Cases reported "Acantholysis"

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1/3. Uncommon vascular naevi associated with focal acantholytic dyskeratosis.

    Cutis marmorata telangiectatica congenita and vascular twin naevi are rare vascular anomalies in which focal acantholytic dyskeratosis is usually not observed. We describe a 44-year-old-man who presented for evaluation of skin lesions that had been present since birth. physical examination revealed anaemic macules adjacent to a naevus telangiectaticus on the chest. Naevus anaemicus was also seen on the shoulders, arms, and left leg. There was bluish-reddish reticulate marking of the skin and cutaneous atrophy. Shortening and hypoplasia of the left leg was observed. Histologic examination of two biopsy specimens revealed focal acantholytic dyskeratosis. In vivo confocal laser scanning microscopy showed dilated capillaries and vessels of the upper dermal plexus in the telangiectatic and decreased capillary blood flow in the anaemic skin sites. The findings were consistent with a diagnosis of cutis marmorata telangiectatica congenita, vascular twin naevi, and incidental focal acantholytic dyskeratosis. The particularities of the present case are the following: firstly, the association of two rare vascular anomalies to which the genetic concept of mosaicism can be applied; secondly, the occurrence of incidental focal acantholytic dyskeratosis in sites of vascular naevi.
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ranking = 1
keywords = naevus
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2/3. Naevus corniculatus: a new acantholytic disorder.

    We describe a 33-year-old man with an unusual epidermal naevus that followed the lines of Blaschko. There were filiform hyperkeratoses, large cutaneous horns and lesions that resembled giant comedones and linear hyperkeratotic plaques. All of these lesions showed acantholysis without dyskeratosis. As the disorder is characterized by multiple small or large horn-like processes, we propose the term 'naevus corniculatus'.
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ranking = 2
keywords = naevus
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3/3. 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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ranking = 5
keywords = naevus
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