Short answer · Medically reviewed summary · Last updated: 2026-05-08
Eosinophilic Fasciitis, also known as Shulman syndrome, is classified under ICD-10 code M72.4 (Pseudosarcomatous fibromatosis) and was historically categorized under ICD-9 code 728.89 (Other disorders of muscle, ligament, and fascia). While these codes facilitate medical billing and documentation, they are broad categorizations for a complex, rare fibrosing disorder of the deep fascia. What is the clinical nature of Eosinophilic Fasciitis? Eosinophilic Fasciitis is a rare connective tissue disease characterized by inflammation, thickening, and induration of the fascia—the connective tissue layer surrounding muscles.
ICD10 code of Eosinophilic Fasciitis and ICD9 code
ICD-10 and ICD-9 codes for Eosinophilic Fasciitis, with classification details for clinicians, coders and patients.
Eosinophilic Fasciitis, also known as Shulman syndrome, is classified under ICD-10 code M72.4 (Pseudosarcomatous fibromatosis) and was historically categorized under ICD-9 code 728.89 (Other disorders of muscle, ligament, and fascia). While these codes facilitate medical billing and documentation, they are broad categorizations for a complex, rare fibrosing disorder of the deep fascia.
What is the clinical nature of Eosinophilic Fasciitis?
Eosinophilic Fasciitis is a rare connective tissue disease characterized by inflammation, thickening, and induration of the fascia—the connective tissue layer surrounding muscles. Patients often report the "groove sign," where skin indentations follow the course of superficial veins. Unlike systemic sclerosis, Eosinophilic Fasciitis typically spares the fingers and does not involve Raynaud’s phenomenon, helping clinicians differentiate the two conditions.
How is Eosinophilic Fasciitis diagnosed?
Diagnosis of Eosinophilic Fasciitis requires a multi-faceted approach, as there is no single blood test that confirms the condition. Physicians rely on a combination of clinical physical examination and specific diagnostic procedures:
- Full-thickness biopsy: This is the gold standard, requiring a deep incision that includes the skin, subcutaneous fat, fascia, and underlying muscle to visualize the inflammatory infiltrate.
- Blood tests: Often reveal peripheral eosinophilia (elevated eosinophil count) and increased inflammatory markers like ESR or CRP.
- Imaging: MRI is frequently used to identify thickening of the fascia and increased signal intensity in the affected areas.
Is Eosinophilic Fasciitis a chronic condition?
The prognosis for Eosinophilic Fasciitis is generally favorable with early intervention, often involving high-dose corticosteroids or immunosuppressive agents. While 14 members of the DiseaseMaps.org community have shared their experiences with Eosinophilic Fasciitis, it is important to remember that every patient's journey is unique, with some achieving remission while others may experience a more protracted course requiring long-term management.
Next steps
- Consult a rheumatologist experienced in rare scleroderma-like conditions to confirm your diagnosis.
- Request a full-thickness biopsy if your current diagnostic results remain inconclusive.
- Join the Eosinophilic Fasciitis community at DiseaseMaps.org to connect with others navigating this rare diagnosis.
- Monitor for any changes in skin texture or joint mobility and report them to your specialist immediately.
Medical disclaimer: This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment; always seek the advice of your physician regarding a medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Eosinophilic Fasciitis.
- Orphanet: Shulman syndrome (ORPHA:3335).
- National Organization for Rare Disorders (NORD): Eosinophilic Fasciitis.
- PubMed/NCBI: Current clinical management of Eosinophilic Fasciitis.
