ORGANUM
Scleroderma, sometimes known as systemic sclerosis, is an uncommon connective tissue disease with the most prominent feature being thickening or fibrosis of the skin. It is an heterogeneous disorder, both in the involvement of internal organs and joint as well in the pace and severity of the skin.
Scleroderma can be divided into two primary forms:
Localised scleroderma (morphea, linear scleroderma, scleroderma en coup de sabre)
Systemic sclerosis
Systemic sclerosis can further be divided into:
Limited systemic sclerosis: CREST syndrome
Diffuse systemic sclerosis
Localised scleroderma are cutaneous changes consisting of dermal fibrosis without internal organ involvement. Localised scleroderma can be further classified into:
Morphea: Single or multiple (generalised) plaques commonly on the trunk
Linear scleroderma: Bands of skin thickening commonly on the legs or arms but sometimes on the face (scleroderma en coup de sabre) that typically follow a linear path
Systemic sclerosis has the involvement of internal organs. It is further classified into:
Limited systemic sclerosis: Fibrotic skin limited to the hands and forearms, feet, neck, and face. This category of patients have Raynaud's for years and may have telangiectasis, skin calcifications, and a late incidence of pulmonary hypertension. These patients have a high incidence of of anticentromere antibody involvement. CREST syndrome is a subgroup of patients with limited systemic sclerosis
Diffuse systemic sclerosis: Fibrotic skin proximal to the elbows or knees but excluding the face and neck. Patients with diffuse systemic sclerosis may have onset of Raynaud's within a year of developing systemic sclerosis and patients with limited systemic sclerosis are more likely to have pulmonary, renal or cardiac involvement. They are more likely to have autoantibodies to topoisomerase-1 (anti-Scl-70) and much less likely to have an anticentromere antibody
Overlap syndromes: Scleroderma associated with other autoimmune disease
CREST syndrome
C: Calcinosis (Calcium deposits in cutaneous, subcutaneous and muscle tissue)
R: Raynaud's phenomenon
E: Esophageal dysmotility
S: Sclerodactyly (Tightening of the skin of the fingers and toes; giving a claw like appearance)
T: Telangiectasis
Systemic sclerosis is most common in women, with a female:male ratio of 3:1 between the ages of 35-64. Systemic sclerosis is rare in children and men under the age of 30. It is slightly more common in black women during the childbearing ages, but over all ages, there is probably no significant predominance amongst the black race.
The hallmark of systemic sclerosis is thickened skin, thought to be due to the abnormal production by a subset of fibroblasts of normal type I collagen along with the accumulation of glycosaminoglycan and fibronectin in the extracellular matrix.
As mentioned above, the hallmark of systemic sclerosis is thickened skin. There is a loss of sweat glands and hair loss in areas of tight skin. Although patients seem to have areas of involved and uninvolved skin, as based on the presence of procollagen-1 and adherence molecules, all skin is abnormal. Skin thickening begins on the hands and fingers in virtually all cases of systemic sclerosis. When it begins elsewhere, other localised forms of scleroderma or eosinophilic fasciitis should be considered.