotics for presumed cellulitis, without improvement. However, the lesions responded rapidly to systemic steroid therapy. On the basis of lesional morphologic features, peripheral eosinophilia, and cutaneous histopathologic features, a diagnosis of Wells' syndrome was made. Wells' syndrome is extremely rare in childhood, with 27 pediatric cases reported in the literature. Because it is seen so infrequently, there are no specific guidelines for evaluation and management of Wells' syndrome among children. W ells' syndrome, or eosinophilic cellulitis, is a rare, recurrent, inflammatory dermatosis of unknown pathogenesis. In 1971, Wells 1 described 4 patients with an acute pruritic dermatitis clinically resembling bacterial cellulitis but with histopathologic findings characterized by dermal eosinophilia, phagocytic histiocytes, and the presence of flame figures. He initially called this syndrome recurrent granulomatous dermatitis with eosinophilia but later simplified the name to eosinophilic cellulitis. 2 Wells' syndrome is seen more commonly among adults but has been observed among children. Some hypothesize that this syndrome may represent a hypersensitivity response to a circulating antigen. 2 Associated precipitants include insect bites, medication reactions, recent immunization, myeloproliferative disorders, malignancies, and infections. We describe a case of a young child with no identifiable triggering factors, and we review the evidence for evaluation and management of these pediatric cases. CASE REPORTA previously healthy, 1-year-old girl presented with acute onset of edematous erythematous plaques, with associated bullae, on her lower extremities and left arm (Fig 1). These lesions were pruritic but not painful, and the patient was afebrile. Her parents denied a history of insect bites, ingestion of medications, trauma, or other intercurrent illness. The patient's most recent immunizations had been received 3 months earlier. The patient did not have a history of asthma, and there was no family history of asthma or atopic disease.The patient was admitted with presumed bacterial cellulitis and was treated with intravenously administered oxacillin, without improvement. Her laboratory studies were significant for an elevated white blood cell count of 30 ϫ 10 9 cells per L, with peripheral eosinophilia of 48%. After the patient failed to respond to systemically administered antibiotics, examination of vesicle fluid was performed and revealed numerous eosinophils. Subsequently, the diagnosis of probable Wells' syndrome was made. Oral steroid therapy was started at 2 mg/kg, and the patient's cutaneous symptoms improved within 24 hours, leaving residual erythema and hyperpigmentation (Fig 2). Five days after the i...

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Keratitis, ichthyosis, and deafness (KID) syndrome is a rare congenital disorder of unknown etiology in which increased susceptibility to viral, bacterial, and mycotic infections has been observed. We report an infant with KID syndrome who died from overwhelming systemic infection. To date, investigations into the immune function of patients with this syndrome have not revealed a common underlying systemic immune deficit. However, the severity of infections and multiplicity of organisms observed in this syndrome suggest that a primary immunodeficiency is present in addition to an impaired cutaneous barrier to microorganisms.