title: Severe aplastic anemia associated with eosinophilic fasciitis: report of 4 cases and review of the literature.
abstract: Diffuse eosinophilic fasciitis (Shulman disease) is a rare sclerodermiform syndrome that, in most cases, resolves spontaneously or after corticosteroid therapy. It has been associated with hematologic disorders, such as aplastic anemia. The clinical features and long-term outcomes of patients with eosinophilic fasciitis and associated aplastic anemia have been poorly described. We report the cases of 4 patients with eosinophilic fasciitis and associated severe aplastic anemia. For 3 of these patients, aplastic anemia was refractory to conventional immunosuppressive therapy with antithymocyte globulin and cyclosporine. One of the patients received rituximab as a second-line therapy with significant efficacy for both the skin and hematologic symptoms. To our knowledge, this report is the first to describe rituximab used to treat eosinophilic fasciitis with associated aplastic anemia. In a literature review, we identified 19 additional cases of eosinophilic fasciitis and aplastic anemia. Compared to patients with isolated eosinophilic fasciitis, patients with eosinophilic fasciitis and associated aplastic anemia were more likely to be men (70%) and older (mean age, 56 yr; range, 18-71 yr). Corticosteroid-containing regimens improved skin symptoms in 5 (42%) of 12 cases but were ineffective in the treatment of associated aplastic anemia in all but 1 case. Aplastic anemia was profound in 13 cases (57%) and was the cause of death in 8 cases (35%). Only 5 patients (22%) achieved long-term remission (allogeneic hematopoietic stem cell transplantation: n = 2; cyclosporine-containing regimen: n = 2; high-dose corticosteroid-based regimen: n = 1).
authors: From Université Paris Diderot, Sorbonne Paris Cité; AP-HP; Service de Dermatologie (AdM, JDB, MR, MB) and Service de Greffe de Moëlle et Centre de Référence Maladies Rares des Aplasies Médullaires (RPdL, DM, GS), Hôpital Saint Louis, Paris; Service de Médecine Interne (YB, HL) and Service d'Anatomopathologie (AL), Hôpital Charles-Nicolle, Rouen; Service d'Hématologie (CMC, JOB), Hôpital Estaing, Clermont-Ferrand; Service d'Anatomopathologie (JMP) and Service d'Hématologie (FJ), Centre Henri Becquerel, Rouen; and Service de Médecine Interne et Immunologie Clinique (VLS, BB), Hôpital Le Bocage, Dijon; France.
authors: Adèle
authors: A
authors: de Masson
authors: Jean-David
authors: JD
authors: Bouaziz
authors: Régis Peffault
authors: RP
authors: de Latour
authors: Ygal
authors: Y
authors: Benhamou
authors: Cécile
authors: C
authors: Moluçon-Chabrot
authors: Jacques-Olivier
authors: JO
authors: Bay
authors: Annie
authors: A
authors: Laquerrière
authors: Jean-Michel
authors: JM
authors: Picquenot
authors: David
authors: D
authors: Michonneau
authors: Vanessa
authors: V
authors: Leguy-Seguin
authors: Michel
authors: M
authors: Rybojad
authors: Bernard
authors: B
authors: Bonnotte
authors: Fabrice
authors: F
authors: Jardin
authors: Hervé
authors: H
authors: Lévesque
authors: Martine
authors: M
authors: Bagot
authors: Gérard
authors: G
authors: Socié
mesh_terms: D000328:Adult; D000368:Aged; D000741:Anemia, Aplastic / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality; D004802:Eosinophilia / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D005208:Fasciitis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D017211:Treatment Failure