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Article
August 1934

PACHYONYCHIA CONGENITA JADASSOHN: A VARIETY OF ICHTHYOSIS (PACHYONYCHIA ICHTHYOSIFORMIS) INVOLVING CHIEFLY THE NAILS

Author Affiliations

Dermatologist and Syphilologist, Columbus and Saint Elizabeth's Hospitals, and Associate Dermatologist, Lincoln Hospital NEW YORK

From the Department of Dermatology and Syphilology, Columbus Hospital.

Arch Derm Syphilol. 1934;30(2):218-226. doi:10.1001/archderm.1934.01460140044007
Abstract

Under the caption "Congenital Dyskeratosis," Erich Schäfer1 described a syndrome based on observations gathered from one case of his own and from thirteen others reported in the literature. The condition is characterized by: pachyonychia; acneform, follicular keratosis, especially about the knees and elbows; symmetrical and circumscribed plantar and palmar keratosis; hyperhidrosis of the palms and soles; leukoplakia of the oral mucosa, and anomalies of the hair. All the fourteen cases showed pachyonychial changes as a constant finding, while the remaining clinical features were variants.

In Schäfer's case a boy, 12 years old, had had the characteristic ungual manifestations from birth. Brettauer's report2 dealt with a boy 9 years old. The finger-nails were thickened, opaque, lusterless, partially loosened and in places swollen and lap-rolled in the longitudinal axis of the finger. According to Heller,1e Jadassohn and Lewandowsky's case3 of pachyonychia congenita belongs in this group. Siemens4 likewise

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