Abstract
Localized scleroderma (LS) or morphea are terms encompassing a group of rare chronic inflammatory fibrosing disorders of the skin and underlying structures such as subcutaneous fat tissue, fascia, muscle and bone. As, in contrast to systemic sclerosis, internal organs like GIT, lungs, heart and kidney are not affected and the disease has normal life expectancy, some classifications use term morphea for all subtypes in order to avoid unnecessary confusion with systemic form, other use term LS.
The subtypes of the disease include plaque, generalized, linear, deep and mixed variants, some classifications comprise also eosinophilic fasciitis and atrophoderma of Pasini-Pierini. The treatment should start early in the course of the disease before complications occur as these are difficult to treat.
The main indication criteria for the treatment are based on disease activity, subtype of the disease and depth of involvement. Current treatment options for superficial forms include phototherapy and topical therapy with corticosteroids, tacrolimus, calcipotriol, alone or in combination with bethamethason dipropionate, some studies suggestt the use of imiquimod.
The well- established systemic therapy for severe forms include systemic methotrexate alone or in combination with systemic corticosteroids, in treatment resistant cases the use of mycophenolate mofetil is promissing. It seems that prolonged systemic therapy with methotrexate for 1-2 years reduce relapses of the disease that are observed in one quarter of patients.