Descriptive review of Morphea subjects from a single cohort center

Background/Purpose: Morphea, or localized scleroderma, is an idiopathic, rare fibrotic skin disorder that may result in tissue atrophy, pigment changes, and contractures. Disability may range from purely cosmetic, causing great anxiety, to lifelong functional disability.

Methods: We conducted a retrospective chart review of pediatric and adults diagnosed with morphea at UMDNJ, and data on types of morphea, possible triggering events, disease course, complications and therapies.  Descriptive analysis was done on all variables. 

Results:  Table 1 lists the clinical manifestations: there were 26 subjects (2 males), mostly Caucasians females of mean age 45±24 (10-85 years).  Five had known associated autoimmune disease. Only 2 patients had a family history of morphea.  Thirty-eight percent had a family history of autoimmune disease including SLE, RA, Crohn’s and thyroid disease.  Types of morphea, and known triggering events are listed in table 1.  

Sizes ranged from small (<3±1.5 cm) to large (16±16 cm) with (85%) associated itching, tightness, dryness, burning, pain, or contracture requiring intervention.  More than 90% had more than one lesion involving the trunk or extremity.  Generalized SQ and eosinophilic fasciitis were only found in adults.  Elevated CPK and inflammatory markers were more common with extensive disease. Extra-cutaneous manifestations presented in 18 subjects, including arthralgias, fatigue, and Raynaud.  Two adults had monoclonal gammopathy associated with GSM.  Tissue atrophy occurred in 14 subjects and 81% had long-term cosmetic issues (pigmentary changes), pain or depression due to their lesions.  One was functionally disabled.

Twenty-four patients received the following therapy: Topical steroid (n=1), intra-lesional steroid (n=1), UV phototherapy (n=1).  Immunosuppression was generally used for extensive disease: Methotrexate (n=17) alone or with systemic corticosteroids, etanercept, penicillamine, hydroxychloroquine, cyclosporine and cyclophosphamide. One patient with GSM required skin grafting.  Morphea improved but did not resolve in 19 subjects; only one had resolution after 6 years.  One patient had a relapse, two progressed and one child had limb length discrepancy.   Of 18 patients with extra-cutaneous manifestations, 5 improved concurrently with their skin lesions.   

Conclusion:  Many morphea subjects sought therapy for their discomfort or complications.  Long-term consequences, including joint pains and cosmetic issues, did not always resolve despite improvement of the morphea.  More studies are needed to understand the various types of morphea and its clinical consequences to improve long-term outcomes.

Table 1: Clinical manifestations of 26 morphea subjects