ICD-11 code : 4A43.4;
Preferred Label : Diffuse eosinophilic fasciitis;
ICD-11 definition : 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.;
ICD-11 synonym : Fasciitis with eosinophilia syndrome; Eosinophilic fasciitis; Shulman syndrome;
ICD-11 inclusion : Diffuse fasciitis;
Origin ID : 1977389237;
Currated CISMeF NLP mapping
- ICD-10 Mapping
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.
