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June 1947

PACHYONYCHIA CONGENITA: Report of Two Cases, with Studies on Therapy

Author Affiliations


From the Department of Dermatology and Syphilology, Temple University School of Medicine.

Arch Derm Syphilol. 1947;55(6):819-827. doi:10.1001/archderm.1947.01520060081009

PACHYONYCHIA congenita is a rare congenital anomaly, originally described under this name by Jadassohn and Lewandowsky.1 The literature was thoroughly reviewed in 1934 by Diasio,2 who reported that Heller had previously called attention to the close resemblance between the condition reported by Jadassohn and Lewandowsky and the syndrome described under the caption "congenital dyskeratosis" by Erich Schäfer. Schäfer's observations were based on 1 case of his own and 13 culled from the literature. In 1935 Sohrweide3 reported a case under the name "pachyonychia ichthyosiformis."

Ormsby4 in 1943 recognized the title "pachyonychia congenita" and summarized the characteristics as follows:

dystrophic changes in the nails, palmar and plantar hyperkeratosis, anomalies of the hair, leukoplakia, follicular keratoses of the acne-form type particularly about the knees and elbows and dyskeratosis of the cornea. Verrucous lesions are described as occurring on the knees, elbows, popliteal regions, buttocks, legs and ankles Bullae