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November 1961

Steatocystoma Multiplex with Pachyonychia Congenita: Eight Cases in Four Generations

Author Affiliations



Dermatology Service, Walter Reed General Hospital, Col. Robert S. Higdon, MC, USA, Chief of Service (Major Vineyard); Chief of Dermatology Service, U.S. Air Force Hospital Keesler, Keesler Air Force Base (Capt. Scott).

Arch Dermatol. 1961;84(5):824-827. doi:10.1001/archderm.1961.01580170118016

Steatocystoma multiplex and pachyonychia congenita are uncommon genodermatoses, judging from the sparcity of reports of either condition in the medical literature.1-6 The observation of a patient in whom both conditions exist and can be traced through 4 generations appears to represent a combination of hereditable dermatoses never before reported. The purpose of this paper is to report 8 cases of these combined disorders and present the results of dermabrasion therapy in the management of one patient.

Report of a Case  A 35-year-old white man came to the dermatology clinic complaining of numerous "bumps on his skin and thick nails." He stated the thickened nails were first noted when he was approximately 7 months of age. His skin was essentially clear until he was 20 years old when numerous yellow, elevated nodules developed on his face. These lesions have progressively increased in size and number and have since appeared over