Hard Skin Lesion? Is it Scleroderma?

Don’t get panic if you also have a similar lesion, because it is a very rare disease with an incidence of 30 cases per 100,000.  It is 4 times commoner in females than in males, so if you are a female, you have a higher chance of getting the disease. People at the age of 30-50 years are affected by this disease.

It can manifest either as localized scleroderma or systemic scleroderma. 

The localized scleroderma can be either linear or morphea, and it only affect the skin.

The systemic disease can be,

1.      Limited
2.      Diffuse
3.     Sine

And it can affect the skin, underlying tissues, and internal organs (heart, lungs, and kidneys).

What are the symptoms?

Initially you may have,

•   musculoskeletal discomfort
•  fatigue
• weight loss 
•   heartburn

The more specific symptoms are referred to as CREST syndrome.

·         Calcinosis

·         Raynaud phenomenon

·         Esophageal reflux

·         Sclerodactily (tightly bound waxy skin on the fingers)

·         Talangiectasias

How to identify a scleroderma skin lesion? 

Thickening or hardening of the skin is the characteristic feature in scleroderma. The area of the body, in which the lesions develop, varies depending on the kind of the disease you have. Those common areas are listed below.

1.      Morphea – face, chest, back, arms, legs

2.      Linear scleroderma – arm, leg, forearm

3.      Limited scleroderma – face, lower arms and legs, hands, fingers

4.      Diffuse scleroderma – face, upper arms, upper legs, hands

·         Course of skin changes in diffuse scleroderma

o   Inflamed fingers – presence of edema and erythema,

o   pigmentory changes are also seen – alternating hyperpigmented and vitiligo like areas slat and pepper appearance)

o   pruritus (itching) in the early phase of the disease

These symptoms may last from weeks to months.

In addition to these, you may have Talangiectasias (dilated capillaries). They occur on the face and palmer surface of the hands. Subcutaneous Calcinosis is also seen along the extensor surface of the forearm and on the fingers.

How the heart is affected

Heart is involved in the late stages of the disease. The disease causes contraction band necrosis and tissue fibrosis, which result in arrhythmias, cardiomiopathy and heart failure. These may manifest as chest pain, palpitation or as dyspnoea on exertion.

How the lungs are affected

The lung involvement may manifest either as interstitial fibrosis or as pulmonary artery hypertension or both. The patient may have dyspnoea on exertion, but this is seen in later stages of the disease. 

How the kidneys are affected

Kidney involvement manifest as sudden onset of malignant hypertension. Earlier, before the drug, angiotensin converting enzyme inhibitors were discovered, renal failure was the cause of death for many scleroderma patients.  Therefore scleroderma patients must have regular blood pressure check ups. These patients may have other symptoms of hypertension such as headache, visual changes or seizures.  

How to diagnose

To diagnose there should be

·         Thickened skin changes proximal to the metacarpophalangial joint

                                   Or two of the following

·         Sclerodactily

·         Digital pitting

·         Bibasilar pulmonary fibrosis

Another way of diagnosing scleroderma is by the presence of 3 of the five features of the crest syndrome.

Presence of Rhaynaud’s phenomenon for longer period indicates limited scleroderma and rapidly progressed Rhaynaud’s phenomenon and other symptoms indicate diffuse scleroderma.

Treatment

Although there is no cure for scleroderma, there are treatment for some of the symptoms of the disease.

•    NSAIDS
•   Immunosuppressant drugs
•   Prostacyclin infusion for digital ulcerations