A Case of Subacute Cutaneous Lupus Erythematosus in a Patient with Mixed Connective Tissue Disease: Successful Treatment with Plasmapheresis and Rituximab
A 30-year-old woman affected by Mixed Connective Tissue Disease with scleroderma spectrum developed a facial eruption, a clinical and histological characteristic of subacute cutaneous lupus erythematosus (SCLE). Speckled anti-nuclear antibodies, high-titer anti-ribonucleoprotein1, anti-Sm, anti-Cardiolipin (aCL) IgG/IgM, and anti-Ro/SSA antibodies were positive. SCLE was resistant to Azathioprine, Hydroxychloroquine, and Methotrexate while Mycophenolate Mofetil was suspended due to side effects. Subsequently, the patient was treated with three cycles of therapeutic plasma exchange (TPE) followed, one month after the last TPE, by the anti-CD20 antibody Rituximab (RTX) (375？mg/m2 weekly for 4 weeks). Eight and 16 months later the patient received other two TPE and RTX cycles, respectively. This therapeutic approach has allowed to obtain a complete skin healing persistent even after 8-month follow-up. Moreover, mitigation of Raynaud's phenomenon, resolution of alopecia, and a decline of aCL IgG/IgM and anti-Ro/SSA antibodies were observed. 1. Introduction Mixed Connective Tissue Disease (MCTD) is currently defined as an overlapping syndrome with clinical features of Systemic Sclerosis (SSc), Systemic Lupus Erythematosus (SLE), Rheumatoid arthritis (RA), and Polymyositis/Dermatomyositis (PM/DM); the presence of high-titer anti-ribonucleoprotein1 (U1RNP) or speckled anti-nuclear antibodies (ANA) at titer ≥1？:？2,000 is necessary for the diagnosis. The disease affects mainly women in the third decade of life (from 80 to 90%) but it has been also reported in children and in over-80-year-old people [1]. The most frequent clinical manifestations are Raynaud's phenomenon (RP), swollen hands, sclerodactyly, arthritis, myalgias, and oesophageal dysmotility, and also alopecia, malar rash, lymphadenopathy, or kidney damage can be present. Rarely, subacute cutaneous lupus erythematosus (SCLE), characterized by annular or papulosquamous lesions, photosensitivity, and presence of anti-Ro/SSA and anti-La/SSB antibodies, has been described in MCTD patients [2, 3]. MCTD therapy should be identified for each patient depending on the affected organ, but generally there is a good response to steroids, different types of vasodilators, and immunosuppressive agents such as Hydroxychloroquine (HCQ), Azathioprine (AZA), Methotrexate (MTX), or Cyclophosphamide (CYC) [1]. 2. Case Presentation A case of a thirty-year-old woman affected by MCTD with scleroderma spectrum and epilepsy since she was fifteen is here reported. At the beginning she presented fever up to 40°C, arthalgias
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