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July 1959

Hyperkeratosis Ichthyosiformis Congenita: Association with Keratosis Areola Mammae Neviformis, Low-Grade Feeblemindedness, Bone Deformities, and Pseudopelade

Author Affiliations

Belgrade, Yugoslavia

From the Department of Dermatology and Venereology, University of Belgrade (Head, Prof. S. Ilic, M.D.).

AMA Arch Derm. 1959;80(1):56-58. doi:10.1001/archderm.1959.01560190058008

The skin condition hyperkeratosis ichthyosiformis congenita (H. I. C.), in the greatest number of cases inherited as a recessive, has been also reported in a few instances as being associated with some other anomalies.

Review of Literature

The direct relationship between H. I. C. and oligophrenia was first suggested by Heinrichs.1 This problem was later discussed by Bredmose2 and recently fully investigated by Sjögren and Larsson.3 In an extensive study from Sweden (with only three exceptions the parents of all patients were born in the county of Vasterbotten), they found a frequent association of oligophrenia and spastic disorders with H. I. C. Spastic disorders were mainly manifested as Little's disease. Eye changes manifested as degeneration of the pigment epithelium of the macula and its surroundings were diagnosed in the same group in three cases.

The association of some other psychological abnormalities, such as

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