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Diagnosis and Therapy of Localized Scleroderma
Author(s) -
Kreuter Alexander,
Krieg Thomas,
Worm Margitta,
Wenzel Jörg,
Gambichler Thilo,
Kuhn Annegret,
Aberer Elisabeth,
ScharffetterKochanek Karin,
Hunzelmann Nicolas
Publication year - 2009
Publication title -
jddg: journal der deutschen dermatologischen gesellschaft
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.463
H-Index - 60
eISSN - 1610-0387
pISSN - 1610-0379
DOI - 10.1111/j.1610-0387.2009.07178.x
Subject(s) - localized scleroderma , medicine , scleroderma (fungus) , dermatology , morphea , fascia , disease , differential diagnosis , medical diagnosis , pathology , guideline , fibrosis , lichen sclerosus , inoculation
Localized scleroderma is a rare autoimmune disease with primary affection of the skin, and occasional involvement of the fat tissue, muscle, fascia, and bone. Depending on the clinical subtype, the spectrum of skin lesions ranges from singular plaque lesions to severe generalized or linear subtypes which may lead to movement restrictions and permanent disability. This German S1‐guideline proposes a classification of localized scleroderma that, considering the extent and depth of fibrosis, distinguishes limited, generalized, linear, and deep forms of localized scleroderma, together with its associated subtypes. The guideline includes a description of the pathogenesis, of differential diagnoses, and particular aspects of juvenile localized scleroderma, as well as recommendations for histopathologic, serologic, and biometric diagnostic procedures. Based on studies of topical and systemic treatments as well as phototherapy for localized scleroderma published in international literature, a treatment algorithm was developed which takes account of the different subtypes and the extent of disease.

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