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Severe combined immunodeficiency (SCID) is a heterogeneous group of disorders characterized by a defect of T and B cell immunity with a genetic origin in most cases. Although the X-linked recessive form is most common (60-70%), there are autosomal recessive forms (20%) and spontaneous mutations. While SCID may present with many nosocomial infections, dermatophyte infections are not common. We reported a case of SCID which was associated with a widespread skin infection with Trichophyton mentagrophytes.

Granulomatous slack skin syndrome is a rare clinical and pathologic disorder. Only 42 patients have been reported, one of whom we described in 1997--the only child so far reported. We now describe the evolution of this patient and the transformation of the disease into a peripheral T-cell lymphoma, and the complications resulting in the child's death.