Cases reported "Acantholysis"

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1/20. In vivo enalapril-induced acantholysis.

    enalapril is a widely used antihypertensive drug with a very powerful in vitro acantholytic effect. It has been known to potentially induce pemphigus in genetically predisposed subjects. The action mechanism is complex and still only partially understood. We describe the case of a 66-year-old man, affected with intermediary basal cell carcinoma, in whom the histological examination showed suprabasal acantholytic clefts in the perilesional epidermis. Surprisingly a second biopsy taken from the apparently healthy skin of his back confirmed the presence of acantholytic changes. Clinical signs of pemphigus were absent. The patient's history did not reveal any relevant data but a mild arterial hypertension that had been treated for 1 year with 10 mg enalapril. Taking into account the patient's history (enalapril long-term administration), the absence of any bullous or erosive lesions and the histological findings, a diagnosis was made of in vivo enalapril-induced acantholysis.
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2/20. Oral acantholytic itching disease responding to dapsone. dermatitis herpetiformis, pemphigus, or a new disease?

    A patient had a blistering and severely itching disease confined solely to the oral cavity. The histopathologic findings had the features of both pemphigus and dermatitis herpetiformis. There were no gastrointestinal symptoms, no IgA could be found in uninvolved skin, and no intercellular or basement membrane antibodies were present in the serum or epidermis. The disease responded favorable to dapsone (diaminodiphenylsulfone), which could be discontinued after 2 1/2 years. The literature is briefly reviewed, and there is a discussion of whether the patient was suffering from pemphigus or dermatitis herpetiformis. The conclusion is drawn that the diagnosis cannot be established with certainty and that we are perhaps dealing with a new disease. It is stressed that in such aberrant cases a therapeutic trial with dapsone should be made.
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3/20. 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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4/20. Acantholytic dermatosis of the vulvocrural area.

    Acantholytic dermatosis of the vulvocrural area is a rare skin disorder characterized by solitary or multiple skin-colored to white, smooth papules or plaques. Histopathological features of both Hailey-Hailey disease and Darler's disease are present. There is acantholysis, which may involve the full thickness of the epidermis, and dyskeratosis with corps ronds and grains. There may be marked hyperkeratosis and focal parakeratosis. We report a case of this rare disease and discuss its differential diagnosis and treatment.
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5/20. Acantholytic dyskeratotic epidermal nevus: a rare histopathologic feature.

    BACKGROUND: Epidermal nevus is a congenital malformation of the epidermis consisting of verrucoid scaly plaques on the skin, often in a linear fashion. Different histologic features have been seen and, at times, acantholytic dyskeratosis has been observed. We report a new case of acantholytic dyskeratotic epidermal nevus. CASE REPORT: A 3-year-old girl presented, since birth, asymptomatic keratotic scaly lesions on the left hemithorax and left arm that followed Blaschko's lines. histology: Biopsies revealed acanthosis, papillomatosis, hyperkeratosis and focal areas of suprabasal clefting with acantholysis, as well as individual dyskeratotic cells (corps ronds et grains) in the upper layers of the epidermis. In the literature, this histologic feature has been reported twice. Generalized or localized Darier's disease are well-established clinical entities with characteristic histologic features of acantholytic dyskeratosis. Because of the linear clinical appearance and the onset at birth or early childhood, the lesions should be regarded as epidermal nevi and not linear Darier's disease. CONCLUSION: We report here an additional case of dyskeratotic epidermal nevus, which is a rare histopathologic feature.
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6/20. A case of acantholytic dermatosis and leukemia cutis: cause or effect?

    leukemia cutis is capable of presenting in a variety of clinical and histologic guises. We describe a 75-year-old man with a recent diagnosis of M0 acute myelogenous leukemia, who presented with multiple pruritic erythematous papules on his chest and back. Microscopically, the epidermis showed acrosyringeal-based acantholysis consistent with transient acantholytic dermatosis (TAD), associated with exocytosis of atypical hematolymphoid cells. In addition, the dermis showed a contiguous atypical hematolymphoid proliferation consistent with conventional leukemia cutis. To our knowledge, this is the first such case combining features of TAD with leukemia cutis. It remains to be determined whether the acantholysis occurred secondary to the leukemia cutis, was initiated by the migration of leukemic cells, or if the association is merely serendipitous.
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7/20. Congenital acantholytic dyskeratotic dermatosis: localized darier disease or disseminated benign papular acantholytic dermatosis?

    Acantholytic dyskeratosis is a histopathologic pattern defined by a hyperkeratotic and parakeratotic epidermis with intraepidermal clefts containing acantholytic and dyskeratotic keratinocytes. These typical features are distinctive but not entirely pathognomonic of darier disease, since they may occur at cutaneous and mucocutaneous sites in other conditions such as Grover disease, acantholytic and dyskeratotic epidermal nevus (nevus of Starink), warty dyskeratoma, or acantholytic papular dermatosis localized to the vulvocrural area. We report a newborn girl who had congenital erosive papules and plaques located on the left thigh, left ankle, and right side of the neck. Histopathologic examination of a punch biopsy specimen disclosed findings typical of acantholytic dyskeratosis. In the absence of any family history or other manifestations of darier disease, we propose the descriptive term "congenital acantholytic dyskeratotic dermatosis." This descriptor characterizes our patient's disease on the basis of the clinical and histopathologic findings and facilitates recognition of this condition until a putative genetic mutation can be demonstrated or ruled out.
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8/20. Trichofolliculoma with incidental focal acantholytic dyskeratosis.

    Incidental focal acantholytic dyskeratosis has been described in a variety of cutaneous lesions, including benign and malignant epithelial lesions, fibrohistiocytic lesions, inflammatory lesions, and melanocytic lesions. It has also been observed in follicular lesions such as comedones and ruptured follicles. We report the case of a 47-year-old man with a firm, flesh-colored 2-mm pruritic papule in the sun-exposed area above the left eyebrow. An excisional biopsy was performed, the tissue was processed, and the hematoxylin and eosin slides were evaluated. Microscopic examination showed a dilated cystic cavity filled with keratinous debris and scattered fragments of hair. Smaller secondary follicular structures branched from the primary cyst's walls into the adjacent dermis. Hyperkeratosis, acantholysis, dyskeratosis, and suprabasilar clefts were also focally present. Correlation of the lesion's clinical morphology and microscopic features established a diagnosis of trichofolliculoma with incidental focal acantholytic dyskeratosis. Hence, trichofolliculoma can be added to the list of follicular lesions in which focal acantholytic dyskeratosis may be observed as an incidental microscopic change.
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9/20. Grover's disease: successful treatment with acitretin and calcipotriol.

    A 74-year-old man presented with a five-month history of highly pruritic keratotic papules on the trunk and extremities. Laboratory data revealed mild eosinophilia, elevated IgE (483KE/I) and no other signs of atopy. Tests to exclude parasitic infestation were negative. Two biopsies showed non-specific changes. The third biopsy revealed small acantholytic clefts in the upper part of the epidermis and confirmed the diagnosis of Grover's disease. Topical treatment with calcipotriol ointment and systemic treatment with acitretin in low doses successfully reduced the pruritus within one week, and brought about a complete remission within three weeks without any recurrence.
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10/20. 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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