Resolution of pansclerotic morphea after treatment with antithymocyte globulin

Abstract

Treatment with prednisone 60 mg per day and methotrexate 15 mg per week was started, but symptoms worsened. Methotrexate was replaced by pulsed intravenous methylprednisolone (1 g daily for 3 days), followed by mycophenolate mofetil started at 1 g per day and titrated up over 4 weeks to 3 g per day. Severe bicytopenia developed that did not improve with an 8-week washout of immunosuppressive agents. His fibrotic skin and hematologic conditions dramatically responded to antithymocyte globulin 40 mg/kg daily for 4 days, plus 10 mg/kg ciclosporin and methylprednisolone 1 mg/kg per day.

Clinical Features of Patients with Morphea and the Pansclerotic Subtype: A Cross-sectional Study from the Morphea in Adults and Children Cohort

“…The results of the present study confirm prior observations in which patients with pansclerotic morphea have a more severe clinical course with a higher relative frequency of males as compared with other morphea patients (7-13). The unique distribution of skin lesions in our case series was also consistent with prior reports of centrifugal spread encompassing near total body involvement except for the digits.…”

“…Historical descriptions of pansclerotic morphea have consistently defined it by a unique general distribution pattern with associated deep involvement (1-13, 22). Unfortunately, they have not been consistent in how depth of involvement is either defined (ranging from deep dermal to bone) or determined (2, 3, 7, 10-13).…”

“…Unfortunately, they have not been consistent in how depth of involvement is either defined (ranging from deep dermal to bone) or determined (2, 3, 7, 10-13). We removed this criteria due to ambiguity and instead solely assigned the pansclerotic subtype based on the clinical criteria of rapid evolution of near total body surface area involvement typically spreading centrifugally with sparing the fingers and toes.…”

“…Pansclerotic morphea was defined as the presence of a cutaneous distribution pattern consistent with prior reports of near total BSA involvement with the characteristic sparing of the fingers and toes. Additional criteria of deep involvement beyond the dermis was not factored into the categorization of patients due to the significant variation in both definition and lack of consistent reporting of deep involvement per prior reports (2, 3, 7, 10-13). Registry participants completed a comprehensive health questionnaire in addition to the collection of blood and/or skin biopsy samples for immunologic and genetic studies.…”

Thrombocytopenia Associated with Localized Scleroderma: Report of Four Pediatric Cases and Review of the Literature

“…A few cases of autoimmune thrombocytopenia associated with localized scleroderma have been reported (Table ). The classification of morphea in the majority of reported cases was of the generalized type, whereas one case had linear scleroderma and one had pansclerotic morphea.…”