SCLERODERMA

By Gary M. White, MD

Scleroderma (also known as systemic sclerosis)


Scleroderma (also known as systemic sclerosis) is characterized by progressive fibrosis of various organ systems including the skin, heart (e.g. cardiomyopathy, symptomatic pericarditis, or arrhythmia), lung (e.g. pulmonary fibrosis), kidney (e.g. "renal crisis") and gastrointestinal tract (e.g. malabsorption, repeated episodes of pseudo-obstruction, or severe problems requiring hyperalimentation). In contrast to limit sclerosis (morphea), patients with diffuse sclerosis have involvement of the face and distal extremities Occupational exposure to silica/solvents is correlated with more severe forms [JAAD 2015;72;456]. Patients occupational background and exposure should be explored.

Subtypes

Cutaneous systemic sclerosis (SS) is divided into limited cutaneous and diffuse cutaneous.

Clinical

Patients often present with red, puffy hands, and this may be especially noticeable upon waking up in the morning due to muscle inactivity at night. Patients may not be able to put their rings on or have difficulty making a fist. There may be swelling of fingers or forearms. Over time, the skin becomes indurated and bound down.

Raynaud's phenomenon is often associated. Digital ulcers are common. A "salt and pepper" depigmentation may occur (see photos below). Various other causes of diffuse induration should be considered. See induration. For example, several drugs may induce diffuse induration, e.g. the taxanes.

Malignancy

Increased age at scleroderma onset is strongly associated with cancer risk overall [Arthritis Rheumatol. 2015 Apr;67(4):1053-61]. Anti-RNA polymerase III status is an independent marker of coincident cancer and scleroderma at any age.

Treatment

Treatment is usually carried out by rheumatologists. Typical medications tried for the diffuse skin induration include methotrexate, mycophenolate mofetil, cyclosporin, D-penicillamine and rituximab.

UVA-1

UVA1 may be considered for any skin involvement, including proximal and distal induration and even microstomia [Photodermatol Photoimmunol Photomed. 2009 Jun;25(3):153-5] and [Photodermatol Photoimmunol Photomed. 2011;27:113–114].

Treatment of Digital Ulcers

Botox Injection

Botox injection to the base of fingers can dilate vessel dramatically, reduce pain and the number of lesions. J Hand Surg Am 2009;34:446-52.

UVA-1 Phototherapy

UVA1 therapy healed digital ulcers and improved Raynaud's in one study [Ann Dermatol Venereol 2009;136:323-9].

Endothelin Receptor antagonists

Bosentan (Tracleer), an endothelin receptor antagonist led to a 48% reduction in new digital ulcers in a DBPC study. Bosentan is approved in the European Union for the prevention of new digital ulcers, but studies did not find that bosentan promoted healing of existing digital ulcers compared with placebo. In addition, elevated LFTs are seen in 12% to 14% of patients.

Ambrisentan caused a higher rate of healing of existing digital ulcers in a study of 20 patients but did not prevent the formation of new ulcers [JAAD Aug 2014;71;400]. No liver toxicity was seen in this study.

Divalproex sodium ER 250 mg (a histone deacetylase inhibitor) cleared one patient [JAAD Case Reports January 2015Volume 1, Issue 1, Pages 44–45].

Hydrocolloid Dressings

Use of a hydrocolloid dressing can dramatically reduce pain and speed healing as has been shown JAAD 1989;21:200-4.

Prognosis

The risk of death in one study was 5 times higher than the general population.

Additional Pictures

A middle aged man presented with livedo and ulceration as the first manifestation of scleroderma.
Livedo and ulceration as a manifestation of scleroderma Livedo and ulceration as a manifestation of scleroderma

Sclerodactyly
sclerodactyly

Salt and Pepper depigmentation in scleroderma.
Salt and Pepper depigmentation in Scleroderma Salt and Pepper depigmentation in Scleroderma

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