Scleroderma: Avoid excessive sunlight, cold exposure

Scleroderma is an autoimmune connective tissue disease in which body produces excess proteins called antibodies that attack the skin and internal organs. The skin becomes thickened and hard due to excessive collagen production and deposition in skin as a response to antibody attack. The condition is not a contagious or infectious disease.

However, health experts have explained that causes of scleroderma are not known but certain factors can increase the chance of having this condition. For example: family history of autoimmune diseases, female sex, ethnicity and exposure to mineral oils, silica, some drugs, petroleum products and some chemicals. It also affects children but more common in adult.

Dr. Femi Adelowo a Professor of Medicine and Consultant Rheumatologist at Lagos State Teaching Hospital (LASUTH), Ikeja, Lagos, said scleroderma belongs to the group of disease called “auto-immune disease” which essentially, refers to the body being at war with itself.” The normal defense cells of the body, white blood cells or immune competent cells, turn their ammunition on the body; rather than on germs, our eternal enemies. Autoimmune diseases can affect any part of the body right from the head to the toes and all organs and structures in between. This condition is also classified as connective tissue disease.

He stated scleroderma as in other autoimmune disease, it may be caused by interplay of genes and certain environmental factors. Certain environmental factors identified include; viruses, toxins, rare seed oil; contaminated oil; silicon; asbestosis; vinyl chloride; drugs such as bleomycin; exposure to radiation.

He, however, advised scleroderma patients to recognise the fact that this condition is not a spiritual attack, rather, they should seek a referral to the specialist early, attend clinic regularly, take prescribed medications, discuss his anxieties with the doctor, avoid physical and emotional stress.He said: “Sufferers of Scleroderma have feeling of wearing a steel jacket. “It feels as my skin cannot breathe”

“Though very rare, Scleroderma, and subsequent inflammation and destruction is targeted at the skin, connective tissue and blood vessels, wherever they occur. The condition, like most other auto-immune disease mostly affects females”.Adelowo noted that scleroderma is quite rare, but for whoever it affects, it is common in the patient. It is particularly commoner in blacks than whites with the highest frequency among certain American, Indian population. In Nigeria, it constitutes about one in every 100 patients seen in a rheumatology clinic.

He explained the commonest structure affected by scleroderma is the skin. This presents as hardening of the skin especially the fingers and the toes. As this progresses, there may be discolouration of the skin with hyper pigmentation, often times associated with depigmentation. If this goes on for long, calcium deposits may be found in the skin. Some patients present with arthritis usually affecting the fingers and toes as well as ankles and wrists.
   
He said: “Blood vessel involvement may be in the form of Raynaud’s phenomenon, where the skin changes colour to blue or red in association with pain. If this progresses, sores may appear on the fingers. Hypertension is common when the stomach or intestines are affected, such persons may present with reflux or ulcer.”
 
Adelowo said the lungs are often affected with scar tissue fibrosis in the lungs, leading to breathlessness and cough. The eyes and mouth may feel dry and there may be problems with dentition. Other manifestations include fatigue, numbness in the fingers and depression and anxiety.

He stated that in terms of investigating the condition the skin doctor or rheumatologist will take the history and examine the sufferer. He will order relevant tests such as blood tests, chest x-ray/CT chest; heart tests such as ECG and echocardiography; Lung function tests.Dr. Olaosebikan Hakeem, Consultant Rheumatologist/Physician at the LASUTH, Ikeja, Lagos said symptoms of the condition vary from patient to patient, adding that some may have mild symptoms with just skin problems while others may have skin problems with life threatening internal organs involvement. Occasionally, there may not be skin problem but patient has internal organs challenges.

“Some of the earliest symptoms include- Raynaud’s symptoms- fingers or toes colour changes on exposure to cold accompanied by burning; hand and feet swelling; Sclerodactyly. Thickened skin of the fingers or toes.“While late symptoms include- thickened skin of the trunk, face, extremities; reduced oral aperture, rea of dark and light skin; itching of the skin; dryness of the skin, mouth and eyes; arthritis, joint contracture or deformity, finger ulcers or wounds and scar tissues in the lung, heart, stomach etc. The Kidney can also be affected, which is called scleroderma renal crisis,” he said.

Hakeem explained there are two major types of scleroderma, which include Localised and systemic scleroderma. The localised type affects specific parts of the skin, but does not affect internal organs and it is more common in children while systemic scleroderma affects the skin and internal organs.

“Scleroderma is commoner in female than male and common among people between the age group of 35 to 55 years, but this does not say that it cannot affect any age group.“No single treatment for all patients; treatment is usually based on symptoms or organ affected. However some treatments such as skin softening cream drug for the skin itching and dryness, medications, drugs for raynaud’s, drugs for lung diseases and pulmonary hypertension, among others. The disease is not as prevalent like Hepatitis, AIDS and other known names,” he said.