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October 1996

Idiopathic CD4+ T Lymphocytopenia Presenting as Mycetoma in a Patient With a Mutation in the Cystic Fibrosis Transmembrane Regulator Gene

Author Affiliations

Department of Dermatology University of Frankfurt am Main Theodor-Stern-Kai 7 D-60590 Frankfurt am Main, Germany

Ulm, Germany

Berlin, Germany

Arch Dermatol. 1996;132(10):1247-1249. doi:10.1001/archderm.1996.03890340113023

Since 1989, an increasing number of patients with unexplained CD4+ T-lymphocyte depletion, but without evident human immunodeficiency virus (HIV) infection, have been described. This condition is now defined as idiopathic CD4+ T lymphocytopenia (ICL).1,2

Report of a Case.  We describe a 73-year-old man suffering from sporotrichoid nodules on his right lower leg (Figure 1). Five of his 7 children died in their early childhood of cystic fibrosis. The patient and all other relatives were free of symptoms of cystic fibrosis. Analysis of the cystic fibrosis transmembrane regulator (CFTR) gene revealed the mutation Δ F508 in the patient and 3 of 8 analyzed relatives, and another less common mutation was found in the patient's wife.3Histological examination of a nodule showed a subcutaneous suppurative granulomatous dermatitis. Periodic acid—Schiff staining revealed fungal elements in hyphal form (Figure 2). Microbiological cultivation of biopsy specimens yielded growth of Exophiala jeanselmei and

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