Case Studies 20 : Scleroderma.
Appearance of the young lady with a glimpse when she entered the consulting was thin, emaciated, dark covered with thick, dry skin all over and a masked face with difficulty in movements of limbs. It gave her a repulsive look with lack of expressions.
She is a lady aged 25 yrs diagnosed to have a grave skin disease called Scleroderma. The disease can be localized or widespread involving the internal organs. In this case it was widespread involving the entire body.
Her case history is as follows:
Initially there were some white spots on the face and thickening of skin in lower limbs especially on the soles. It had cracks and wounds associated with it.
It then gradually increased for which she was consulted with dermatologist who diagnosed it to be Scleroderma. She was informed about the prognosis of the disease and the conservative treatment was started but there was hardly any relief.
When she came for consultation her condition was such that it was difficult for her to eat food or give a smile.
She was consulted on 4th August 2009.
Sclerodermais a chronic systemic autoimmune disease (primarily of the skin -"derma") characterized by fibrosis (or hardening -"sclero"), vascular alterations, and autoantibodies Typical scleroderma is classically defined as symmetrical skin thickening, with about 90% of cases also presenting with Raynaud's phenomenon, nail-fold capillary changes, and antinuclear antibodies .
There are two major forms:
Limited systemic sclerosis/sclerodermainvolves cutaneous manifestations that mainly affect the hands, arms, and face. It was previously called crest syndrome in reference to the following complications: Calcinosis, Raynaud’s phenomenon, Esophageal, Esophageal dysfunction, Sclerodactyly, and Telangiectasias.
Diffuse systemic sclerosis/sclerodermais rapidly progressing and affects a large area of the skin and one or more internal organs, frequently the kidneys, esophagus, heart, and lungs. This form of scleroderma can be quite disabling.
The prognosis is generally good for limited cutaneous scleroderma patients who escape pulmonary complications, but is worse for those with the diffuse cutaneous disease, particularly in older age, and for males. Death occurs most often from pulmonary, heart, and kidney complications.
The cause is unknown. Scleroderma runs in families but the genes have not been identified. It affects the small blood vessels (arterioles) in all organs.
Severe complications from scleroderma include:
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Heart: Untreated high blood pressure strains the heart; irregular heart rhythm and enlargement of the heart lead to heart failure.
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Kidney: Scleroderma renal crisis in which malignant hypertension develops and causes acute renal failure.
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Lung: Two-thirds of all patients suffer from respiratory problems, such as shortness of breath, coughing, difficulty breathing, alveolitis (inflammation of lung air sacs), pneumonia, and cancer.
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Digestive:Esophagus damage can make it difficult to swallow food, and acid reflux is common. The stomach can develop gastric antral vascular ectasia (GAVE), which occasionally may bleed profusely. A sluggish intestine may cause pain and bloating; undigested food can result in diarrhea, weight loss, and anemia.
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Skin and joints: Carpal tunnel syndrome is common, as are muscle weakness, joint pain, and stiffness.
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Mouth: Flat white patches, loss of attached gingival mucosa, gingival recession, and diffuse widening of the periodontal ligament(PDL) space are seen. Dysphagia may result from collagen deposition in the lingual and esophageal submucosa. Resorption of the posterior ramus of the mandible, the coronoid process, and the condyle are seen due to pressure from abnormal collagen production in adjacent areas. Inelasticity of the mouth may make dentures or dental prostheses difficult to insert and remove.
Treatment :
There is no direct cure for scleroderma. Because the exact cause is unknown, any treatment is patient-specific and aimed at ameliorating symptoms of the disease.
Prognosis :
People with scleroderma have very different life expectancies. Some — for example, those with limited or mild diffuse disease — can expect to live 20 to 50 years after diagnosis. Others with severe, rapidly progressive disease — a group which makes up less than 10% of the total number of patients with diffuse scleroderma — might have a 50% chance of a five-year survival.
C/C:
1. Thickening of skin all over the body.
- Thickening of skin started on Rt. Foot on soles, Rt. Foot then Lt. foot.
- Unhealed wound on Rt. Hand. Now C/o same to all fingers of both hands.
- Ascending direction.
2. Dryness of skin of fingers.
- Deformities of nails, nails become thick, yellowish in color.
- Burning of fingers.
- Difficulty in movements of fingers.
3. Thickening of skin of soles, horny corns? Since 5 yrs.
- Stitching, pricking type of pain of affected part.
- Affected part is very sensitive to touch, painful on pressure during walking.
