Marginal Papular Acrokeratodermas: No Racial Limitations for a Clinical Spectrum That Responds to Acitretin

Abstract

Marginal papular acrokeratoderma (MPA) encompasses a group of disorders that share crateriform, keratotic papules along the margins of the hands and feet. We report a unique case of a Philippino woman, with focal acral hyperkeratosis (FAH) associated with sensorineural hearing loss. The clinical phenotype of FAH (hereditary MPA without elastorrhexis) is expanded and the hypothesis of a racially limited disorder is weakened. Effective treatment of this embarrassing condition with acitretin is demonstrated.

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

“…It is one of the rare groups of diseases that are believed to be due to some unknown pathophysiological process where genetic variations or heredity may be one of the main causal factors. AKE was believed to have a racial predilection towards Arabs and Negroes; although this hypothesis has been challenged [3]. …”

“…For the differential diagnosis of AKE, focal acral hyperkeratosis has a similar clinical appearance, but during histopathological examination, elastorrhexis is not seen and alterations are confined to the epidermis (hyperkeratosis and acanthosis) [3]. Another condition with a similar clinical appearance to AKE is marginal keratoelastoidosis, which is generally associated with intense sun exposure, marked actinic damage, and genetic inheritance.…”

“…Another condition with a similar clinical appearance to AKE is marginal keratoelastoidosis, which is generally associated with intense sun exposure, marked actinic damage, and genetic inheritance. 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].…”

Acrokeratoelastoidosis as an example of marginal papular acrokeratoderma with prominent elastorrhexis

“…Due to the rarity of the condition, treatment recommendations are mainly based on single cases. Several reports on the efficacy of acitretin or other systemic retinoids in marginal papular acrokeratodermas have been published recently [4,17,18]. However, the potential teratogenicity of this therapy should always be taken into consideration in young patients with AKE.…”

Acrokeratoelastoidosis successfully treated with 10% salicylic acid ointment

“…A recent study showed a slight improvement by treating with topical salicylic acid or topical retinoids, although there were generally recurrences after discontinuation of treatment. 4 It is considered that these topical treatments do not improve dermal elastolytic change. Our case has not been recurrent because he continued to apply 10% salicylic acid ointment.…”