Scleroderma on the Face: Symptoms, Types and Diagnosis

Scleroderma is not a single disease but a group of autoimmune conditions in which the skin becomes hard and firm in places. On the face, such changes occur in different forms, and both the prognosis and the set of examinations depend on which form a person has.

What scleroderma is and how its forms differ

The name comes from the Greek words for "hardness" and "skin". At its core is excessive collagen production, which causes tissue to scar and thicken. There are two main types. Localized scleroderma (morphea) affects mainly the skin, sometimes the muscles and bones beneath it, and occurs in both children and adults. Systemic scleroderma, or systemic sclerosis, can affect blood vessels and internal organs — the heart, lungs and kidneys; it more often develops in women aged 30–50 and is almost never seen in children. Within the systemic form, limited cutaneous and diffuse are distinguished.

What scleroderma on the face looks like

In linear morphea, the thickening runs in a band — sometimes across the forehead and scalp. In limited cutaneous systemic scleroderma, the facial skin thickens and may become taut. In the diffuse form, skin changes spread more widely across the body. CREST syndrome is described separately: calcinosis, Raynaud's phenomenon, esophageal problems, thickening of the skin of the fingers and small dilated blood vessels on the skin.

Other symptoms of skin scleroderma, including itching

Morphea patches may be oval, discolored, sometimes shiny and hairless. Itching is not among the main described signs of skin scleroderma, but if the skin itches, this should be reported to a doctor. In the systemic form there may be fatigue, joint pain and stiffness, thickening of the skin on the hands, red spots and firm nodules under the skin, heartburn and difficulty swallowing.

• firm oval patches on the skin
• bands of thickened skin across the forehead or limbs
• red spots on the skin and firm nodules under the skin
• whitening of the fingers in the cold (Raynaud's phenomenon)
• heartburn, difficulty swallowing

Causes and risk factors

The exact cause is unknown. It is believed that part of the immune system becomes overactive and triggers excessive collagen formation. The role of genes is discussed: a close relative with the disease increases the risk, although the disease is not passed directly from parent to child. Possible triggers include certain chemicals, viruses, medications and silica dust, but in most people no trigger can be found. Women are affected more often than men. The systemic form develops annually in approximately 3 people per 100,000.

How skin scleroderma is diagnosed

There is no single test, and symptoms can resemble other diseases, so the diagnosis is made based on the overall picture. The doctor asks about the medical history, examines the skin, and may order a blood test for antinuclear antibodies, a skin biopsy and imaging examinations to check the internal organs. Such patients are managed by a rheumatologist and a dermatologist.

Possible complications and when to see a doctor

The localized form sometimes resolves on its own within a few years, but linear morphea can leave persistent problems — limb shortening, vision impairment. The systemic form is more dangerous: shortness of breath, increased blood pressure, pulmonary hypertension, and damage to the heart, kidneys and digestive tract are possible. In systemic scleroderma, average life expectancy is about 11 years from the first symptoms, although in the localized form it usually does not differ from normal.

Scleroderma itself is not a reason to call an ambulance. Call 103 or 112 if severe shortness of breath suddenly appears or the condition sharply worsens.

Ask your doctor which form you have, which examinations are needed for the organs and how often to check blood pressure. This material is for reference and does not replace a doctor's consultation.

This content is for reference and does not replace a doctor's consultation. Medical disclaimer