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GIORNALE ITALIANO DI DERMATOLOGIA E VENEREOLOGIA

A Journal on Dermatology and Sexually Transmitted Diseases


Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
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Giornale Italiano di Dermatologia e Venereologia 1999 October;134(5):477-80

language: Italian

Pachyonychia congenita. Report of 4 cases

Tosti A., Ghetti E., Piraccini B. M., Fanti P. A.

Università degli Studi - Bologna, Dipartimento di Medicina Clinica Specialistica e Sperimentale - Sezione di Clinica Dermatologica


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From 1994 to 1997, four patients, 2 males and 2 females, ranging in age from 13 to 50 years, were referred to our Department because of dystrophic lesions in twenty nails, palmar and plantar hyperkeratosis, leukokeratosis of the tongue and mucous membranes and follicular hyperkeratosis.
Detailed questioning of the patients revealed that, in all of them, lesions appeared at birth or in early childhood and nail disease arised symmetrically and before the other anomalies.
Patient’s history as well as the clinical and histological features suggested the diagnosis of pachyonychia congenita syndrome (PCS).

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