Document Detail


Disabling pansclerotic morphea of childhood poses a high risk of chronic ulceration of the skin and squamous cell carcinoma.
MedLine Citation:
PMID:  18048875     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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 x 20 mg/day and repeated application of a porcine small intestinal submucosal acellular matrix.
Authors:
Uwe Wollina; Michael Buslau; Birgit Heinig; Ivailo Petrov; Eleonore Unger; Eugenia Kyriopoulou; André Koch; Erich Köstler; Jaqueline Schönlebe; Gunther Haroske; Thorsten Doede; Kyrill Pramatarov
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  The international journal of lower extremity wounds     Volume:  6     ISSN:  1534-7346     ISO Abbreviation:  Int J Low Extrem Wounds     Publication Date:  2007 Dec 
Date Detail:
Created Date:  2007-11-30     Completed Date:  2008-01-08     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101128359     Medline TA:  Int J Low Extrem Wounds     Country:  United States    
Other Details:
Languages:  eng     Pagination:  291-8     Citation Subset:  IM    
Affiliation:
Department of Dermatology and Allergology, Hospital Dresden Friedrichstadt, Academic Teaching Hospital of the Technical University of Dresden, Dresden, Germany. wollina-uw@khdf.de
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Carcinoma, Squamous Cell / etiology*
Chronic Disease
Collagen / therapeutic use
Combined Modality Therapy
Contracture / etiology
Female
Humans
Male
Piperazines / therapeutic use
Purines / therapeutic use
Scleroderma, Localized / complications*,  therapy
Skin Neoplasms / etiology*
Skin Ulcer / etiology*
Skin, Artificial
Sulfones / therapeutic use
Vasodilator Agents / therapeutic use
Chemical
Reg. No./Substance:
0/Apligraf; 0/Piperazines; 0/Purines; 0/Sulfones; 0/Vasodilator Agents; 139755-83-2/sildenafil; 9007-34-5/Collagen

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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