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Erosive arthropathy associated with scleromyxedema
Abstract:
Mucin deposition in the skin (scleromyxedema) is a rare disorder with a historically confusing nomenclature. The etiology and pathogenesis of the disease is poorly understood. The precise extent of potential systemic involvement is uncertain but the prevailing hypothesis is that scleromyxedema is a disorder of skin fibroblasts without internal organ involvement. Two patients with scleromyxedema skin lesions are described in whom an erosive arthropathy developed in joints contiguous to clinically involved skin. Because of the absence of other causes of destructive arthritis, it is postulated that the associated skin disease resulted in infiltration of adjacent synovium with subsequent erosions.
Insights
Scleromyxedema, a rare skin disorder, may involve joint erosion in affected skin areas. This suggests potential systemic involvement, challenging previous hypotheses of skin-only fibroblast issues.
Area of Science:
- Dermatology
- Rheumatology
- Pathology
Background:
- Scleromyxedema is a rare mucin deposition skin disorder with unclear etiology and pathogenesis.
- Historically, scleromyxedema was considered a localized skin disease without systemic organ involvement.
- The prevailing hypothesis suggested scleromyxedema primarily affects skin fibroblasts.