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Seborrheic Distributed Papules With Palmoplantar Hyperkeratosis—Diagnosis
Arch Dermatol. 2005;141:779-784.
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Diagnosis: Ichthyosis hystrix, Curth-Macklin type.
MICROSCOPIC FINDINGS AND CLINICAL FINDINGS
Microscopic sections revealed papillomatosis and compact orthokeratotic hyperkeratosis. The granular layer was markedly thickened, with vacuolated keratinocytes exhibiting perinuclear edema and numerous binucleate cells. No significant epidermolysis or acantholysis was seen. Other family members had had similar clinical presentations and had previously been studied, although this information was not known at the time that the patient described herein was initially seen. Electron microscopy of skin specimens from those relatives revealed abnormal organization of keratin intermediate filaments with cytoplasmic shell formation.1
DISCUSSION
In 1954, Curth and Macklin2 classified this rare autosomal dominant genodermatosis, which presents in early childhood with massive debilitating palmoplantar keratoderma. Perinuclear shells of unbroken tonofilaments within spinous and granular layer keratinocytes are the pathognomonic ultrastructural feature. Examination using light microscopy reveals perinuclear vacuolization and formation of binucleated cells, without the histologicfeatures of epidermolytic hyperkeratosis.1, 3-4 Since the histologic findings may occasionally be similar, electron microscopy may still be necessary to . . . [Full Text of this Article]
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Arch Dermatol. 2005;141(6):779-784.
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