A novel association of pseudoainhum and epidermolytic ichthyosis, successfully treated with full thickness skin graft after failed z-plasty repair.

Pseudoainhum is a rare constriction band variant thatmay progress to spontaneous digital strangulationand auto-amputation. Although its association withpalmoplantar keratodermas is well established, ithas not been reported in conjunction with classicepidermolytic ichthyosis. We describe the first suchcase in a 25-year-old woman who presented witha painful constricting band of the fifth toe. We alsodescribe her treatment course, which consisted ofa failed z-plasty, the traditional therapeutic optionfor acute pseudoainhum, and report the success ofsubsequent full thickness skin graft, suggesting thebenefit of this procedure as a therapeutic alternativefor patients with pseudoainhum.

An Alternative Surgical Approach to Pseudoainhum: A Case Report.

Pseudoainhum is a disorder of unknown etiology that is usually congenital and nonprogressive. One theory states it is caused by amnion band constriction in utero. These constriction bands lead, not only to a less cosmetically appealing foot, but also to long-term neurovascular compromise, resulting in amputation of the digit. The described surgical approaches have been limited within published data. The 2 procedures that have been described are either a Z-plasty or amputation, depending on the degree of the deformity. The present case report provides an alternative surgical approach to a pseudoainhum deformity of a third digit.

A woman with a dangling digit.

Ainhum (dactylolysis spontanea) is a distinct clinical and radiological disorder of dark-skinned people characterized by a progressive development of a constricting band encircling the toe which usually results in spontaneous amputation. Ainhum mainly occurs in African natives, but in times of global migration and tourism, Ainhum is likely to be more often encountered outside Africa. Even though the clinical presentation can mimic more common entities such as arthritis and trauma, the correct diagnosis and treatment is easy if one knows this unusual entity.

Vohwinkel syndrome: treatment of pseudo-ainhum.

BACKGROUND: Vohwinkel syndrome or keratoderma hereditaria mutilans is a rare autosomal dominant palmoplantar keratosis which manifests in infants and becomes more evident in adulthood. Patients with this mutation present hyperkeratosis of the palms and soles, constricting bands of the digits, usually at the fifth, and starfish-shaped hyperkeratosis on the dorsal aspects of the hands and feet. The disease mostly occurs in white women, where constricting fibrous bands appear on the digits and can lead to progressive strangulation and auto-amputation (pseudo-ainhum). AIM: The treatment of this keratoderma is very difficult and tends to be symptomatic: topical keratolytics and systemic retinoids have been used to treat hyperkeratosis, but without consistent results. Reconstructive surgery is utilized for the treatment of pseudo-ainhum. RESULTS AND CONCLUSION: In this study, we present an additional case of Vohwinkel syndrome in which constrictive bands of the fifth digit in the left hand were treated with a cross finger flap, with a favorable outcome after 18 months of follow-up.

Pseudoainhum and erythropoietic protoporphyria.

We report the development of pseudoainhum in a patient with erythropoietic protoporphyria, the first report, to our knowledge, of these two unusual conditions occurring together. An excellent cosmetic and functional result was achieved with a Z plasty.

The surgical pathology of ainhum (dactylolysis spontanea).

Ainhum (dactylolysis spontanea) is a poorly understood disease characterized by spontaneous autoamputation of the fifth toe. Its incidence is highest among blacks and in the tropical and subtropical climates. The present study is the first to present certain gross and semimicroscopic findings. Moreover, scanning electron microscopic and histochemical data are presented and these reportings are believed to be unique. Hyperkeratosis and parakeratosis, together with elongation of the epidermal rete pegs and acanthosis, are observed and these findings are associated with the presence of numerous fibroblasts and wound repair phenomena. The leukocytic infiltrate is identified as being principally of T lymphocyte type, suggesting the existence of an unknown immunologic response. Relevant intraoperative data, radiographs, and light microscopy, as well as transmission electron microscopy, have been reported in this study. The spontaneous amputation of the fifth toe, designated as dactylolysis spontanea, has no known etiology, and no treatment short of amputation. The worldwide incidence of this condition is rare. Two cases have been observed at the Baltimore Veterans Administration Medical Center in the last 5 years. One patient is in an early stage of the disease. The present study involves the single instance in which amputation was elected as the treatment of choice. Because the occurrence of ainhum (dactylolysis spontanea) is rare, discussion of the disease is lacking in medical literature. It is the intent of the present study to be the definitive analysis of clinical, surgical, and surgical-pathologic data of this rare disease. Unique applications of semimicroscopic, histochemical, and light microscopic findings, as well as transmission and electron microscopy, will be presented. Speculation as to the implications of immunologic and/or wound repair responses as implicated in ainhum needs to be explored.

Digital constriction bands in pseudoainhum: morphological radiographic, and histological analysis.

Pseudoainhum is a rare condition of unknown etiology that produces digital constricting rings, most commonly on the small fingers. A thorough discussion of pseudoainhum in the plastic surgical literature is apparently lacking at this time. The authors describe the gross morphology, radiographic and laboratory features, and surgical pathology of the disease, and provide well-defined guidelines for its treatment.

Enfermedad de ainhum: presentación de 1 caso / Ainhum disease: report of a case

Se presenta un caso de enfermedad de ainhum (dactilolisis espontánea) al que se realiza la desarticulación quirúrgica de ambos quintos dedos de los pies