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November 1985

Dyskeratosis Congenita in a Girl Simulating Chronic Graft-vs-Host Disease

Author Affiliations

From the Department of Internal Medicine, Division of Dermatology (Drs Ling, Fenske, and Espinoza) and the Department of Pathology (Drs Fenske and Espinoza), University of South Florida College of Medicine and James A. Haley Veterans Hospital, Tampa, Fla; the Department of Pediatrics, University of South Florida College of Medicine, Tampa (Dr Julius); and the Department of Dermatology, Emory University School of Medicine, Atlanta (Dr Drake).

Arch Dermatol. 1985;121(11):1424-1428. doi:10.1001/archderm.1985.01660110072019

• Dyskeratosis congenita (DCG) is a rare genodermatosis characterized primarily by reticular hyperpigmentation of the skin, dystrophy of the nails, and leukoplakia. It is frequently associated with Fanconi-type pancytopenia. Although DCG has a male predisposition, it has been reported in several female patients. We encountered a case of DCG occurring in a girl whose clinical features simulated chronic graft-vs-host disease (GVHD). Because DCG and chronic GVHD share several clinical and histologic features, physicians should always examine a patient for possible DCG whenever a diagnosis of chronic GVHD is considered. In addition, the similar manifestations of the two disorders suggest a similar pathogenesis on a cellular level in the immunologic system.

(Arch Dermatol 1985;121:1424-1428)