The Impact of Scleroderma

This blog series highlights conditions that have a strong impact on people of color and appears as part of Project IMPACT: Improving Medicine’s Power to Address Care and Treatment.

Scleroderma can affect people of all ages, but it is most common in individuals aged 40 and 60 years.

Localized scleroderma is more likely to be seen in children, whereas systemic scleroderma is more likely to be seen in adults. There is a female-to-male predilection of 4:1, the cause of which is unknown. Scleroderma is not directly inherited, but individuals with a family history of rheumatic illnesses are predisposed to it.

While the etiology remains unknown, the disease is characterized by autoantibody production, collagen deposition, and vascular dysfunction.

How does scleroderma impact people of color?

Scleroderma has a higher prevalence and is often more severe in American Indians and Black individuals in comparison to other populations.

Pigmentary changes including diffuse hyperpigmentation as well as depigmentation with perifollicular sparing may be seen. The depigmentation appears more obvious in people with darker skin colors.

What to look for:

There are two variants of scleroderma:

  • The localized variant affects a specific area of skin in patches.
  • The systemic variant affects the internal organs of the body as well as the skin, diffusely.

Localized

Limited cutaneous systemic sclerosis (distal skin sclerosis, Raynaud phenomenon, and frequent severe late-stage complications such as pulmonary hypertension and gastrointestinal involvement). CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias) refers to a subset of patients with limited scleroderma. Linear scleroderma is a form of localized scleroderma that often begins as a linear, indurated plaque on the arm, leg, or forehead. Linear scleroderma can sometimes impede joint mobility. When this occurs in children, it can negatively affect the development of the involved limb.

Systemic

Diffuse cutaneous systemic sclerosis (proximal extremity or trunk skin sclerosis, Raynaud phenomenon of shorter duration, high risk of renal crisis, and cardiac and lung fibrosis).

Internal organ changes include the following:

  • Pulmonary hypertension and interstitial pulmonary fibrosis
  • Hypertensive renal crisis
  • Cardiomyopathy
  • Esophageal dysmotility
  • Sicca syndrome
  • Myositis
Differential:
Diagnostic pearls:

In systemic sclerosis, indurated, taut, shiny skin is seen. This is usually seen first in the fingers and hands and can lead to joint contractures. It will ultimately progress proximally and can involve the trunk. Pigmentary changes may give rise to a “salt-and-pepper” appearance.

Treatment:

The approach to the patient with scleroderma should be multidisciplinary. A rheumatologist and dermatologist should be involved. Depending on the manifestations and course of the disease, other specialties may need to be consulted (nephrology, pulmonology, gastroenterology, or hand surgery).

Besides oral glucocorticoids, the mainstay of treatment is methotrexate and mycophenolate mofetil. Cyclophosphamide may be used temporarily in patients with rapidly progressive disease or in those in whom methotrexate and mycophenolate mofetil is not effective. Adjunctive therapies such as phosphodiesterase-5 inhibitors, calcium channel blockers, endothelin receptor antagonists, and antihistamines may be used to treat other aspects of scleroderma.

 

Sources:

https://www.scleroderma.org/

This Project IMPACT blog series was created to highlight dermatologic conditions that disproportionately affect people of color. By improving diagnosis in skin of color we can reduce racial disparities in healthcare.

More in this series:

 

LEARN MORE