S6C15aP9a-Hailey-Hailey  Disease

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S6C15aP9a-1: The patterns of acantholysis, affecting the basal unit of the epidermis, and the location of the cleft at the interface between the basal and the superficial units of the epidermis, produce a classic pattern for a lesion of Hailey-Hailey disease. Dyskeratosis is a prominent feature. Dermal papillae are elongated; some are cut in cross-section. The papillae cut in cross-section appear to be free within the defects.

S6C15aP9a-2: In this field of the same lesion as seen in S6C15aP9a-1, dyskeratotic changes are present in both the basal and the superficial units of the epidermis. Some of the dyskeratotic cells in the superficial unit resemble the “corps ronds” of Darier disease. The differential diagnosis would include Darier disease.

S6C15aP9a-3: In this field, the cytologic features of the area to the left of the center of the field resemble those of a lesion of Darier disease (corps ronds, lacking a prominent component of keratohyaline granules, are a prominent feature) (Hailey-Hailey disease).

 

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