Pachyonychia congenita is a rare hereditary disorder characterized mainly by nail hypertrophy and dyskeratoses of skin and mucous membranes. A thorough literature survey since its first description in 1904 up to 1985 revealed 168 cases of pachyonychia congenita. There were no indications of any sex or ethnic group predilection. Based on this survey the following classification is suggested: type I (56.2% of cases), hyperkeratosis of nails, palmoplantar keratosis, follicular keratosis, and oral leukokeratosis; type II (24.9% of cases), clinical findings of type I plus bullae of palms and soles, palmar and plantar hyperhidrosis, natal or neonatal teeth, and steatocystoma multiplex; type III (11.7% of cases), clinical findings of types I and II plus angular cheilosis, corneal dyskeratosis, and cataracts; and type IV (7.2% of cases), clinical findings of types I, II, and III plus laryngeal lesions, hoarseness, mental retardation, hair anomalies, and alopecia.

SUMMARY.— Three hundred and sixty‐six patients with ichthyosis vulgaris were examined, including 229 with X‐linked recessive ichthyosis (X.L.I.) and 137 with autosomal dominant ichthyosis (A.D.I.), among the Jewish population of Israel. The minimal incidence of the disease in this population is about 1:6500. The disease affected the various ethnic groups differently; among Iraqi Jews there were cases only of X.L.I, compared with only A.D.I, among Indian Jews. The disease appeared in both forms in 95% of cases before 5 years of age, and half of the cases showed improvement in the course of time; 80%‐95% of the patients noted improvement in the summer months. A family history of “atopy” was found only in about a third of cases of A.D.I, and in none of those with X.L.I. The most pronounced clinical features of the disease were found over the anterior aspect of the legs, and equally in both forms. Follicular keratosis of the arms and hyperkeratosis with prominent creases of the palms and soles were found only in A.D.I. The histological findings were diagnostic, especially in those areas where the scaling was obvious, and thus it is recommended to biopsy the anterior shin region. The histology in X.L.I, showed compact laminated hyperkeratosis, with thickening of the granular layer; whereas, in A.D.I., the hyperkeratosis was usually of basket‐weave type, with a thin or absent granular layer. The sweat glands were normal in structure and number in both forms, while the sebaceous glands were absent only in cases of A.D.I.

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A family in which two sisters are affected by pemphigus is described. Literature research has revealed the presence of 25 families, comprising 53 patients, in which pemphigus existed in more than one member. The relationships between those affected were mainly parent-child or sibling-sibling. Pemphigus was found to be associated with specific HLA antigens: A26, Bw38, DRw4, and additionally occurred with a higher frequency in certain ethnic groups. The variety of etiologic factors in the pathogenesis of pemphigus is discussed and the significance of a genetic role elucidated.