Reconstruction of Intractable Ulcer of the Knee Joint in Werner's Syndrome with free Latissimus Dorsi Myocutaneous Flap

Taniguchi, Y.; Tamaki, Toru

doi:10.1055/s-2008-1040775

Cited by 5 publications

(2 citation statements)

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“…Surgical techniques like rotation flaps have been applied with success in some cases [22,23], but certain areas such as the ankle and foot do not have adequate perfusion and supportive subcutaneous tissue to allow flap viability. For this reason, we tried a Thiersch graft, using the postero-lateral aspect of the thigh as donor to cover the ulcer, once the Pseudomonas aeruginosa over-infection was cleared.…”

Section: Discussionmentioning

confidence: 99%

Leg ulcer in Werner syndrome (adult progeria): A case report

Pau¹,

Patta²,

Aste³

et al. 2013

Dermatology Online Journal

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Werner syndrome (WS; MIM#277700) or adult progeria, is a rare disease, associated with mutations of a single gene (RECQL2 or WRN), located on chromosome 8 (8p12). It codes a DNA-helicase, whose defects cause genomic instability. The highest incidences are reported in Japan and Sardinia (Italy). On this major island of the Mediterranean Basin, the WS cases have been observed in the northern areas. The authors describe the apparently first case reported in southern Sardinia, a 51-year-old woman, who was born in and resides in the province of Cagliari. She presented with a 9-year history of an intractable leg ulcer and other characteristic symptoms, including "bird-like" face, high-pitched voice, premature greying, short stature, abdominal obesity in contrast with thin body type, scleroderma-like legs, decreased muscle mass, diabetes, atherosclerosis, and premature menopause. A specialized genetic Institute of Research (IRCCS-IDI, Rome) confirmed the clinical diagnosis. There is no cure or specific treatment and patients must be periodically screened for an increased risk of cardiovascular and cerebrovascular disease and malignancies. Among the many findings, leg ulcers significantly affect the patient's quality of life. This problem may send the patient to the dermatologist, who finally suspects the diagnosis. Poor response to medical treatment may require aggressive repeated surgery, with poor or temporary results.

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Section: Discussionmentioning

confidence: 99%

Leg ulcer in Werner syndrome (adult progeria): A case report

Pau¹,

Patta²,

Aste³

et al. 2013

Dermatology Online Journal

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“…Flap surgery is highly applicable to ulcers with an exposed knee‐joint cavity. There are reports on cases of anterior tibial artery flap, sartorius muscle flap and free latissimus dorsi myocutaneous flap 10,11 …”

Section: Treatmentmentioning

confidence: 99%

Management guideline for Werner syndrome 2020. 6. Skin ulcers associated with Werner syndrome: Prevention and non‐surgical and surgical treatment

Kubota

Takemoto

Taniguchi

et al. 2020

Geriatrics Gerontology Int

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Aim To provide guidelines on the diagnosis, treatment, and prevention of skin ulcers in Werner syndrome. Methods This article was based on literature from 1996, when WRN was identified as a gene responsible for Werner syndrome, and we evaluated several authentic clinical cases of genetically diagnosed patients. There were 63 patients with Werner syndrome in the Japanese reports retrieved from Medical Online between January 1996 and December 2017. There were 56 patients with Werner syndrome in English reports written by Japanese authors and retrieved from PubMed during the same period. Results Records on skin ulcers were found in 27 (43%) out of 63 patients and 22 (40%) out of 56 patients from the Japanese and English reports, respectively. The reported ulcers were often located at the distal one‐third of the lower legs. There were 8 patients with callosities in the foot in the Japanese reports and 9 patients in the English reports. A skin ulcer in Werner syndrome is generally intractable. Weight‐bearing ulcers or callosity should be critically assessed in surgical procedures because they have effects on patient pain and gait. By adopting a recently advanced technique to facilitate wound healing, the cases of ulcers that were difficult to treat and those requiring major operations can be closed with minimally invasive surgery. Conclusions Skin ulcers in Werner syndrome are refractory, and they lead to reduced quality of life of patients. A callosity in Werner syndrome is an important therapeutic target for the prevention of ulcers. Geriatr Gerontol Int 2021; 21: 153–159.

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