S6C15P9-Darier's Disease

S6C15P9-1: Hypertrophic Darier’s disease is an exaggerated expression of the disease; it is found in association with poor personal hygiene. It is the type of change which may be encountered in afflicted patients in the setting of a mental institution. The characteristic changes are represented in this field. Both the basal unit and the superficial unit are hyperplastic; rete ridges are elongated. The acantholytic cleft is suprabasilar. Characteristically, the acantholysis affects the entire thickness of the epidermis beneath the keratin layer. Parakeratotic debris ( corps grains) accummulates in the defects. Corps ronds are not a prominent feature in this field. The dermis contains infiltrates of lymphocytes and histiocytes. In hypertrophic variants, follicular components are common; they may be associated with a plasmocytosis in the dermal inflammatory infiltrates.

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S6C15P9-2: This is another example of hypertrophic Darier’s disease. The acantholysis above the basal layer has produced a full-thickness defect in the viable portion of the epidermis. Dermal papillae are elongated and broad. There are “parakeratotic” cells in the overlying keratin layer. To the left in the superficial unit of the epidermis, some of the keratinocytes are individually enlarged and rounded in outline. The cytoplasm of these large, round cells is pale peripherally. These cells have large nuclei, marginated chromatin, and a prominent nucleolus; they are coprs ronds.

S6C15P9-3: The pattern in this field is characteristic of  hypertrophic Darier’s disease.The clefts are supra-basilar. In areas, defects extend from the basal layer to the intact keratin layer. The defects contain parakeratotic debris (corps grains). To the right of the center of the field, the granular layer is prominent. Scattered, plump, rounded cells in this area have pulled away from their neighbors; they appear to lie in lacunae. These distinctive cells are corps ronds. The papillary dermis is widened. Perivascular infiltrates of lymphoid cells are present in the upper portion of the dermis. The sebaceous gland lobules on the left are hyperplastic.

S6C15P9-4: At higher magnification, there is marked hypergranulosis. Some of the granular cells have separated from their neighbors; they are angulated and acantholytic (green arrows). The red arrow identifies a striking example of a corps rond.

S6C15P9-5: Hailey-Hailey disease shares many features with the immune-mediated disorders of the pemphigus group. In this example, the epidermis shows parakeratosis, a hyperplastic superficial unit, and suprabasilar acantholysis. The suprabasilar acantholysis affects mostly the keratinocytes of the basal unit. The cleft accentuates the division between the two epidermal units. It is as if cells of the basal unit have entered the phase of terminal differentiation, but have done so being genetically ill-equipped to die in a normal fashion; they keratinize prematurely and, in the process, lose attachments to their neighbors. The orderly sequences leading to the formation of an impervious barrier at the surface are compromised. The papillary dermis is widened, edematous, and inflamed. A follicle in the lesion is little affected by the acantholytic process. In excluding pemphigus, immunofluorescence studies may be required.

 

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