Disabling pansclerotic morphoea: a case report

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

“…One novel observation in our cohort is the adult-predominant composition whereas most reported cases are of childhood-onset disease (1, 4, 7-10, 22). The age distribution of patients in the study is not the result of preferential enrollment in the MAC cohort, which is composed of 97 children and 263 adults.…”

“…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.…”

“…Lesions were noted to spare only the fingers and toes. A number of case reports, predominantly involving children, detail similar clinical findings, however the definition and frequency of “deep tissue involvement” was ambiguous and inconsistent (8-10). Further, the relative frequency among morphea patients, demographic features, clinical features, and response to treatment of pansclerotic morphea remains unknown, especially among adults.…”

Localized Scleroderma: MR Findings and Clinical Features

“…Circumscribed morphea can be either superfi cial or deep. Because the superfi cial variant is limited to the epidermis and dermis, it was not included in the present problems, and irreversible functional impairment (6)(7)(8). Musculoskeletal manifestations should be carefully monitored in patients with LS, and the disease should be treated aggressively (9)(10)(11).…”