Sweet's syndrome in a patient with idiopathic myelofibrosis and thymoma-myasthenia gravis-immunodeficiency complex: efficacy of treatment with etretinate.
Here we present the case of a patient affected with a unique association of Sweet's syndrome, idiopathic myelofibrosis, spindle-cell thymoma, myasthenia gravis and Good's syndrome (a rare form of thymoma-related, combined immunodeficiency presenting with recurrent respiratory infections). Conventional therapies (corticosteroids, colchicine, DDS, clofazimine) were ineffective or were contraindicated. Treatment with etretinate (50 mg/day) proved effective on skin lesions. Moreover, the patient's general condition unexpectedly improved, with long-lasting (11 months) suppression of respiratory infections and a slight but consistent improvement of hematological parameters such as Hct, MCV, Hb level, RBC, WBC and lymphocyte absolute counts.
Istituto di Dermatologia, Università degli Studi di Milano, Italy.
G
G
Altomare
G L
GL
Capella
E
E
Frigerio
D000368:Aged; D005050:Etretinate / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D009157:Myasthenia Gravis / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D055728:Primary Myelofibrosis / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology; D016463:Sweet Syndrome / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D013945:Thymoma / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D013953:Thymus Neoplasms / Q000150:complications / Q000188:drug therapy* / Q000276:immunology
D005050:Etretinate