Acrokeratoelastoidosis

Rambhia, Kinjal Deepak; Khopkar, Uday

doi:10.4103/2229-5178.169718

Cited by 10 publications

(8 citation statements)

References 4 publications

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“…Because of this strong association, it is recommended that all patients with AK undergo genetic testing for CF. 2,3 Aquagenic keratoderma must be differentiated from other palmoplantar conditions like hereditary papulotranslucent acrokeratoderma, acrokeratoelastoidosis, and symmetric acral keratoderma.…”

Section: Discussionmentioning

confidence: 99%

A unilateral, transient palmar plaque with papules

et al. 2023

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Section: Discussionmentioning

confidence: 99%

A unilateral, transient palmar plaque with papules

et al. 2023

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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%

“…22,24,28,29 The duration between onset of lesions and presentation was quite varied, two cases had the lesions present from birth, two presented within months of onset while eleven cases had less than 10-year history. 6,21,22,[24][25][26][27][28][29][30][31]34,39,40 The ethnicity of eight cases was not given; however, African/dark-skin was reported in five of the remaining nineteen cases. 6,26,31,35,40 Family history was reported in only four cases.…”

Section: Discussionmentioning

confidence: 99%

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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“…1 Sporadic cases have their onset later in life and are usually related with a history of excessive sun exposure, hyperhidrosis 1,3 or repeated trauma. 4 Association with systemic 5 and localized scleroderma 6 has been described, raising the question of an autoimmune process, and a recent case report suggested an association with immunosuppression. 7 In our case, both late onset and absence of family history suggest a sporadic aetiology, probably related to recurrent trauma in the occupational context, which can also explain more intense lesions in the right hand, the dominant hand.…”

Section: Acrokeratoelastoidosismentioning

confidence: 99%