Non-Neoplastic Dermatopathology

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5 Disorders of the Subcutis

Eosinophilic Fasciitis

Scleroderma

Treatment

High-dose systemic corticosteroids are first-line therapy. Other immunosuppressant agents, such as methotrexate, are used as steroid-sparing agents and in nonresponsive cases. A majority of patients show response to corticosteroid therapy, especially if treatment is initiated in early phases of the disease. Patients unresponsive to corticosteroids or other immunosuppressive therapy may have chronic skin thickening and develop contractures.

Methotrexate or mycophenolate mofetil are treatments of choice for extensive skin disease without visceral involvement. Cyclophosphamide may be used for refractory cases and rapidly progressive disease, and in patients with associated pulmonary fibrosis. Dependent upon extent of cutaneous disease and presence of other organ involvement. Increased mortality is linked to extensive skin disease; cardiac, pulmonary, and/or renal disease; and presence of anti-topoisomerase I antibodies.

Prognosis

Figure 5.6.1 Eosinophilic fasciitis. Superficial portion of biopsy showing expansion of the dermis into the subcutis by sclerotic collagen (arrows). There is a patchy perivascular and perieccrine infiltrate of lymphocytes and plasma cells. These features are similar to those seen in morphea/scleroderma.

Figure 5.6.2 Eosinophilic fasciitis. Sclerotic collagen bundles, with loss of normal fenestrated pattern, encroaching upon the eccrine unit.

Figure 5.6.4 Eosinophilic fasciitis. Infiltrate within fascia consists of lymphocytes, plasma cells, and many eosinophils. Eosinophils may be only focally present, or transient, so ample sampling of fascia may be necessary to demonstrate them. Copyright © Wolters Kluwer, Inc. Unauthorized reproduction of the content is prohibited. 2023

Figure 5.6.3 Eosinophilic fasciitis. Deeper component of biopsy showing portion of fascia (arrows) with moderately dense inflammatory infiltrate.