Immunologic and nonimmunologic sclerodermal skin conditions - review

Abstract

Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected skin biopsies described histopathological changes similar to those of scleroderma located strictly on the skin or those of systemic sclerosis. These skin lesions can be found in inflammatory diseases with autoimmune substrate (generalized morphea, chronic graft versus host disease, eosinophilic fasciitis), tissue storage diseases (scleredema, scleromyxedema, nephrogenyc systemic fibrosis, systemic amyloidosis), metabolic diseases (porphyrya cutanea tarda, phenylketonuria, hypothyroidism, scleredema diabeticorum), progeroid syndromes. Given the multiple etiologies of sclerodermal lesions, a correct differential diagnosis is necessary to establish the appropriate treatment.

Scleredema Diabeticorum: A Rare Metabolic Connective Tissue Manifestation of Type 2 Diabetes Mellitus Causing External Restrictive Lung Disease

“…These histological changes are distinct from those of scleroderma, which is an autoimmune connective disorder. Skin histology in scleroderma shows excessive fibroblast proliferation and extensive deposition of new type 1 collagen fibers [ 5 ].…”

“…SD seldom involves internal organs such as the heart, lungs, muscles, intestines, salivary glands, and synovial joints (frozen shoulder) [4]. Recognized manifestations of SD include external restrictive lung disease, diabetic cheiroarthropathy, disfiguration of the face, ophthalmoplegia, slurring of speech, and restriction in joint mobility [4][5][6]. There are no identified treatments for SD.…”