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J Dermatol 2019 Feb 19;46(2):154-157. Epub 2018 Dec 19.

Department of Dermatology, The First Affiliated Hospital of Huzhou University, Huzhou, China.

Vohwinkel syndrome (VS) is a rare autosomal dominant condition, also known as mutilating palmoplantar keratoderma accompanied by sensorineural deafness. The LOR and GJB2 genes are reported to be responsible for VS. The GJB2 gene encodes connexin 26, a component of intercellular gap junctions expressed in various tissues. Read More

Dermatol Online J 2018 Jan 15;24(1). Epub 2018 Jan 15.

5New York University School of Medicine, Department of Internal Medicine, New York. daren.

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. Read More

Pediatr Dermatol 2018 Mar 16;35(2):e138-e139. Epub 2018 Jan 16.

Department of Dermatology, Hospital Infantil Universitario Niño Jesús, Madrid, Spain.

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. Read More

Indian J Dermatol Venereol Leprol 2017 Nov-Dec;83(6):728-729

Department of Dermatology, Venereology and Leprology, JIPMER, Puducherry, India.

Dermatol Ther 2017 09 21;30(5). Epub 2017 Jul 21.

Department of Pathology, JIPMER, Puducherry 605006, India.

J Rheumatol 2016 11;43(11):2076

Autoimmunity Department, Serviço de Medicina 2.3, Hospital Santo António dos Capuchos, CHLC, Lisbon, Portugal.

Indian J Dermatol 2016 Jul-Aug;61(4):448-50

Department of Dermatology, Venereology and Leprology, Victoria Hospital, Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India. E-mail:

J Clin Diagn Res 2016 Apr 1;10(4):PD17-8. Epub 2016 Apr 1.

Assistant Professor, Department of General Surgery, Mahatma Gandhi Medical College & Research Institute, Sri Balaji Vidyapeeth , Pondicherry, India .

The term 'AINHUM' is derived from the African word meaning 'to saw or cut'. True ainhum otherwise called dactylolysis spontanea is a condition involving soft tissue or digits with constricting rings commonly presenting in fifth toes, usually bilateral. It is to be differentiated from Pseudo-ainhum that occurs secondary to some hereditary and nonhereditary diseases that lead to annular constriction of digits. Read More

Indian J Surg 2015 Dec 28;77(Suppl 3):1411-2. Epub 2014 Aug 28.

Department of Plastic Surgery Unit 2, Christian Medical College, Ground Floor, Paul Brand Building, Vellore, 632004 India.

Ainhum is an acquired progressive condition presenting with a constriction ring around the fifth toe. Classically, it was reported in people of African origin and has been very rarely reported in India. Ainhum when diagnosed and treated early can be prevented from progressing to mutilating deformities. Read More

J Foot Ankle Surg 2016 May-Jun;55(3):605-8. Epub 2016 Mar 5.

Associate Professor and Chief, Foot and Ankle Surgery, Department of Orthopaedics, University of Florida College of Medicine, Jacksonville, FL.

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. Read More

Acta Med Port 2016 Jan 29;29(1):79. Epub 2016 Jan 29.

Serviço de Imagiologia Geral. Hospital de Santa Maria. Centro Hospitalar Lisboa Norte. Lisboa. Portugal.

BMJ Case Rep 2016 Feb 2;2016. Epub 2016 Feb 2.

Department of Orthopaedics and Trauma, Luton & Dunstable University Hospital, Luton, UK.

Pseudoainhum is a rare and poorly characterised phenomenon involving autoamputation of a digit associated with an identifiable or related disease process. We describe the case of a 19-year-old man with long-standing neurodisability, sensorineural hearing loss and severe keratoderma, presenting with painless rapidly-progressing autoamputation of his left little toe. Genetic analysis thus far has been inconclusive. Read More

Radiol Bras 2015 Jul-Aug;48(4):264-5

Universidade Federal do Espírito Santo, Vitória, ES, Brazil.

Pan Afr Med J 2014 23;19:60. Epub 2014 Sep 23.

Université de Lubumbashi, République Démocratique du Congo.

Indian J Dermatol 2015 Jan-Feb;60(1):106

Department of Dermatology, Kilpauk Medical College, Chennai, India.

