Caso para diagnóstico

Costa, Mariana Carvalho; E, Bornhausen Demarch; Hertz, Amanda; Fb, Pereira; Dr, Azulay

doi:10.1590/s0365-05962011000600030

Cited by 5 publications

(5 citation statements)

References 6 publications

(7 reference statements)

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“…Since 1990, slightly more than two dozen cases of AKE have been reported. 6,7,[21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40] We reviewed twenty-four cases in the literature. The patients were also older with a median age of 36 years (range between 5 and 79 years old).…”

Section: Discussionmentioning

confidence: 99%

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Marginal acrokeratoderma: a case series

Ibekwe

Ogunbiyi

2020

Int J Res Dermatol

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<p>Marginal acrokeratoderma (MAK) are complex disorders characterized by distribution of cornified papules/plaques along the dorso-plantar and dorso-palmar junction of the feet and hands. They belong to the family of punctate palmoplantar keratoderma. No associated gene defect has been detected. There are two clinically identical types of hereditary/familial MAK; namely acrokeratoelastoidosis (AKE) and focal acral hyperkeratosis (FAH); differentiated by the presence of elastorrehexis in AKE. The aim of this article is to report MPK lesions that are not included in the familiar groupings of punctate and polygonal papules. We described thirteen cases and reviewed the literature. Average age at presentation was 24 years, age ranged from 5 to 79 years. Majority of our cases had lesions on only their feet, that of FAH had lesions on only their hands and AKE had lesions on both hands and feet. Although most lesions reported in literature with FAH and AKE were characterized as 2-5mm flesh-colored to yellowish flat-topped/polygonal, keratotic/punctate papules, coalescing into plaques, we also reported lesions that were keratotic with cracks, desquamating and cobble-stone in appearance. This case series calls for more studies on variations in clinical presentation of MAK lesions and an opportunity to revisit the genetic basis and investigate triggers of this rare disorder.</p>

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Section: Discussionmentioning

confidence: 99%

“…6,26,31,35,40 Family history was reported in only four cases. [33][34]37,38 Other co-morbidities include Crohn's disease, asthma and dystrophic nails. 25,27,31,32 Two cases reported a unilateral affectation; one on the left and hand and foot and the other on right hand and foot respectively; in these cases, lesions were noticed at birth.…”

Section: Discussionmentioning

confidence: 99%

Marginal acrokeratoderma: a case series

Ibekwe

Ogunbiyi

2020

Int J Res Dermatol

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“…This condition includes: focal acral hyperkeratoses, AKE, papulotranslucent acrokeratoderma, mosaic acral keratosis, and punctuate palmoplantar keratoderma [3]. Other conditions that should be taken into consideration in the differential diagnosis of AKE include acrokeratosis verruciformis of Hopf, degenerative collagenous plaques, and punctate palmoplantar keratoderma [4]. An association with systemic and localized scleroderma was reported by Yoshinaga and Tajima, raising the question of an autoimmune process [5,6].…”

Section: Introductionmentioning

confidence: 99%

A sporadic case of unilateral acrokeratoelastoidosis in Saudi Arabia: a case report

et al. 2014

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IntroductionAcrokeratoelastoidosis is a rare condition characterized by bilateral multiple hyperkeratotic papules on the palms, soles, and dorsum of the hands and feet. To the best of our knowledge, only around 40 cases of acrokeratoelastoidosis have been reported worldwide, which are mostly familial.Case presentationWe report the first case from Saudi Arabia in a 5-year-old Saudi girl of Arabian origin, who presented symptoms of acrokeratoelastoidosis with unilateral sporadic keratosis on her left hand and left foot. We also review the clinical and histopathologic features, etiology, differential diagnosis and its treatment.ConclusionsGiven the rarity of acrokeratoelastoidosis, little is known about the disease. Further studies are required to understand the pathogenesis of the disease for better treatment options. Additional case reports of acrokeratoelastoidosis will help in recognizing risk factors, patient characteristics, environmental influences and possibly new etiological factors.

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“…It is frequently seen in infancy or adolescence, but has also been reported in adults. [2][3][4][5] The pathogenesis of the disease is still unknown, and there is no predominance of gender or race. 1 The clinical presentation is characterized by multiple yellowish keratotic papules, sometimes translucent, measuring 2-4 mm in diameter, sometimes umbilicated, located symmetrically on the lateral margin of the hands and feet, only palmar involvement was already described, similarly to our case.…”

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confidence: 99%

“…Furthermore, there may be an association with hyperhidrosis. 2,6 The most common histopathological findings are hyperkeratosis, mild acanthosis and alterations in the elastic fibres of the dermis, which are diminished and fragmented (elastorrhexis). 3 Previous TEM findings suggest a defect in the production and fragmentation of elastic fibres in the dermis.…”

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confidence: 99%

Ultrastructure of acrokeratoelastoidosis

Lopes

Almeida

Filho

et al. 2017

Acad Dermatol Venereol

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Editor Acrokeratoelastoidosis (AKE) is a genodermatosis characterized by small, firm papules or plaques on the sides of the hands and feet, 1 and it was first described in 1953 by Costa, in a Brazilian patient. It is a rare disease, and both autosomal dominant and sporadic forms have been observed. 2 It is characterized by multiple hyperkeratotic papules on the palms, soles and dorsum of the hands and feet. Typical histopathological findings in AKE include hyperkeratosis, acanthosis with fragmented and decreased elastic fibres in reticular dermis (elastorrhexis). 3 Electron microscopy (EM) findings in AKE are limited. A 45-year-old white woman presented with small asymptomatic palmar papules that emerged at the beginning of adolescence. These papules were waxy and in great number along the borders of the hands, but were also present in the flexures of the fingers (Fig. 1a). In detail, the dermatoglyphics were not affected (Fig. 1b), suggesting a dermal involvement. The patient had a bureaucratic job and her activities and hobbies did not require constant use of hands. The feet were not affected. She denied a positive family history. Light microscopy with HE staining showed unspecific findings with compact hyperkeratosis and marked acanthosis.

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Caso para diagnóstico

Cited by 5 publications

References 6 publications

Marginal acrokeratoderma: a case series

Marginal acrokeratoderma: a case series

A sporadic case of unilateral acrokeratoelastoidosis in Saudi Arabia: a case report

Ultrastructure of acrokeratoelastoidosis

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