Diagnostic hints that another disorder is responsible include the absence of Raynauds phenomenon, a lack of abnormalities in the skin on the hands, a lack of internal organ involvement, and a normal antinuclear antibodies test result. Treatment
No cure for scleroderma is known, though treatments exist for some of the symptoms, including drugs that soften the skin and reduce inflammation. Some patients may benefit from exposure to heat. Holistic care of patients comprising patient education tailored to patients education level is useful in view of the complex nature of the disease symptoms and progress. Topical/symptomatic
Topical treatment for the skin changes of scleroderma do not alter the disease course, but may improve pain and ulceration. A range of nonsteroidal anti-inflammatory drugs, such as naproxen, can be used to ease painful symptoms. The benefit from steroids such as prednisone is limited. Episodes of Raynauds phenomenon sometimes respond to nifedipine or other calcium channel blockers; severe digital ulceration may respond to prostacyclin analogue iloprost, and the dual endothelin-receptor antagonist bosentan may be beneficial for Raynauds phenomenon. Skin tightness may be treated systemically with methotrexate and ciclosporin. and the skin  thickness can be treated with penicillamine. Kidney disease
Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis that may be the initial manifestation of the disease. Renal vascular injury (due in part to collagen deposition) leads to renal ischemia, which results in activation of the renin-angiotensin-aldosterone system (RAAS).
Pili torti (singular pilus tortus; also known as "twisted hairs") is characterized by short and brittle hairs that appear flattened and twisted when viewed through a microscope. : 638 : 764 This phenotype is noted in Menkes disease and Lichen planopilaris. Pili torti can also occur after use of retinoids, such as isotretinoin. See also
List of cutaneous conditions
Crandall syndrome

References


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