253 results match your criteria Ainhum

[Clinical Characteristics and Gene Mutations in 186 Cases of Kindler Syndrome].

Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2022 Apr;44(2):227-235

Department of Dermatology,State Key Laboratory of Complex Severe and Rare Diseases,PUMC Hospital,CAMS and PUMC, National Clinical Research Center for Dermatologic and Immunologic Diseases,Beijing 100730,China.

Objective To investigate the clinical characteristics and genetic mutations in Kindler syndrome(KS)and provide a theoretical basis for the diagnosis and treatment of KS. Methods The clinical data of one case of KS from Peking Union Medical College Hospital and 185 cases reported in literature were collected. The gene mutation types,patient clinical data,and tumor characteristics were statistically analyzed. Read More

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Mal de Meleda Palmoplantar Keratoderma with Pseudoainhum.

Skinmed 2021;19(5):383-384. Epub 2021 Oct 1.

Department of Pathology, Institute of Pathology, Indian Council of Medical Research, New Delhi, India.

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February 2022

First report of Mal de Meleda with pseudo-ainhum treated with gabapentin in a 17-year-old Chinese girl.

Australas J Dermatol 2021 Aug 13;62(3):e466-e468. Epub 2021 May 13.

Department of Dermatology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, China.

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What's the resolutive surgery for pseudo-ainhum in Vohwinkel syndrome? A case report and review of the literature.

Orthop Rev (Pavia) 2020 Dec 1;12(4):8868. Epub 2021 Feb 1.

Department of Orthopaedic Surgery (DICHIRONS), Università degli Studi di Palermo.

Vohwinkel Syndrome, also known as Keratoderma Hereditarium Mutilans, is an extremely rare dominant autosomal keratosis. It typically presents with "starfish" keratoses on the knuckles, palmoplantar keratoderma (PPK), hearing impairment and mutilating digital constriction bands (pseudoainhum) that cause strangulation, often leading to autoamputation of the affected digit. Both medical and surgical treatment haven't shown to date consistent results, in the treatment of pseudoainhum. Read More

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December 2020

Ainhum: revisited.

Postgrad Med J 2022 05 15;98(1159):e1. Epub 2021 Feb 15.

Dermatology, Venereology & Leprosy, Sri Manakula Vinayagar Medical College and Hospital, Puducherry, India

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Pseudo-ainhum in a patient with CREST syndrome.

Int J Dermatol 2020 Sep 18;59(9):1163-1164. Epub 2020 Jun 18.

Department of Dermatology and Venereology, Medical Faculty, Medical University, Sofia, Bulgaria.

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September 2020

Novel p.Ala675Thr missense mutation in TRPV3 in Olmsted syndrome.

Clin Exp Dermatol 2020 Aug 14;45(6):796-798. Epub 2020 May 14.

Department of Dermatology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.

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Unilateral Pseudo-Ainhum in Liver Cirrhosis.

Open Access Maced J Med Sci 2019 Sep 10;7(18):3013-3014. Epub 2019 Sep 10.

Department of Dermatology, University of Rome "G. Marconi", Rome, Italy.

Background: Pseudo-ainhum is defined as any case of auto-amputation not associated with the classic spontaneous ainhum seen in Africans with unknown etiology.

Case Presentation: A severely ill 58-year-old male patient presented with a painless constricting circular band on his left second toe. His medical history was remarkable for severe alcoholic liver cirrhosis with ascites formation leading to dyspnea. Read More

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September 2019

Ainhum (dactylolysis spontanea): a rare case from Nepal.

Suman Nepal

Int J Dermatol 2019 Dec 25;58(12):e235-e236. Epub 2019 Sep 25.

Helping Hands Community Hospital, Chabahil, Kathmandu, Nepal.

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December 2019

Ainhum, a rare mutilating dermatological disease in a female Cameroonian: a case report.

BMC Dermatol 2019 08 12;19(1):12. Epub 2019 Aug 12.

Health and Human Development (2HD) Research Network, Douala, Cameroon.

Background: Ainhum is an idiopathic dermatological disease characterized by a progressive constricting ring usually on the fifth toe, which may lead to spontaneous auto-amputation of the affected toe. Timely diagnosis and treatment are the key elements to avert amputations with resultant mutilating deformities, permanent handicaps and psychological sequelae. Though common in African descents, this pathology has not been described in the Cameroonian literature. Read More

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Pseudo ainhum and facial malformation secondary to Streeter's dysplasia.

J Eur Acad Dermatol Venereol 2019 Dec 8;33(12):e443-e444. Epub 2019 Jul 8.

Pediatric Dermatology, Dermatology Unit, University of Campania Luigi Vanvitelli, Naples, Italy.

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December 2019

Visual Vignette.

Endocr Pract 2019 11 13;25(11):1236. Epub 2019 Mar 13.

From the Department of Endocrinology, Command Hospital (Western Command), Chandimandir, Panchkula, India.

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November 2019

G59S mutation in the GJB2 gene in a Chinese family with classic Vohwinkel syndrome.

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

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February 2019

Loricrin palmoplantar keratoderma: full-thickness skin grafting for pseudoainhum.

M Zamiri S Watson

Clin Exp Dermatol 2019 Jun 27;44(4):444-446. Epub 2018 Sep 27.

Department of Plastic Surgery, Canniesburn Unit, Glasgow Royal Infirmary, Glasgow, UK.

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

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

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January 2018

Utility of ultrasonography in hair-thread tourniquet syndrome.

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

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Pseudoainhum and autoamputation associated with lamellar ichthyosis.

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

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

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November 2018

Pseudoainhum in Systemic Sclerosis.

J Rheumatol 2016 11;43(11):2076

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

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November 2016

Late Onset Progressive Symmetric Erythrokeratoderma with Pseudo Ainhum.

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:

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Ainhum - A Rare Case Report.

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

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Ainhum: A Spot Diagnosis.

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

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December 2015

An Alternative Surgical Approach to Pseudoainhum: A Case Report.

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

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Ainhum or Dactylolysis Spontanea: a Missing Toe.

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.

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January 2016

Hanging on by a thread: a rare case of secondary pseudoainhum.

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

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February 2016

[Ainhum (spontaneous dactylolysis): about a clinical case].

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

Université de Lubumbashi, République Démocratique du Congo.

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October 2015

Familial ainhum: a case report of multiple toe involvement in a father and son, staging of ainhum with insight into different types of constricting bands.

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

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February 2015

[Ainhum and "African acral keratoderma": three cases].

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

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