Utility of ultrasonography in hair-thread tourniquet syndrome.

A 2-year-old girl presented with a 2-month history of an erythematous, indurated plaque with well-defined borders on the third toe of the right foot. Bedside high-resolution ultrasonography demonstrated a thickened epidermis overlying a hyperechoic focus within the dermis. Her clinical and sonographic presentation was in keeping with a foreign body causing hair-thread tourniquet syndrome. The foreign body was surgically extirpated without neurovascular sequelae. Ultrasonography expedited accurate diagnosis and is a promising adjunct to clinical evaluation for radiolucent foreign bodies.

[Ainhum and "African acral keratoderma": three cases]. / Aïnhum et « kératodermies acrales africaines ¼ : trois cas.

BACKGROUND: Ainhum, or spontaneous dactylitis, involves the formation of a gradual constriction in the digital-plantar fold of the fifth toe that leads, after several years, to autoamputation of the digit. This condition is classically distinguished from "true" ainhum, of unknown aetiology and affecting only subjects of African origin, from "pseudo-ainhum", resulting from different causes such as inflammatory constriction or constriction by a foreign body, and finally from ainhumoid palmoplantar keratoderma, which is of genetic origin and occurs for instance in Vohwinkel syndrome. Herein, we report three cases of ainhum in women of sub-Saharan African origin; in addition, all three subjects were also presenting various forms of hyperkeratosis of the hands and feet known to primarily affect subjects of African origin. PATIENTS AND METHODS: The three patients, aged 30, 48 and 44 years, were respectively from Mali, Guinea and Senegal. They had consulted a dermatologist for violent pain in the fifth toe, which frequently prevented sleep and was inexplicable despite several consultations, and even in one case in spite of surgical investigation. Once the diagnosis had been made, relief was promptly provided for all three patients through Z-plasty to remove the circular constriction around the toe in question. In addition to ainhum, the first patient was also presenting diffuse palmoplantar keratoderma, together with an aspect of acrokeratoelastoidosis on the edges of her hands and feet, and knuckle pads, while the second was presenting diffuse palmoplantar keratoderma and an aspect of marginal acrokeratoelastoidosis, and the third was presenting small knuckle pads. DISCUSSION: A recent study has confirmed the high incidence of several forms of palmoplantar keratoderma of African origin, as well as frequent association of these different varieties with one another. These consist of diffuse keratoderma having a relatively non-specific aspect, keratoderma punctata of the palmar creases, marginal keratoderma known also as focal acral hyperkeratosis, and acrokeratoelastoidosis, despite the absence of histological evidence, and finally, inverted keratoderma, i.e. affecting the dorsal aspects of the extremities, such as knuckle pads. In the three cases presented here, ainhum was associated with these different forms of acral keratoderma seen chiefly in subjects of African origin. CONCLUSION: So-called "true" ainhum may be included in a broader group of African acral keratoderma, further reinforcing the unity of this group. Genetic studies are required to enable validation and refinement of these clinical findings.

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.

Destructive deformation of the digits with auto-amputation: a review of pseudo-ainhum.

Pseudo-ainhum is an auto-amputation of the digits. Although extremely rare, it is a traumatic and painful experience that can be alleviated with early recognition and intervention. The scientific literature is filled with reports of this interesting but unfortunate phenomenon. To date, a firm causative aetiology has not yet been established. Although reports on this phenomenon have attempted to further our understanding of pseudo-ainhum, a clear understanding has been complicated by the interchangeable use of terms describing this auto-amputation. In this review, we discuss the current understanding, diagnostic criteria, and management of pseudo-ainhum. Furthermore, the nomenclature of pseudo-ainhum is clarified. Ideally, this will allow for more efficient exploration of pseudo-ainhum, its causes, and therapeutic approaches.

Pseudoainhum in chronic psoriasis.

Pseudoainhum is a term used to describe the presence of constricting bands of the extremities due to a variety of underlying causes. Progression of the lesions can cause irreversible damage and autoamputation of the affected digit. This report documents a rare association of pseudoainhum and psoriasis and emphasizes the importance of recognizing this condition.

[Ainhum and pseudoainhum: clinical aspects and etiopathogenic hypotheses]. / Ainhum et pseudo-ainhum: panorama clinique et hypotheses etiopathogeniques.

Pseudoainhum is a rare acquired or congenital disorder characterized by progressive development of a fibrotic band on a finger or toe until spontaneous autoamputation occurs. Ainhum is an idiopathic disease involving the fifth toes of black people mainly in tropical zones. Based on experience in five cases and a review of the literature, the authors describe clinical features of these diseases and analyze various etiological hypotheses including infectious (mycosis, mycobacteria), traumatic (plants), vascular (andysplasia), neurological (polyneuritis), and genetic (keratodermia) mechanisms.

Pseudoainhum of all fingers associated with Reynolds' syndrome and breast cancer: report of a case and review of the literature.

Pseudoainhum is a rare disorder characterized by digital constrictions leading to autoamputation. We report a bilateral pseudoainhum of all fingers in a 64-year-old fair-skinned woman with breast cancer, systemic scleroderma, and primary biliary cirrhosis. The overlap between scleroderma and primary biliary cirrhosis with antimitochondrial antibodies M2 is also known as Reynolds' syndrome. Although pseudoainhum has been associated with many conditions, this particular association is exceptional and has yet not been described.

Mal de Meleda keratoderma with pseudoainhum.

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.

Pseudoainhum in porokeratosis of Mibelli.

Porokeratosis of Mibelli in an adult man with involvement of the skin and oral and genital mucous membranes is described. The unusual features in this case were the late onset and pseudoainhum resulting from a porokeratotic lesion in the right foot. The probable cause of pseudoainhum is reviewed.