Eosinophilic FasciitisDz

Synonyms: Schulman syndrome, diffuse fasciitis with eosinophilia.

ICD-9 Code: 728.89.
ICD-10: M35.4

Definition: Eosinophilic fasciitis is a rare disorder of unknown cause characterized by inflammation in the skin that leads to skin tightening limited to the extremities and spares the face. Onset is often abrupt and may be preceded by vigorous exercise.

Etiology: Most cases are sporadic and of unknown cause. Few cases are associated with exposure to organic solvents, drugs (i.e., simvastatin), Borrelia infection, or hematologic disorders (myelodysplasia, leukemia, lymphoma, aplastic anemia).

Pathology: Full-thickness skin biopsy must include the fascia. Specimens show collagen accumulation in the dermis, with panniculitis and a cellular infiltrate, often containing significant numbers of eosinophils.

Demographics: Onset is usually in middle age. Males and females are equally affected.

Cardinal Findings: Pitting edema may be an early finding. Symmetric skin thickening, with a corrugated (peau d’orange) appearance, frequently affects (in decreasing frequency) the forearms, legs, hands, trunk, and neck. If periarticular areas are involved, joint contractures may develop. In contrast to scleroderma, Raynaud phenomenon is absent and nailfold capillaroscopy is normal.

Uncommon Findings: Pulmonary hypertension, thromboembolic disease, and liver abnormalities have been reported.

Keys to Diagnosis: Peripheral eosinophilia and hypergammaglobulinemia are usually present. Serologic tests for ANA and RF are negative. Skin biopsy specimens may show skin thickening accompanied by tissue eosinophilia.

Differential Diagnosis: Rare cases of this syndrome develop in association with underlying malignancies. Eosinophilia myalgia syndrome associated with ingestion of L-tryptophan more commonly shows muscle, CNS, and visceral involvement. Other localized forms of scleroderma should be considered.

Therapy: Treatment with prednisone benefits most patients. Hydroxychloroquine, penicillamine, and MTX may also be beneficial.

Prognosis: Most patients respond to medical therapy and show significant improvement. Spontaneous remissions have been reported.

BIBLIOGRAPHY

Claw DW, Crofford LJ. Eosinophilic rheumatic disorders. Rheum Dis Clin North Am 1995;21:231–246.PMID:7732171

Mori Y, Kahari VM, Varga J. Scleroderma-like cutaneous syndromes. Curr Rheumatol Rep 2002;4:113–122.PMID:11890876