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Scleroderma-form Skin Diseases

Diseases featuring sclerosis (hardening) of the skin and subcutaneous tissue.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Scleroderma-form Skin Diseases?

Morphea (localized scleroderma) is characterized by localized fibrosis of skin and subcutaneous tissue without internal organ involvement. Plaque, generalized, linear and deep forms occur. Linear morphea (en coup de sabre) appears as a linear sclerosing band on the face.

Systemic sclerosis is an autoimmune disease that adds Raynaud phenomenon, pulmonary fibrosis, pulmonary hypertension, gastrointestinal dysmotility and renal crisis to cutaneous fibrosis.

Eosinophilic fasciitis (Shulman syndrome) is a rare disease characterized by eosinophilic infiltration and fibrosis of the fascia. It typically begins abruptly after strenuous exercise with extremity swelling and skin tightening.

Symptoms

Skin sclerosis and thickening
Raynaud phenomenon (cold-triggered color change in fingertips)
Pitting edema then sclerosis (early edema, later hardening)
Digital ulcers (ischemic lesions on fingertips)
Telangiectasias (on face and hands)
Lilac ring (active inflammatory border of morphea plaques)

Risk Factors

Female sex (systemic sclerosis — 4:1 female predominance)
Age 30-50
Silica or polyvinyl chloride exposure (occupational risk)
History of autoimmune disease
Genetic predisposition (HLA-associated)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • When skin hardening and thickening are noticed
  • When Raynaud phenomenon and skin tightening coexist — refer to rheumatology
  • When digital ulcers develop
  • When dyspnea and skin tightening coexist (suspected pulmonary involvement)

Treatment Methods

01
Morphea: topical corticosteroid plus calcipotriol; methotrexate (in active and widespread forms)
02
Linear morphea: UVA1 phototherapy, methotrexate plus short-course systemic steroid
03
Systemic sclerosis: mycophenolate mofetil (skin fibrosis), nifedipine or sildenafil (Raynaud)
04
Digital ulcer: IV iloprost, bosentan (prophylaxis), wound care
05
Pulmonary fibrosis: nintedanib or tocilizumab
06
Renal crisis: ACE inhibitor (URGENT — ramipril or captopril)

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.