Ainhum, also known as dactylolysis spontanea, is a painful constriction of the base of the fifth toe, frequently followed by spontaneous amputation a few years later. The disease is often symmetrical on both the feet, but, occasionally, other toes are also affected and rarely the distal phalanx of the fifth finger. Pseudoainhum is a similar condition that occurs as a secondary event resulting from certain hereditary and nonhereditary diseases that lead to annular constriction of digits. Read More

Ann Dermatol Venereol 2015 Mar 23;142(3):170-5. Epub 2015 Jan 23.

Service de dermatologie, hôpital Saint-Louis, AP-HP, 1, avenue Claude-Vellefaux, 75010 Paris, France. Electronic address:

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. Read More

Indian J Dermatol Venereol Leprol 2014 Nov-Dec;80(6):583-7

J Emerg Med 2015 Feb 22;48(2):209-10. Epub 2014 Nov 22.

Lincoln Medical and Mental Health Center, New York, New York.

Indian J Dermatol Venereol Leprol 2014 Nov-Dec;80(6):572-4

Department of Dermatology, Venereology and Leprosy, Grant Government Medical College, Mumbai, Maharashtra, India.

Vet Pathol 2015 Jul 5;52(4):700-11. Epub 2014 Nov 5.

Department of Experimental Oncology, European Institute of Oncology, Milano, Italy COGENTECH S.C.A.R.L., Milano, Italy.

Ringtail is a pathologic condition of laboratory rodents characterized by annular constrictions of the tail. Traditionally, it is classified as an environmental disorder caused by low relative humidity, but other factors (temperature, dietary deficiencies, genetic susceptibility, and caging type) have also been proposed. Twenty litters of mice with ringtail lesions occurred from September 2010 to August 2013 in a facility located in the northern Italy. Read More

BMJ Case Rep 2014 Oct 8;2014. Epub 2014 Oct 8.

Department of Cardiology, Akdeniz University Faculty of Medicine, Antalya, Turkey.

J Am Osteopath Assoc 2014 Oct;114(10):806-8

From the Touro University Nevada College of Osteopathic Medicine in Henderson.

Secondary pseudoainhum is an autoamputation that develops in individuals aged approximately 20 to 50 years and is caused by diseases such as keratodermas, trauma, or congenital factors. The authors report a novel case of secondary pseudoainhum in a patient with Turner syndrome (45,X) who presented with bandlike constrictions in the toes bilaterally. To the authors' knowledge, secondary pseudoainhum has not been reported to be associated with Turner syndrome. Read More

J Invest Dermatol 2015 Jan 22;135(1):170-180. Epub 2014 Aug 22.

Department of Biochemistry and Molecular Biology, University of Maryland School of Medicine, Baltimore, Maryland, USA; Department of Dermatology, University of Maryland School of Medicine, Baltimore, Maryland, USA; Department of Obstetrics and Gynecology and Reproductive Sciences, University of Maryland School of Medicine, Baltimore, Maryland, USA. Electronic address:

Keratodermas comprise a heterogeneous group of highly debilitating and painful disorders characterized by thickening of the skin with marked hyperkeratosis. Some of these diseases are caused by genetic mutation, whereas other forms are acquired in response to environmental factors. Our understanding of signaling changes that underlie these diseases is limited. Read More

Int J Dermatol 2014 Sep 25;53(9):1111-3. Epub 2014 Jun 25.

Institute of Dermatology and Department of Dermatology at No. 1 Hospital, Anhui Medical University, Hefei, Anhui, China; Department of Dermatology and Venereology, Anhui Medical University, Hefei, Anhui, China; State Key Laboratory of Dermatology Incubation, Ministry of Science and Technology, Hefei, Anhui, China.

Objective: Progressive symmetric erythrokeratodermia (PSEK) is characterized by symmetric and growing erythematous hyperkeratotic patches over the body shortly after birth, particularly trunk and limbs, the buttocks, and the face, sometimes together with palmoplantar keratoderma (PPK). The GJB2, GJB3, GJB4, GJB6, ARS (Component B), and LOR gene mutation might contribute to PSEK manifestation. This study aimed to identify sequence alteration of these genes in a Chinese PSEK patient with pseudoainhum. Read More

BMJ Case Rep 2014 May 20;2014. Epub 2014 May 20.

New York College of Podiatric Medicine, New York, New York, USA.

Cutis 2013 Oct;92(4):E9-10

Department of Dermatology, No.1 Hospital of China Medical University, 155 N Nanjing St, Heping District, Shenyang 110001, China.

