Fokale akrale Hyperkeratose

Abstract

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.

Focal acral hyperkeratosis and acrokeratoelastoidosis: birds of a feather?

“…To the best of our knowledge, such histopathologic abnormalities have not yet been described in FAH. Thus, we think that FAH and acrokeratoelastoidosis are separate entities, a notion that is in line with the opinion of other groups 2,5–7 …”

“…Furthermore, the authors note that we recently reported on a patient with acrokeratoelastoidosis, 3 a disease closely resembling FAH 2,4,5 . By coincidence, our patient also showed a striking onychodystrophy affecting all her nails.…”

“…Although Khamaysi et al . state that nail dystrophy in FAH had not been previously reported, we would like to direct their attention to a publication from 1999 describing a 63‐year‐old woman with FAH who also revealed a distal dystrophy of the fingernails 2 …”

“…Unfortunately, we rather do not think they are. For years, there is an ongoing and still unsettled debate within the field of dermatology if FAH and acrokeratoelastoidosis indeed represent different diseases 2,5–7 or should rather be considered histopathologic variations of the same entity 1,4,8 …”

“…In conclusion, we believe that there is sufficient evidence to suggest that FAH and acrokeratoelastoidosis are different entities. Since sporadic as well as hereditary variants with autosomal‐dominant transmission have been described for both diseases, 2,4 identification of the genetic defect underlying either one of these disorders should eventually resolve the current debate.…”