Disabling pansclerotic morphea of childhood poses a high risk of chronic ulceration of the skin and squamous cell carcinoma
| Autor: | Eugenia Kyriopoulou, G Haroske, Erich Köstler, Kyrill Pramatarov, Uwe Wollina, André Koch, Ivailo Petrov, Jaqueline Schönlebe, Birgit Heinig, Thorsten Doede, Eleonore Unger, Michael Buslau |
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| Rok vydání: | 2007 |
| Předmět: |
Chronic leg ulcers
Adult Male medicine.medical_specialty Pathology Contracture Skin Neoplasms Adolescent Pansclerotic Morphea Vasodilator Agents 030209 endocrinology & metabolism Scleroderma Piperazines Sildenafil Citrate 030207 dermatology & venereal diseases 03 medical and health sciences Scleroderma Localized 0302 clinical medicine Skin Ulcer medicine Humans Basal cell Sulfones Skin Artificial business.industry General Medicine medicine.disease Dermatology Combined Modality Therapy Purines Chronic Disease Carcinoma Squamous Cell Surgery Female Collagen business |
| Zdroj: | The international journal of lower extremity wounds. 6(4) |
| ISSN: | 1534-7346 |
| Popis: | Disabling pansclerotic morphea of childhood (DPMC) is a rare and severe variant of scleroderma. This report presents 3 cases that presented to the authors and studies 25 patients from the literature (English language only) for the presence of chronic nonhealing ulcers of skin and skin cancer. The authors identified a total of 30 patients (9 male and 21 female) aged between 1 and 37 years at time of presentation. All cases were less than 14 years old when the disease started. The majority of patients had an aggressive course with deep sclerotic lesions leading to joint contractures and immobility. Five patients suffered from chronic nonhealing leg ulcers (17%), but ulcers were present on other parts of the body (upper limbs, trunk, head) as well (n = 6). Four patients died because of complications of the disease such as sepsis or gangrene. Two patients developed a squamous cell carcinoma at the age of 16 years and 19 years, respectively (6.7%). The available treatment of DMPC-associated ulcers is unsatisfying. Only temporary improvements have been seen in a minority of patients. We report on marked improvement of chronic leg ulcers by a combination of sildenafil 3 × 20 mg/day and repeated application of a porcine small intestinal submucosal acellular matrix. |
| Databáze: | OpenAIRE |
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