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[Strip papular mucinosis associated with systemic sclerosis]
N Martin-Bracciani1, O Cogrel, M Beylot-Barry
1Service de Dermatologie, Hôpital du Haut-Lévêque, CHU Bordeaux Groupe Sud, 33604 Pessac Cedex.
Annales De Dermatologie Et De Venereologie
|July 20, 2004
Summary
Dermal mucinosis, a skin condition, can rarely occur with scleroderma (a collagen disorder). This case highlights a unique presentation of strip-like lesions, distinct from papular mucinosis, responding well to treatment.
Area of Science:
- Dermatology
- Rheumatology
- Pathology
Background:
- Clinically apparent dermal mucinosis is uncommon and can be associated with collagenosis, typically lupus erythematosus and rarely scleroderma.
- Dermal mucinosis presents a diagnostic challenge, requiring differentiation from papular mucinosis, which has a different prognosis.
Observation:
- A 51-year-old woman presented with unusual, infiltrated, erythematous lesions in linear "strip" patterns on her thighs and legs.
- Associated symptoms included myalgia, arthralgia, puffy fingers, sclerodactyly, telangiectasias, Raynaud's phenomenon, and esophageal involvement, leading to a diagnosis of CREST-type systemic scleroderma.
Findings:
- Biopsy of the lesions confirmed dermal mucinosis.
- Treatment with hydroxychloroquine resulted in the regression of mucinous lesions and stabilized the scleroderma.
Implications:
- This case reports a novel presentation of dermal mucinosis as "strip" lesions associated with systemic scleroderma.
- The findings suggest that dermal mucinosis in collagenosis may stem from enhanced fibroblast mucin synthesis driven by inflammatory cytokines.
- Distinguishing this presentation from papular mucinosis is crucial due to differing prognoses.