Eosinophilic fasciitis responsive to treatment with pulsed steroids and cyclosporine

Abstract

Drug name cyclosporine: Sandimmune A 58‐year‐old white woman presented with a 7‐month history of indurated hyperpigmented plaques on her upper back, chest, abdomen, and arms. On the forearms and shins, associated with the induration, there was a waxy and shiny appearance. The lesions affected the dorsal aspect of both hands and feet, but spared the fingers and toes ( ). Gradually, as her skin became more indurated, she developed flexural contractures, limiting her range of movement and impairing her daily physical activities. She denied other systemic symptoms. 1 Bilateral legs with symmetric, firm, indurated plaques with a waxy and shiny appearance She was empirically begun on prednisone, 20 mg daily, with a presumptive diagnosis of eosinophilic fasciitis; however, she did not respond to this therapy. At this point, laboratory examination revealed a normal complete blood count, and negative antinuclear antibodies and rheumatoid factor. The biopsy specimen showed a sparse perivascular lymphocytic infiltrate without eosinophils. The deep reticular dermis was thickened, and edematous, homogenized, horizontally oriented collagen bundles extended down fibrous septa into the fat. The underlying fascia was massively thickened and sclerotic with focal lymphoplasmacytic inflammatory infiltrate extending to the muscle ( ). 2 Biopsy specimen showing horizontally oriented homogenized collagen bundles replacing subcutaneous septa (hematoxylin and eosin, ×10) 3 Biopsy specimen showing markedly thickened fascia with focal inflammatory infiltrate extending to the underlying muscle (hematoxylin and eosin, ×10) The patient then received a course of pulsed methylprednisolone, 1 g daily, for 5 days, and was started simultaneously on cyclosporine, 150 mg, twice a day. Within 3 weeks, she had significant reduction of the skin induration as well as improved range of motion of her joints.

Eosinophilic Fasciitis 30 Years after – What Do We Really Know?

“…In the meantime steroid therapy is indicated [13] to prevent symptoms such as joint contractures and carpal tunnel syndrome. Nonresponders and patients with contraindications for corticosteroids have been treated with different therapies which have all been reported to lead to improvement alone or in combination, such as antihistamines (hydroxyzine) [9], histamine 2 antagonists (cimetidine) [10, 16, 63], nonsteroidal antirheumatic drugs (ibuprofen) [10], hydroxychloroquine [11], extracorporeal or bath photochemotherapy [12, 13], cytostatics [4, 6, 64] and immunosuppressive agents [39, 65, 66]. Recently some in vitro data have suggested the use of α-interferon in EF, which could inhibit IL-5 production [17].…”

Eosinophilic Fasciitis: A Case Report and Review of the Literature

“…In cases resistant to corticosteroids, hydroxychloroquine, azathioprine (66) and methotrexate have been used (67). In some other studies, favorable results were obtained with cyclophosphamide, cyclosporine A and antithymocyte globulin application (13,68,69). Weaker effects have been reported for colchicine and D-penicillamine (67).…”

Eosinophilic Cellulitis (Wells' Syndrome) Successfully Treated With Low-dose Cyclosporine

“…Cutaneous diseases associated with increased eosinophils include drug-induced eosinophilia (7), hypereosinophilic syndrome (8,9), eosinophila myalgia syndrome (10), eosinophilic pustular folliculitis (11), eosinophilic fasciitis (12,13), and angiolymphoid hyperplasia with eosinophilia (Kimura' s disease) (14), all of which have already proven dermatologic conditions amenable to cyclosporine. Besides, on the grounds that overlap has been documented (15,29,30), it is conceivably presumed that a series of eosinophilic dermatoses may constitute subsets of the wide spectrum of a non-specific eosinophilic hypersensitivity reaction to a variety of provoking stimuli.…”

“…The patients with EC show variable responses to treatment regimens (2-4). Cyclosporine has been used in the management of some immune-mediated cutaneous diseases including hypereosinophilic dermatoses (7)(8)(9)(10)(11)(12)(13)(14). Thus, we were encouraged to use cyclosporine in attempts to treat recalcitrant EC.…”