Cases reported "Acantholysis"

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1/23. Generalized pruritic eruption with suprabasal acantholysis preceeding the development of bullous pemphigoid.

    We report a patient who presented with a papular pruritic eruption of a 3-month duration that histologically showed suprabasal acantholysis accompanied of an eosinophilic inflammatory infiltrate that was consistent with the diagnosis of Grover's disease. Later, erythematous plaques and vesicles appeared which showed a histopathological pattern of eosinophilic spongiosis. The direct immunofluorescence (DIF) study showed lineal IgG and C'3 at the dermal epidermal junction which was consistent with the diagnosis of bullous pemphigoid. No anti-intercellular deposits of immunoglobulin g (IgG) or C'3 were observed. We consider that suprabasal acantholysis may represent the early phase of bullous pemphigoid.
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2/23. Benign persistent papular acantholytic and dyskeratotic eruption of the vulva: a case report.

    We report a case of 44-year-old woman with persistent pruritic papules on the left and right labium majus of the vulva. Histopathologic examination of the vulvar biopsy specimen revealed a suprabasal separation of the epidermis with acantholysis and dyskeratosis.
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3/23. Zosteriform Darier's disease versus acantholytic dyskeratotic epidermal nevus.

    patients with keratotic lesions distributed in a unilateral, linear, zosteriform or localized pattern and revealing histologic features of dyskeratotic acantholysis have been reported. There is still some controversy regarding the appropriate nosologic placement of this entity. Some believe it represents a localized form of Darier s disease, while others argue it is a variant of epidermal nevus. We report a case of a 42-year-old physician who presented with a 15-year history of an asymptomatic eruption that had been diagnosed as "chronic zoster." Physical exam revealed hyperkeratotic papules and plaques in a dermatomal distribution. The controversy regarding the correct nosologic placement of such a patient is discussed.
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4/23. Grover disease (transient acantholytic dermatosis) and piebaldism.

    A 35-year-old white man with lifelong stable white lesions on the anterior trunk and extremities presented with a pruritic papular eruption limited to the white patches. Results of a histologic examination led to a diagnosis of Grover disease (transient acantholytic dermatosis). To our knowledge, this article is the first to report an association between Grover disease and piebaldism or other depigmented disorders. We review the literature and speculate on the association between these conditions.
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5/23. 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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6/23. Extensive Grover's-like eruption with lentiginous 'freckling': report of two cases.

    We have described two females with extensive freckling and scaly lesions and conclude that they have a widespread form of Grover's disease associated with lentiginous sun-induced 'freckling'. We report these cases to draw attention to an unusual pattern of Grover's disease that can simulate Darier's disease.
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7/23. A peculiar acantholytic dermatosis.

    This paper reports a peculiar case seen in a 36-year-old woman who every summer since the age of 30 has developed numerous solitary vesicles or crusted papules with intense pruritus in the seborrheic zones. There are no general symptoms and no hereditary relations. The individual rash disappears completely in a week to a month and there is no eruption in winter. The clinical picture resembles that of Darier's disease, but histopathological tests reveal intraepidermal cleft or bulla formation due to acantholysis but no dyskeratosis. Clinicopathological consideration indicates a disease akin to familial benign chronic pemphigus, but as our case does not correspond to any known disease, it is therefore reported as a peculiar acantholytic dermatosis.
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8/23. 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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9/23. Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia.

    BACKGROUND and methods. We describe five patients with underlying neoplasms in whom painful mucosal ulcerations and polymorphous skin lesions developed, usually with progression to blistering eruptions on the trunk and extremities. Histologic examination showed vacuolization of epidermal basal cells, keratinocyte necrosis, and acantholysis. Immunofluorescence testing revealed atypical pemphigus-like autoantibodies in perilesional epithelium and serum from all five patients. We studied the antigenic specificities of the autoantibodies by indirect immunofluorescence and immunoprecipitation, using extracts of 14C-labeled human keratinocytes. IgG purified from the serum of one patient was passively transferred to four neonatal mice to test for pathogenicity. RESULTS. Immunofluorescence testing showed that the autoantibodies bound to the surface of tissues containing desmosomes, including complex and simple epithelia, and myocardium. An identical and unique complex of four polypeptides with molecular weights of 250, 230, 210, and 190 was immunoprecipitated by all serum samples. The 250-kd polypeptide comigrated with desmoplakin I (a protein found in the desmosomes of all epithelia), and the 230-kd antigen comigrated with the antigen of bullous pemphigoid. Cutaneous blisters, a positive Nikolsky's sign, and epidermal and esophageal acantholysis developed in all mice into which the autoantibody was injected. Electron microscopy showed epidermal acantholysis similar to lesions of experimentally induced pemphigus vulgaris. CONCLUSION. These five patients with cancer had a novel acantholytic mucocutaneous disease characterized by autoantibodies that were pathogenic after passive transfer. The autoantibodies from these patients reacted with an antigen complex composed of desmoplakin I and the 230-kd antigen of bullous pemphigoid and two as yet unidentified epithelial antigens. We suggest the term "paraneoplastic pemphigus" for this disease.
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10/23. Transient acantholytic dermatosis associated with lymphomatous angioimmunoblastic lymphadenopathy.

    Transient acantholytic dermatosis is a papulovesicular cutaneous eruption first described in 1970. There have been subsequent reports of similar disorders occurring in patients with malignancy. Angioimmunoblastic lymphadenopathy with dysproteinemia is a disorder characterized by an acute onset of generalized lymphadenopathy associated with fever, malaise, pruritus, night sweats, and hepatosplenomegaly. The patient described had a papular acantholytic dermatosis associated with the development of angioimmunoblastic lymphadenopathy with dysproteinemia-like T-cell lymphoma. The cutaneous manifestations of angioimmunoblastic lymphadenopathy with dysproteinemia are discussed.
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