Cases reported "Acantholysis"

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1/7. A localized variant of paraneoplastic pemphigus: acantholysis associated with malignant melanoma.

    We report a 72-year-old male patient with a nodular malignant melanoma that was associated with focal suprabasal acantholysis (FSA). This phenomenon, which is regarded as an incidental finding by dermatopathologists, may be associated with inflammatory and also neoplastic skin diseases. Haematoxylin and eosin stained sections from an erythematous plaque surrounding the patient's tumour showed FSA, direct immunofluorescence (DIF) and indirect immunofluorescence (IIF) on normal human skin, monkey oesophagus and rat urinary bladder were negative. On electron microscopy few desmosomes could be detected in the basal cell layer of the acantholytic areas and there was a nearly complete loss of these structures in the spinous cell layer. Only remnants of cytoplasmic plaques and keratin filaments could be observed in those areas. In contrast, adherens junctions appeared to be well preserved. An enzyme-linked immunosorbent assay (ELISA) using recombinant fusion proteins as antigens did not show circulating autoantibodies against desmoglein 1 (Dsg1) or desmoglein 3 (Dsg3). In contrast, immunoblotting revealed autoantibodies directed against keratinocyte antigens with a molecular weight of 85 kDa and 250 kDa, the first band corresponding to the molecular weight of comigrating plakoglobin. immunoprecipitation with patient serum also revealed a 85-kDa band. We conclude that these autoantibodies, probably in conjunction with cofactors produced by the tumour, could play a part in the pathogenesis of this variant of FSA, for which we propose the term 'localized paraneoplastic pemphigus.'
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2/7. Kaposi's varicelliform eruption in a patient with Grover's disease.

    Kaposi's varicelliform eruption is a secondary herpes simplex virus infection that affects patients in the setting of primary dermatologic conditions. It occurs with a variety of skin diseases, although association with Grover's disease has never been reported in the literature. This report describes the manifestations and clinical course. A review of the literature on Kaposi's varicelliform eruption includes disease associations, pathogenesis, and treatment.
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3/7. Papular acantholytic dyskeratosis of the vulva.

    We describe an 11-year-old girl with a persistent pruritic papular eruption on the vulva. Clinically, the lesions consisted of whitish papules and erosions located on the inner aspect of the labia majora. There was no familial history of skin diseases. Histologically, a biopsy specimen showed difuse hyperkeratosis, parakeratosis, acantholysis throughout the thickness of the epidermis, and the presence of corps ronds. Those findings were consistent with a diagnosis of acantholytic dyskeratosis. At 3 years follow-up, only isolated hyperkeratotic, asymptomatic papules on the same location remained. The occurrence of this focal and sporadic, localized form of acantholytic dyskeratosis seems to be rare in the pediatric population, as we could find no other child with this entity reported in the literature.
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4/7. Familial dyskeratotic comedones. A report of three cases and review of the literature.

    Three family members, at initial evaluation, had generalized comedonal lesions with histologic changes of acantholysis and dyskeratosis. A total of nine cases of this entity, termed familial dyskeratotic comedones, have been documented in the literature. It appears to have autosomal dominant inheritance and onset in childhood or adolescence. Lesions are asymptomatic except for occasional pruritus or inflammation, and general health is undisturbed. A history of acne vulgaris is seen in four of nine patients and is the only associated skin disease. Treatment with oral isotretinoin produced no improvement in two patients. Electron microscopy revealed changes similar to those seen in Darier's disease.
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5/7. Relapsing linear acantholytic dermatosis.

    A 5-year-old girl with a unilateral linear systematized skin lesion showing the clinical, histological and ultrastructural features of Hailey-Hailey disease is described. We suggest that this relapsing linear acantholytic dermatosis represents a new distinct entity within the spectrum of unilateral linear skin diseases.
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6/7. Invisible dermatoses.

    An invisible dermatosis is defined as a skin disease manifesting no clinically apparent lesion but histologic changes of a characteristic nature. An example of an invisible form of transient acantholytic dermatosis is presented. Invisible forms of pseudoxanthoma elasticum, sarcoidosis, lepromatous leprosy, and lichen planus are reviewed. Dermal deposits may also be found on biopsy of clinically normal skin in amyloidosis, Hunter's and Hurler's syndromes, and thyroid disease.
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7/7. Acantholytic dyskeratotic epidermal naevus localized unilaterally in the cutaneous and genital areas.

    A 38-year-old woman presented with unilateral lesions on the left side of the body and in the genital area. Clinically, the lesions showed a polymorphic pattern: brownish papules in the axilla, keratotic comedo-like papules on the hand and foot, and whitish papular plaques on the labia majora and anal canal. There was no family history of skin diseases. Histologically, cutaneous and mucosal specimens were characterized by acantholytic and dyskeratotic cells, corps ronds and grains in the parakeratotic zone, and by hyperkeratosis and parakeratosis. A diagnosis of epidermal naevus with acantholytic dyskeratosis was made.
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