Int J Dermatol 2014 Mar 24;53(3):357-61. Epub 2013 Jul 24.

Department of Dermatology, Faculty of Medicine, University of Harran, Sanliurfa, TurkeyDepartment of Dermatology, Ministry of Health, Batman Regional Government Hospital, Batman, TurkeyDepartment of Dermatology, Sanliurfa Education and Research Hospital, Sanliurfa, Turkey.

J Clin Rheumatol 2013 Aug;19(5):277-9

Rheumatology Department, Hospital do Servidor Público Estadual de São Paulo, São Paulo, Brazil.

Dactylolysis spontanea is a rare disease of unknown etiology, basically restricted to digits without systemic involvement. It evolves, in the form of a local constriction, affecting typically the distal phalanx of the fifth toe, rarely involving other toes and even more rarely involving fingers as in the case described herein. Read More

J Coll Physicians Surg Pak 2012 Dec;22(12):786-8

Department of Dermatology, Military Hospital, Rawalpindi.

The term Pseudoainhum is used in medical literature to elaborate the presence of constricting bands around the digits of hands and feet due to variety of etiologies. This phenomenon can lead to irreversible damage to the supplying neurovasculature and sequential autoamputation of the affected digits. The report herein, describes the rare presentation of pseudoainhum occurring concomitantly in acute psoriasis. Read More

Int J Low Extrem Wounds 2011 Jun;10(2):96-7

Belfast City Hospital, Belfast, Northern Ireland.

Background: Ainhum and pseudoainhum are the conditions identified by the presence of a constriction band, usually over the extremities.

Case Details: We report a case of pseudoainhum in a Caucasian female with underlying chronic lower limb peripheral arterial disease (PAD). The management of pseudoainhum in patients with PAD is discussed. Read More

Eur J Dermatol 2010 Nov-Dec;20(6):817-8. Epub 2010 Oct 18.

Am J Med Genet A 2010 Aug;152A(8):2043-7

Medical Genetics, Department of Experimental Medicine, Sapienza - University of Rome, San Camillo-Forlanini Hospital, Rome, Italy.

Palmoplantar keratoderma (PPK) may concur with congenital alopecia (CA) in various genodermatoses. We report on a 10-year-old girl with generalized atrichia and a severe form of PPK causing pseudo-ainhum, sclerodactyly, and contractures, a phenotype not consistent with any well-defined condition. Non-specific additional findings comprised mild nail dystrophy and widespread keratosis pilaris including ulerythema ophryogenes. Read More

Joint Bone Spine 2010 Jul 2;77(4):368-9. Epub 2010 Jun 2.

Int J Dermatol 2010 Jan;49(1):79-82

Unit of Plastic Surgery, University of Padua, Padua, Italy.

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). Read More

Br J Dermatol 2009 Aug 30;161(2):452-5. Epub 2009 Mar 30.

Department of Dermatology, Hokkaido University Graduate School of Medicine, North 15 West 7, Sapporo, Japan.

Background: Connexins, components of the gap junction, are expressed in several organs including the skin and the cochlea. Mutations in connexin genes including GJB2 (Cx26), GJB3 (Cx31), GJB4 (Cx30.3), GJB6 (Cx30) and GJA1 (Cx43) are responsible for various dermatological syndromes and/or inherited hearing loss, frequently showing overlapping phenotypes. Read More

J Plast Reconstr Aesthet Surg 2009 Jul 25;62(7):e191-3. Epub 2009 Apr 25.

Department of Plastic & Reconstructive Surgery, Institute for Human Tissue Restoration, Yongdong Severance Hospital, Yonsei University College of Medicine, 146-92 Dogok-Dong Gangnam-Gu, Seoul, Korea.

Pseudoainhum is a rare condition of unknown aetiology that is characterised by the presence of constriction bands of extremities due to a variety of underlying causes that progress to irreversible damage and auto-amputation of the affected digit. Epidermolysis bullosa is a group of disorders characterised by extremely fragile skin and recurrent blister formation resulting from minor mechanical trauma. The association of epidermolysis bullosa with other hand defects, such as hand contracture and pseudosyndactyly, have been reported; however, ainhum-like digital constriction bands have not been reported in association with this disorder. Read More

Med Trop (Mars) 2008 Apr;68(2):111-8

Service de Dermatologie, Hôpital d'instruction des armées Laveran, BP 50, 13998, Marseille.

