Short answer · Medically reviewed summary · Last updated: 2026-05-08
Eosinophilic Fasciitis, also known as Shulman syndrome, is a rare fibrosing disorder with an estimated annual incidence of approximately 0.1 to 0.3 cases per 100,000 individuals. Because of its rarity and the frequent overlap with other scleroderma-spectrum conditions, true prevalence is likely underestimated due to consistent underdiagnosis and misdiagnosis in clinical settings. Is Eosinophilic Fasciitis considered a rare disease? Yes, Eosinophilic Fasciitis is classified as a rare disease.
What is the prevalence of Eosinophilic Fasciitis?
Prevalence of Eosinophilic Fasciitis: how many people are affected worldwide, differences by sex and region, with sources.
Eosinophilic Fasciitis, also known as Shulman syndrome, is a rare fibrosing disorder with an estimated annual incidence of approximately 0.1 to 0.3 cases per 100,000 individuals. Because of its rarity and the frequent overlap with other scleroderma-spectrum conditions, true prevalence is likely underestimated due to consistent underdiagnosis and misdiagnosis in clinical settings.
Is Eosinophilic Fasciitis considered a rare disease?
Yes, Eosinophilic Fasciitis is classified as a rare disease. While exact global prevalence numbers are not definitively established, it is widely recognized by organizations like NIH GARD as an uncommon condition. Within the DiseaseMaps.org community, we have connected with 14 individuals living with Eosinophilic Fasciitis, providing a unique, real-world perspective on a condition that many primary care physicians may only encounter once in their careers.
Who is most affected by Eosinophilic Fasciitis?
The demographic profile of Eosinophilic Fasciitis reveals specific patterns regarding age and gender:
- Age of Onset: While it can occur at any age, including in children, the peak onset for Eosinophilic Fasciitis is typically between 30 and 60 years of age.
- Gender Distribution: Research indicates a slight female predominance, though the condition affects both males and females across various ethnic groups.
- Geographic Variation: There is no strong evidence suggesting that Eosinophilic Fasciitis is limited to specific geographic regions, though its rarity makes large-scale epidemiological tracking difficult.
Why is accurate data on Eosinophilic Fasciitis difficult to obtain?
Obtaining precise statistics for Eosinophilic Fasciitis is challenging because the clinical presentation—characterized by skin thickening and inflammation of the fascia—is often mistaken for systemic sclerosis (scleroderma) or other dermatological conditions. The diagnostic delay contributes to the difficulty in calculating exact incidence rates, as many patients may remain undiagnosed or misclassified in medical databases for years.
Next steps
- Consult a rheumatologist or a specialist in connective tissue diseases to discuss your symptoms.
- Join the DiseaseMaps.org community to connect with other patients who understand the journey of managing Eosinophilic Fasciitis.
- Keep a symptom log to share with your care team, noting any skin changes or limitations in mobility.
Medical disclaimer: This content is for informational purposes only and does not constitute medical advice, diagnosis, or treatment; always seek the advice of your physician or other qualified health provider with any questions regarding a medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Eosinophilic fasciitis overview.
- Orphanet: Rare disease database entry for Shulman syndrome (ORPHA:333).
- PubMed/NCBI: Clinical reviews on the epidemiology and diagnostic criteria of eosinophilic fasciitis.
- The Scleroderma Foundation: Resources regarding scleroderma-spectrum disorders.
