Background Localized scleroderma (LS), also known as morphea, is an inflammatory, fibrosing skin disorder that leads to sclerosis of the dermis and subcutaneous tissue. However, many of the affected patients have extra-cutaneous manifestations and associated autoimmune diseases (AD), despite which there are few studies about their prevalence in LS.
Objectives To determine the prevalence of extra-cutaneous manifestations and AD in both adult and paediatric patients with LS at a Spanish tertiary hospital.
Methods We performed a retrospective study of patients diagnosed with LS (confirmed by biopsy) treated at our hospital between January 1988 and December 2014. Cases of isolated eosinophilic fasciitis (EF) or lichen sclerosus et atrophicus (LEA) were excluded. The data were obtained by reviewing their medical records.
Results A total of 78 cases were included, all of them Caucasian, with a mean age at diagnosis being 48 years (range: 2-81) and female:male ratio of 3.2:1. Adult-onset cases were 61 cases (78.2%) and paediatric cases were 17 (21.8%). The mean age of onset in children was 10.5 years, while in adults was 51.8 years. The distribution of types of LS was different between both groups. The most common types in adults were plaque morphea (47.5%), deep morphea (16.3%), generalized morphea (8.2%) and LEA/morphea overlap (8.2%), whereas that in children were linear morphea (35.3%), plaque morphea (35.3%) and deep morphea (11.8%). Morphea lesions occurred concomitantly with FE in 3 adult patients (deep morphea 2, plaque morphea 1), and there was history of malignancy in 7 cases, toxic oil syndrome in 2 cases and Borrelia infection in 1 case. A paediatric patient had previous history of Schönlein-Henoch purpura. One or more extra-cutaneous manifestations were found in 41 patients (52.6%) and more frequently affected the gastrointestinal tract (39%), eyes (31%), musculoskeletal system (22%), central nervous system (22%), peripheral nervous system (20%), oral cavity (17%) and respiratory tract (7%). Constitutional symptoms were present in 17%. Extra-cutaneous manifestations were more frequent in adults (59%) than children (29.4%). ANA were analysed in 24 cases and were positive in 17 of them (70.1%), 5 patients were also positive for rheumatoid factor, 6 for anti-TPO, 1 for anti-centromere, 1 for anti-DNA and 1 for anti-DNA/anti-Ro/anti-Sm/anti-β2 glycoprotein I. One or more associated AD were diagnosed in 17 cases (21.8%), being 16 adults and 1 child. Diagnosed AD were autoimmune thyroid disease (8 cases), alopecia areata (3), psoriasis (3), vitiligo (2), autoimmune thrombocytopenia (1), rheumatoid arthritis (1) and systemic lupus erythematosus (1). The last patient subsequently developed a macrophage activation syndrome.
Conclusions More than half of the patients in our series had extra-cutaneous manifestations, and a significant percentage of them had associated autoantibodies and both organ-specific and systemic AD, similar to that is reported in other series. LS should not be considered as an exclusively limited to the skin but as a disease that may potentially develop systemic involvement. These patients should be studied more thoroughly and followed with close collaboration between the dermatologist and rheumatologist.
Disclosure of Interest None declared