Scleroderma (systemic sclerosis) is a chronic disease that causes the skin to become thick and hard (sclerotic) with a buildup of scar tissue, resulting in loss of skin elasticity, joint range of motion, muscle strength, and mobility.  Patients with scleroderma usually have proliferation of fibroblasts and excessive collagen proteins. There is also damage to internal organs such as the heart and blood vessels, lungs, stomach, kidneys, heart, and other organs. Scleroderma is an autoimmune disorder of unknown etiology that usually affects women between the age of 30 and 50 and results in extensive scarring and disfigurement as it progresses. The sequence of scleroderma involves the following: arteriole endothelial cells die by apoptosis and are replaced by collagen; inflammatory cells infiltrate the arterioles and cause more damage, thus resulting in the scarred fibrotic tissue that is the hallmark of scleroderma.

Clinical Presentation The two main types of scleroderma are localized and systemic. Localized is further differentiated into morphea, characterized by discolored patches on the skin; and linear,  by streaks or bands of thick hard skin on the arms and legs.  Localized scleroderma only affects the skin and not the internal organs. Systemic scleroderma can be limited (affecting only the arms, hands, and face) or diffuse (rapidly progressing, affecting large areas of the skin and one or more organs).

Thirty-five percent of the patients with scleroderma develop skin ulcers that are painful, refractory, and over bony prominences. (See attached photo of scleroderma wounds on a fibrotic, scarred hand).

CREST, a limited systemic form of scleroderma, is described by the following symptoms:

• Calcinosis – calcium deposits, usually in the fingers
• Raynaud phenomenon – color changes in fingers and sometimes toes after exposure to cold temperatures
• Esophageal dysfunction – loss of muscle control, which can cause difficulty swallowing
• Sclerodactyly – tapering deformity of the bones of the fingers
• Telangiectasia – small red spots on the skin of the fingers, face, or inside of the mouth

The symptoms do not reflect the internal organ involvement that may also be present.

In addition, patients may experience joint pain, fatigue, depression, reduced libido, and altered body image. A third type is limited systemic sclerosis, also known as sine scleroderma, which includes Raynaud

Phenomenon and internal involvement without sclerotic skin. Scleroderma may initially be difficult to distinguish from other systemic autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis.

Medical Management Because the etiology is unknown, treatment of scleroderma centers on alleviating symptoms, preserving skin integrity with protective strategies, and preventing infection. D-penicillamine, colchicine, PUVA, relaxin, cyclosporine, and omega-oil derivatives have been used to treat the skin fibrosis. Immunosuppressive agents such as methotrexate and cyclosporine have been used to treat the systemic disease. Plasmapheresis can be used in severe cases.

Wound Management Local wound care is tedious because of the high-pain levels associated with open wounds on sclerotic skin, and wound healing is impeded by the scarring of the subcutaneous tissue and the immunosuppressive medications with which patients are frequently treated. Enzymatic debridement with collagenase may be helpful with painful wounds, as well as occlusive dressings to help with autolytic debridement. Non-adherent dressings are advised both to minimize pain and avoid tearing the skin upon dressing removal. Silicone-backed foam dressings are useful as secondary dressings. Patient education regarding protective measures for skin is crucial, for example, using gloves when doing housework, avoiding caustic liquids, wearing warm clothes to avoid Raynaud Phenomenon, and using moisturizers to avoid dry skin. As the disease progresses, custom shoes with molded inserts to accommodate changes in the shape of the feet can help to maintain independent ambulation.

In summary, Scleroderma is an autoimmune disorder that causes fibrosis and scarring of the connective tissue, resulting in loss of skin elasticity and loss of joint range of motion.  Dermal wounds may occur spontaneously or from trauma, and healing is impaired by both the epithelial fibrosis and the medication used to treat the systemic disorder.

References:

Gabriella A, Avvedimento EV, Krieg T.  Scleroderma.  N Eng J Med. 2009;360:1989-2003.

Hafner J, Schneider E, Gunter B, Paolo C.  Management of leg ulcer in patients with rheumatoid arthritis or systemic sclerosis: the importance of concomitant arterial and venous disease.  J Vasc Surg.  2000;32(2):322-329.

Hamm R, Shah JB. Atypical Wounds.  In Hamm R (Ed.). Text and Atlas of Wound Diagnosis and Treatment, 2^nd edition.  New York: McGraw-Hill Education.  2019;235-266.