Abstract:Two familial cases of acrokeratoelastoidosis (AKE) are reported. As a quite uncommon feature, both showed a distinct violaceous hue in addition to the characteristic clinicopathological changes. Furthermore a case of keratoelastoidosis marginalis of the hands, also showing involvement of the feet, is described. This latter syndrome may cause confusion with AKE, but appears to be a separate entity. The classification of AKE is discussed. It is suggested that AKE is a syndrome with a broad spectrum of clinical a… Show more
“…146 In addition to presumptively acquired cases, both sporadic and autosomal dominant forms of AKE have been reported. 146,148,[151][152][153][154][155] There is some evidence to suggest that the pathogenesis of AKE may be due to a failure of elastic fiber synthesis rather than degeneration. Results of an ultrastructural study showed that fibroblasts of lesional skin contain dense granules at the periphery of their cytoplasm, while extracellular elastic fibers are deficient; such findings are consistent with a defect in the secretion of elastic material.…”
“…146 In addition to presumptively acquired cases, both sporadic and autosomal dominant forms of AKE have been reported. 146,148,[151][152][153][154][155] There is some evidence to suggest that the pathogenesis of AKE may be due to a failure of elastic fiber synthesis rather than degeneration. Results of an ultrastructural study showed that fibroblasts of lesional skin contain dense granules at the periphery of their cytoplasm, while extracellular elastic fibers are deficient; such findings are consistent with a defect in the secretion of elastic material.…”
“…hen wurden [1,8], können sie doch, wegen fehlender Elastorrhexis, eher als Varianten oder Phasen der hereditären fokalen akralen Hyperkeratose angesehen werden [31]. Möglicherweise handelt es sich sogar um eine einzige Entität.…”
unclassified
“…Weitere Differentialdiagnosen der fokalen akralen Hyperkeratose sind die Keratoelastoidosis marginalis der Hände [1,20] [12].…”
Focal acral hyperkeratosis is a disorder characterized by hyperkeratotic papules along the border of the hands and feet. Focal acral hyperkeratosis and several marginal punctate keratodermas closely resemble acrokeratoelastoidosis of Costa. The question if there is a difference between acrokeratoelastoidosis of Costa and focal acral hyperkeratosis or if they are variants of the same entity are discussed. We report a case showing focal acral hyperkeratosis, review the clinical and histological features of related conditions and present a modified concept of marginal papular acrokeratodermas.
“…The papules in acrokeratoelastoidosis are characteristically small, shiny, firm, and yellowish, and grouped over the dorsal hand and lateral margins of the palms and soles, the space between the thumb and forefinger, on the anterior surface of the lower legs, and over the knuckles and nail folds 2,3 . Lesions with a distinct violaceous hue have also been reported 4 . The papules are usually asymptomatic.…”
A 45-year-old white woman presented with several years' history of firm, shiny papules on the lateral hands with slight extension to the dorsal fingers. The lesions first appeared between the index fingers and thumbs on both hands. They gradually increased in number, coalescing into plaques and affecting the junction between the palmar and dorsal skin. The patient did not have involvement of her feet. She had been diagnosed previously with chronic eczema that had failed to respond to multiple topical medications. In addition, the patient's sister had similar lesions on both hands. The patient denied any symptoms of hyperhidrosis, excessive sun exposure, or trauma. The plaques were asymptomatic, but were cosmetically unappealing to the patient. On physical examination, small, firm, skin-colored, hyperkeratotic papules, coalescing into plaques, were located on the junction between the palmar and dorsal skin on both lateral margins of the thumb and on the radial side of the index finger (Fig. 1). There were no lesions on the feet. A biopsy taken from a papule on the patient's left hand was consistent histologically with acrokeratoelastoidosis. The biopsy showed marked degeneration of collagen in the dermis with solar elastosis and some smudging of the papillary dermal collagen (Fig. 2). She was treated with clobetasone cream to the affected areas on the hands. After 6 weeks of treatment, she reported no significant improvement.
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