PubMed Health⌕ Search
Home/Archive/PubMed

PubMed · 8457455

Mal de Meleda keratoderma with pseudoainhum.

R Bergman·O Bitterman-Deutsch·M Fartasch·R Gershoni-Baruch·R Friedman-Birnbaum
View original source ↗Export metadata ↓

Abstract

Pseudoainhum is an infrequent complication in the autosomal-recessive keratodermas. We describe two related families in which the diagnosis of mal de Meleda keratoderma has been confirmed by mode of inheritance and ultrastructural findings. One family member, a 9-year-old girl, developed pseudoainhum which threatened the viability of her little fingers. This responded to treatment with etretinate. The treatment dilemma posed by keratoderma-induced pseudoainhum in children, i.e. the concern over the possible skeletal toxic effects of long-term etretinate treatment vs. the risks and outcome of surgery, is discussed.

Explore related subjects

AinhumChildEtretinateFemaleFoot DermatosesHand DermatosesHumansKeratosisMicroscopy, ElectronPedigreeSkinCase ReportsJournal Article

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

R Bergman, O Bitterman-Deutsch, M Fartasch, R Gershoni-Baruch, R Friedman-Birnbaum. 1993. Mal de Meleda keratoderma with pseudoainhum.. https://doi.org/10.1111/j.1365-2133.1993.tb15153.x

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

PubMed2006

[Pseudoainhum and peripheral neuropathy].

BACKGROUND: Pseudoainhum is a rare disease characterised by gradual fibrous constriction of the fingers and/or toes eventually resulting in their amputation. In this article, we report the first case seen in Morocco, highly unusual in terms of its severity. CASE REPORT: A 46-year-old woman with no toxic habits was hospitalised for spontaneous amputation of the fingers and toes. This condition began when the patient was 12 years old with the appearance of a circular constriction band at the base of the fifth toe, eventually resulting in its loss. The patient gradually lost all her other toes and fingers except for the first joint of her left index finger. There was no family history of any similar condition. Clinical examination also revealed perforating plantar disease in two of the stumps and peripheral neuropathy in all four limbs, comprising predominantly axonal disease responsive to electromyogram. Amputation of the index finger was completed and histological examination of the removed section showed nothing unusual, with no signs of diabetes. DISCUSSION: The peculiarity of our case resides in the exceptional severity of these amputations and their association with isolated polyneuropathy. Pseudoainhum has been described chiefly in patients with congenital keratoderma, certain systemic diseases, diabetes and alcohol dependence. A number of etiopathogenic hypotheses have been suggested: traumatic, infectious, vascular, neurological and genetic processes.

Ainhum↗
PubMed2005

Dactylolysis spontanea or ainhum involving the big toe.

We describe the case of an Italian Caucasian man with ainhum involving both big toes. Ainhum or dactylolysis spontanea is characterized by the development of a constricting band around a toe, which progresses to spontaneous autoamputation. It usually affects the fifth toe bilaterally, but in rare cases other toes may be involved. The disease occurs in Black people living in tropical regions but occasionally has been reported in persons having fair skin.

Ainhum↗