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November 1985

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

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)