Abstract
Scleroderma-like disorders include a set of entities involving cutis, subcutis and, sometimes, even muscular tissue, caused by several pathogenetic mechanisms responsible for different clinical–pathological pictures. The absence of antinuclear antibodies (ANA), Raynaud’s phenomenon and capillaroscopic anomalies constitutes an important element of differential diagnosis with systemic sclerosis. When scleroderma can be excluded, on the basis of the main body sites, clinical evolution, any associated pathological conditions and specific histological features, it is possible to make a correct diagnosis.
Highlights
Scleroderma-like disorders (SLDs) include various diseases in which sclerosis-like changes occur in the dermis and subcutaneous tissue, sometimes with extension to the deeper planes, involving the underlying muscles and bones
Each entity is distinguished from systemic sclerosis by the different distribution pattern of sclerosis and the lack of other peculiar features, such as Raynaud’s phenomenon, capillaroscopic abnormalities or scleroderma-specific autoantibodies
To better define the specific clinical and histopathological characteristics of these entities, we have divided them into groups based on the underlying pathogenetic mechanisms, as follows (Table 1): inflammatory, sclerodermiform mucinoses, genetic (Werner’s syndrome, scleroatrophic Huriez syndrome, stiff-skin syndrome and melhoreostosis), drug induced, toxic, metabolic and paraneoplastic (POEMS syndrome, myeloma with scleroderma-like changes, carcinoid syndrome and scleroderma-like manifestations in myeloproliferative hypereosinophilic syndrome) [2]
Summary
Scleroderma-like disorders (SLDs) include various diseases in which sclerosis-like changes occur in the dermis and subcutaneous tissue, sometimes with extension to the deeper planes, involving the underlying muscles and bones. To better define the specific clinical and histopathological characteristics of these entities, we have divided them into groups based on the underlying pathogenetic mechanisms, as follows (Table 1): inflammatory (morphea, atrophoderma of Pasini and Pierini, eosinophilic fasciitis and graft-versus-host disease), sclerodermiform mucinoses (scleromyxedema, Buschke scleredema, nefrogenic systemic fibrosis and cutaneous amyloidosis), genetic (Werner’s syndrome, scleroatrophic Huriez syndrome, stiff-skin syndrome and melhoreostosis), drug induced, toxic (toxicoil syndrome, eosinophilia–myalgia syndrome and vinyl-chloride syndrome), metabolic (porphyria cutanea tarda, diabetes mellitus and phenylketonuria) and paraneoplastic (POEMS syndrome, myeloma with scleroderma-like changes, carcinoid syndrome and scleroderma-like manifestations in myeloproliferative hypereosinophilic syndrome) [2]. The diagnosis of morphea is usually based on clinical data, histopathological confirmation is sometimes required [1,3,4,5,6] Another rare variant on the face is Parry Romberg’s syndrome, which is characterized by a progressive atrophy of adipose tissue, muscles, cartilage and bones that is responsible for a characteristic facial dysmorphism. Histological specimens show a thin epidermis, hyperkeratosis with follicular obstruction, dermatitis of the focal interface, the homogenization of collagen with a loss of elastic fibers in the papillary dermis, thickened collagen bundles and perivascular lymphocytic infiltrates in the deep reticular dermis
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