ICD-11 classes
15 Diseases of the musculoskeletal system or connective...
Nonorgan specific systemic autoimmune disorders
4A43 — Overlap or undifferentiated nonorgan specific systemic...
4A43.4 — Diffuse eosinophilic fasciitis

ICD-11 4A43.4 — Diffuse eosinophilic fasciitis

Also called Shulman disease/diffuse fasciitis, diffuse eosinophilic fasciitis is a rare idiopathic disorder associated with induration of the skin (orange-peel sign) that generally develops rapidly. It is a dermal and hypodermal sclerosis associated with fibrotic thickening of the subcutaneous adipose lobular septa, superficial fascia, and perimysium. Full thickness excisional biopsy of skin lesions revealing fibrosis of the subcutaneous fascia is generally required for diagnosis. Onset follows unusual physical exertion and trauma, especially in males.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

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