The foot is particularly exposed to injury and infection in the tropical areas. This article provides a review of the main diseases affecting the foot in the tropics including leprosy, ainhum, ulceration due to Mycobacterium ulcerans, mycetoma, chromomycosis, Kaposi's sarcoma, elephantiasis, podoconiasis, dracunculosis, tungiasis, syphilis and endemic treponematosis, larva migrans, scytalidiosis, and envenomation. Prevention is essential. Read More

Rofo 2008 Apr;180(4):348-50

Regensburg.

J Dtsch Dermatol Ges 2007 Nov;5(11):1008-9

Department of Dermatology, Regensburg University Medical Center, Regensburg, Germany.

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. Read More

Int Wound J 2007 Sep;4(3):251-4

Diabetic Foot Clinic, King's College Hospital, NHS Trust, Denmark Hill, London, UK.

We describe a case of ainhum, a mutilating condition, usually seen in peoples of West African origin. A patient presented at the Diabetic Foot Clinic, Kings College Hospital, with a painful infected ulcer between the fourth and fifth toe of his right foot. He was a 43-year-old Nigerian and did not have diabetes. Read More

J Eur Acad Dermatol Venereol 2007 Jul;21(6):732-7

Loyola Stritch School of Medicine, Maywood, IL 60153, USA.

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. Read More

Pediatr Dermatol 2007 May-Jun;24(3):342-4

Mymensingh Med J 2007 Jan;16(1):100-3

Department of Dermatology and Venereology, BSMMU, Dhaka.

Olmsted syndrome is a rare disorder characterized by symmetrical sharply marginated mutilating keratoderma of palms & soles & hyperkeratotic plaques around the body orifices, onychodystrophy, ainhum & amputation of digital phalanges, flextion deformities of the fingers, localized alopecia, leukokeratosis of the tongue, shortness of stature & laxity of large joints. Inheritance is autosomal dominant, although sporadic cases have been reported. Here we describe two cases of this rare disorder with thickened hyperkeratotic lesion over palm & soles & along with amputation of 3rd , 4th & 5th toes in one case. Read More

Ann Dermatol Venereol 2006 Oct;133(10):791-4

Service de Dermatologie-Vénéreologie, CHU Ibn-Sina, Rabat 10000, Maroc.

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. Read More

Clin Podiatr Med Surg 2006 Oct;23(4):761-7, vii

Section of Podiatric Surgery/Traumatology, Department of Surgery, Broadlawns Medical Center, 1801 Hickman Road, Des Moines, IA 50314, USA.

Congenital transverse deficiency is a rare structural anomaly, primarily occurring in utero. Even though it is rare, congenital transverse deficiency is seen in the private practice setting, and many physicians may be unprepared to make the diagnosis or offer treatment. Furthermore, congenital transverse deficiencies, particularly of the lower extremity, are given little attention in literature. Read More

J Eur Acad Dermatol Venereol 2006 Nov;20(10):1332-4

Indian J Dermatol Venereol Leprol 2006 Sep-Oct;72(5):379-81

Dermatologist, Skin Centre, Base Hospital, Delhi, India.

A 23-year-old young woman presented with recurrent episodes of painful bruising along with linear erosions on the accessible areas of the body of nine years duration with a pseudo-ainhum of her left nipple for the past three months. Her case history included repeated visits to various physicians at different centers and an extensive investigative profile. A diagnosis of autoerythrocyte sensitization was made on the basis of the clinical history, dermatological examination complemented by a positive autoerythrocyte sensitization test, psychiatric evaluation and absence of any organic cause for her ailment. Read More

J Rheumatol 2005 Dec;32(12):2437-9

Rheumatology Department of Lucania, San Carlo Hospital, Potenza, Italy.

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. Read More

East Afr Med J 2004 Aug;81(8):427-9

Department of Surgery, Jos University Teaching Hospital, Jos, Nigeria.

Objective: To determine the pattern of lower limb amputation (LLA) and highlight preventable causes in our hospital.

Design: A retrospective study.

Setting: University Teaching Hospital, Jos, Nigeria. Read More

J Am Acad Dermatol 2004 Aug;51(2 Suppl):S134-6

Department of Dermatology, Combined Military Hospital, Peshawar, Pakistan.