Back issues No.2 - 1997 / Case Report  

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Mal de Mededa with Incidental Acantholysis
Shumin Young, Shoiu-Hwa Jee, Chun-Hsiang Chang
Dermatol Sinica 15: 87-92, 1997

A-25-year old female patient was diagnosed to have Meleda-type palmoplantar keratoderma (PPK). The disease had its onset when the patient was 20 days old, and had followed a progressive course. At presentation, the patient had so-called "glove-and-sock erythrokeratoderma". Flexion contracture of the right third, fourth, and fifth fingers had become as complication at age 15. The patient's parents were consanguineous and one of her brothers was similarly affected. A biopsy specimen was taken from her right palm. In addition to marked orthohyperkeratosis and psoriasiform acanthosis, histopathological examination also revealed incidental focal acantholysis of the Hailey-Hailey disease pattern involving four of the elongated rete ridges. Ultrastructural study showed a small number of composite keratohyalin granules in the interductal epidermis and widening of the epidermal cell-cell junctions with sloughing of hemidesmosomes.

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