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Abstracts, British Journal of Dermatology, Volume 176, Issue 5, 1 May 2017, Pages e101–e105, https://doi.org/10.1111/bjd.15384
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Extract
BSMD Medical Dermatology Meeting, Royal College of Physicians, London, 12th January 2017
Oral presentations
O01
Pansclerotic morphoea and eosinophilic fasciitis: a spectrum or distinct entities?
H. Leeman, A.M. Saracino and C.H. Orteu
Royal Free London NHS Foundation Trust, London, U.K.
The classification of morphoea is controversial, especially generalized and deep subtypes. Pansclerotic morphoea (PSM) and eosinophilic fasciitis (EF) both denote progressive, often widespread confluent/circumferential sclerosis, with debated differences regarding site of onset/distribution, depth of tissue involvement and serological parameters. However, presentations are often blurred and the resultant clinical confusion can lead to diagnostic and treatment delays. We retrospectively reviewed a series of patients with circumferential/confluent sclerosis (PSM and/or EF) in order to explore distinguishable and overlapping characteristics. Among 18 cases, 61% (n =11) had confirmed fascial involvement (FI), and all had concurrent overlying dermal sclerosis. While EF classically commences on the limbs, skin changes were initially truncal in 73% of cases with FI (n =8), and 60% of cases without FI had initial skin changes exclusively on the limbs. Symptoms suggesting inflammation (pruritus, swelling and/or pain) were reported by all, although raised inflammatory markers were present in 82% of cases with FI (n =9) and 57% of cases without FI (n =4). Hypergammaglobulinaemia was a specific marker of FI, which was confirmed in all cases with a raised IgG (+/− IgA) level. However, a peripheral eosinophilia was present in 57% of cases without FI (n =4), and 46% of cases with FI (n =5) had normal eosinophil counts. The presence of extracutaneous manifestations, reduced the range of joint movement and antinuclear antibody positivity were similar between those with and without FI. This small case series demonstrates significant clinical and serological overlap between patients with progressive circumferential/confluent dermal and/or subdermal sclerosis. Therefore, there may be merit in considering PSM and EF as a spectrum of the same condition and it may be useful to adopt the unifying terminology of ‘PSM with or without deep tissue involvement’.
