Morphea

Reed M. Garza and Heidi T. Jacobe

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports

Morphea, also known as ‘localized scleroderma’ (a term that should be discouraged because of unnecessary confusion with scleroderma among providers and patients), is an autoimmune disorder characterized by inflammation and subsequent sclerosis of the dermis and sometimes subcutis and beyond. Although previously considered self-limited, emerging evidence suggests a remitting relapsing course may be common. Further, untreated lesions may leave behind cosmetic and functional disfigurement, warranting treatment to avoid these sequelae. Morphea has a spectrum of manifestations ranging from skin only to internal involvement (musculoskeletal is most common) that is different from scleroderma. It can be classified into subtypes including circumscribed, linear or generalized. Any subtype may have involvement of the subcutis. Onset is bimodal and can occur in childhood where linear, morphea is most common or in adults where circumscribed and generalized predominate.

Management strategy

The management of morphea is challenging in large part due to the lack of standardized methods of evaluation and minimal evidence for the efficacy of many treatments. Studies conducted by rheumatologists and dermatologists underscore large discrepancies in the manner in which patients with morphea are evaluated and treated, and frequent use of therapy that is not supported by current evidence. Recently published works are addressing these gaps by providing consensus treatment plans, clinical outcome measures for use in practice, and evidence for therapeutic efficacy.

Initial evaluation

The initial evaluation of patients with morphea should involve:

Questions regarding recent development of new lesions/extension of existing lesions to determine level of activity

Symptoms (pain/itch) associated with activity, musculoskeletal complaints, eye complaints (especially en coup de sabre/hemifacial atrophy)

Total body examination of the skin (patients frequently do not appreciate the extent of lesions) looking for active/inactive lesions

Palpation of affected areas for depth of sclerosis

Examination for limited range of motion, contracture, limb length discrepancy, and evidence of musculoskeletal involvement

Photography

Clinical scoring (LoSCAT)

Imaging with MRI if musculoskeletal involvement is suspected.

Active morphea lesions should be treated as the lesions are frequently symptomatic and can produce permanent cosmetic and functional sequelae. Initial evaluation should be focused on determining the extent, severity, and activity of morphea lesions laying the groundwork for rational therapeutic choices.