Spotlight on Scleroderma

June is Scleroderma Awareness Month. This disease, as many others, is not often talked about despite the burden it imparts on patients. Scleroderma refers to hardening of the skin, although this is a disease that can also affect internal organs. The impact caused by this disease can be debilitating.

There are overarching subsets of scleroderma, including those with symptoms localized only to skin or those with organ involvement. When internal organs are involved, the term systemic sclerosis can be used.

Clinical subsets:

1. Limited cutaneous systemic sclerosis. CREST syndrome is also a subset of limited scleroderma involving calcinosis cutis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias
2. Diffuse cutaneous systemic sclerosis
3. Systemic sclerosis sine scleroderma (without skin sclerosis)

Women tend to get the disease more often than men do, particularly women 30-50 years old. African Americans can get earlier disease, with increased severity and mortality. The disease etiology is not fully elucidated. Autoantibody production, vascular dysfunction, and deposition of collagen occur in the disease.

Skin manifestations of scleroderma:

• Skin involvement includes tightening and hardening of skin.
• The skin becomes shiny and taut.
• Telangiectasias occur and are sometimes described as mat-like.
• Changes in skin pigmentation can occur, described as poikiloderma.
• Skin on distal fingertips may become taut.
• Characteristic beak like facies occur in some cases.
• Raynaud’s phenomenon leading to cutaneous ulceration can reveal underlying vascular involvement.

Although skin changes occur, other organ systems can be affected. Patients should be assessed for systemic involvement. For example, the musculoskeletal system may be impacted by arthralgias, joint contractures, or distal phalangeal bone resorption. Patients may experience difficulty swallowing, reflux, strictures, or other gastrointestinal symptoms due to esophageal involvement. Pulmonary involvement may reflect interstitial lung disease. Cardiac manifestations can occur, including cardiomyopathy and pulmonary hypertension. Renal injury is possible as well.

For workup and management options, visit our scleroderma page.