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April 1976

Cornifying Darier Disease— A Unique Variant: I. Report of a Case

Author Affiliations

From the Dermatology Branch (Drs Peck, Kraemer, Wetzel, and Klingler) and the Surgery Branch (Dr Cohen), National Cancer Institute, Bethesda, Md. Dr Kraemer is currently with the Department of Dermatology, University of Miami School of Medicine, Miami, Fla. Dr Klingler is with the Dermatology Department, University of North Carolina School of Medicine, Chapel Hill, NC. Dr Cohen is with the Division of Plastic Surgery, Medical College of Virginia, Richmond, Va.

Arch Dermatol. 1976;112(4):495-503. doi:10.1001/archderm.1976.01630280023006

• A unique variant of Darier disease is described in which a patient was disabled by large, painful, cutaneous horns present on all extremities. The cornified lesions were distinguished by the presence of numerous corps ronds in the basal portion of the greatly hyperkeratotic stratum corneum, hypertrophic dermal villi containing enlarged capillaries, vacuolar dilatation of rough endoplasmic reticulum in sublacunar basal cells, unusually numerous Odland bodies in spinous cells adjacent to lacunae, and persistent attachment of tonofilaments to disrupted desmosomes. Complete separation of tonofilaments from intact desmosomes was not observed. Scanning electron microscopy revealed varied surface morphological appearances of corps ronds and of the epidermal cells covering the elongated dermal villi. The surface cells of cutaneous horns showed little tendency to desquamate.

(Arch Dermatol 112:495-503, 1976)

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