4. Loss of appetite since 2 yrs.
- No desire to eat, no feeling of hunger.
5. Blackish discoloration of soles & palms.
- < Winter. > Summer.
- < Rainy season.
6. Dryness of skin .
- < summer.
Physical Generals:
- Diet : Mixed. Now Vegetarian since 5 yrs.
- Appetite : Reduced since 2 yrs. Can tolerate hunger.
- Desire : Spicy++, Sour+, Fruits+, Green Veg.+ , Ice Cream++.
- Aversion : Milk, Sweet+.
- Tongue : slight coated.
- Thirst : 6-7 glasses /day. Moderate.
- Bowel : Once a day, satisfactory.
- Bladder : 6-7 times/day, once at night.
- Perspiration : Scanty. Mainly on face.
- Sleep : 10:00 pm to 6:00 am.
Sound sleep.
Position : Lying on sides.
Lying on soft / hard bed.
Covering : Requires 1 pillow.
- M/H/o : Menarche at 15 yrs of age.
Regular cycle. Duration : 4 days.
- Obs H/o : F.T.N.D. 1 daughter.
- Dreams : of water.
- Thermals : Chilly.
Mind :
1. Tell me about your childhood.
I have spent my childhood in village. We lived as a separate family. We are 2 brothers and 1 sister. I am 2nd amongst them.
To describe my nature.., I was short tempered and occasionally aggressive. Anger was usually expressed by weeping, throwing things.
2. Tell me about your education?
I have appeared std. 10th but couldn’t pass through. I was not much interested in studies.
3. Tell me about your life situations?
I got married at the age of 20 yrs. I have 1 daughter. I use to work in fields when I was all well.
Nature wise.., I get angry on lie or things against will, and when criticized or mortified. Usually I expressed it by weeping. Otherwise I am mild. I am selective about talking to people. I talk to people whom I like or else keep no relation. I am straight forward.
I myself permitted and carried out my husband’s 2nd marriage. I have a daughter. I wanted someone to look after her.
What I feel is why others should suffer for me. I don’t want things to spoil because of me. Even my husband must be feeling that his wife should be beautiful.
At times I do feel lonely.
Her husband added,
She is confident and manages problems well. She is not suppressed by anyone. She is dominating and courageous. She is Religious. Doesn’t like to mix with people d/t her disease. I share a Good relation with all family members.
4. Tell me about your worries?
I feel I will never recover. I am worried about my daughter’s future. (Who will take care of her?) What about her marriage when people get to know about my disease. I feel she shouldn’t suffer. This was the main reason for my husband’s 2nd marriage.
Totality:
- Anxiety conscience
- Inferior feeling.
- Anxiety future.
- Tough.
- Bold.
- Confident.
- C/o Rt. to Lt.
- Despair.
- Thick hardening of the skin.
- C/o Ascending.
- Darkening of skin.
- Thermal : Chilly.
- Miasm : Syphilitic.
Remedy Selected: ANACARDIUM 30.
Response :
It was a difficult condition to treat as when examined one could feel the hardness of the limbs as wooden log.
She was admitted for observation. Improvement was a gradual process. Initially development was seen in the wounds and the cracks which had started healing.
Over the period of next 6 months, hardness in the proximal part of upper limb and that of soles and foot had reduced.
Follow-up as on 2nd August 2010 :
Cracks and wounds were absent. Thickness of the proximal part of the limbs and that of foot and soles had reduced drastically. It appeared as good as normal.
She could expand her mouth to a greater degree.
She could walk normally as the wounds in the soles had completely reduced.
A latest follow up was taken dated to 7th August 2012.
She entered the cabin with a fast walk which helped me to conclude the skin of soles was back to normal.
Thickened skin had still more reduced so that there ease of movements of foot, hand, fingers, toes.
She could open her mouth well to eat comfortably.
Which other medicine could give better result than this?
D/D:
Alumina:
It is a very close match to pathophysiological presentation of the case. But the characters which differ from the case are as follows:
- The progress of side from Rt. To Lt. is not covered.
- The blackish discoloration in the skin of the patient is not well covered.
- Alumina is soft metal and so is the remedy. There is lot of confusion of idea, of identity.
- Alumina is a Ambithermal remedy and patient is Chilly.
Graphitis:
Thermally it is close to the patient but, there are following major differences:
- Mentally Graphitis people are soft, easily impressionable and have a strong weeping tendency. These
are timid people with low confidence. They have lot of anticipation.
Patient is strong, bold, hard at heart.