My wife and I live in Trinidad. About two years ago, my 47-year-old wife started to get severe ulcers in her mouth. She was diagnosed at a London hospital after a biopsy as suffering from pemphigus. We were told that the treatment is to regulate outbreaks with steroids (prednisone up to 15mg daily currently). There doesn’t seem to be much alternative treatment here, and steroids are not a cure. The long-term side effects, especially the weight gain, has been very difficult for her. We would so appreciate your help and advice. H.V., Trinidad
Dr. Julian Schamroth, a veteran Jerusalem dermatologist, replies:
Pemphigus is an uncommon disorder of the skin characterized by severe and extensive blistering of the skin. It is more common in elderly Ashkenazi Jews and affects females more than males.
The disorder is caused by an autoimmune process in which the body makes antibodies against the fibers that anchor the skin to underlying tissues. Without these adhesive “anchors,” the skin becomes detached from the deeper skin layers and forms blisters. The same thing happens on mucosal surfaces, such as the mouth, vagina or urethra, but the blisters are rarely seen as they rapidly break down to leave ulcers. There are several different forms of the disease – pemphigus vulgaris being the most common.
In extreme cases – especially if left untreated – secondary infections can occur and the disorder can be life-threatening. The standard therapy involves topical steroid creams as well as oral cortisone. High doses of cortisone doe have side effects, but this can be minimized by taking a “cortisone-sparing” drug such as azothiaprine (Imuran) or other immune-suppressant drugs. (Imuran is used worldwide and is probably available in the West Indies). A newer biological agent called retuximab (Rituxan) is available for severe life-threatening cases.
All of the above drugs have side effects, mild as well as serious. It is thus imperative that patients with pemphigus be closely monitored by their dermatologist.
We’re desperate to get some help for our nephew, Valentino. He is a fun-loving, 15-year-old boy who loves gymnastics, running, soccer, video games and hanging out with his friends. But during the last eight months, he has been in constant pain and has lost the ability to walk unaided. He no longer sees his friends and is unable to go to school and enjoy the things a normal boy of his age would do. The family have spent months visiting a general practitioners and hospitals via Britain’s National Health Service, but so far, they have been unable to find a diagnosis so doctors can even start to treat his condition. Various specialists have suggested gastritis, Guillain Barré Syndrome, Lyme disease and chronic fatigue syndrome, but so far none have conclusively arrived at a diagnosis. After eight months and almost $26,000 spent on treatments and remedies already, Valentino’s health is still declining, and no answers are forthcoming. All the family’s savings have run out. Valentino wasn’t even able to hold a conversation without exhausting himself, he was taken to a hospital emergency room. An x-ray of his chest and an ECG were performed, but the results came back normal. He was diagnosed with gastritis. Within a week, Valentino went back to the GP; the GP agreed with the emergency room doctor’s diagnosis of gastritis and prescribed Omeprazole to treat acid reflux. Valentino finished the course in two weeks with no improvement, in fact, he was getting weaker by the day.
After further visits to the GP, he was referred to the pediatric team at the hospital. He had a series of blood tests and examinations all returning normal. With no improvement or diagnoses from the doctors, and Valentino’s parents desperate for answers they took Valentino to a private pediatrician. He examined Valentino and could find no problem but increased his dosage of Omeprazole to rule out any esophageal issues. His condition deteriorated even further. He now needed support to walk around the house, using furniture or a family member to support him.
His parents took him to a neurologist, who carried out a thorough physical examination. Again, everything came back normal. He was sent for a muscle and nerve test, and again, all came back normal. By this point the funds were quickly dwindling; so rather than spending $1,000 to carry out this test in the UK, the family traveled to Cyprus to have this test carried out there for less than a quarter of the price.
The family traveled with Valentino to a cardiologist and professor of medicine in Cyprus. It was suggested that they take him to see a holistic doctor in Cyprus. He carried out a full body examination. He carried out a test using a Vega machine and detected possible liver toxicity and Lyme disease. This holistic doctor recommended a course of supplements to strengthen his immune system to fight off any toxicity or infections in his body.
After almost 4 months taking these supplements, no changes or improvements have been seen. Back at home, he also had a brain and spinal MRI – results came back normal. A physiotherapist gave Valentino exercises to try and reduce muscle wastage in his legs, as he wasn’t able to walk, and agreed that he would need weekly visits with a physiotherapist and psychologist. Continuing with the supplements prescribed by the holistic doctor, a further visit with him in August showed zero improvement.They took him in September to a consultant medical herbalist in Harley Street, London. Blood was taken here and sent to the US and Germany for screening. She believes that the infections attacking his nervous system could possibly be caused by mold, but nothing was proven. He is still without a diagnosis or treatment. What can they do now? Via Internet
Prof. Eitan Kerem, chief of pediatrics at Jerusalem’s Hadassah Medical Center, comments:
I consulted with Dr. Tal Gilboa, head of pediatric neurology at our hospital. We note that after repeated physical and neurological findings are normal, normal brain and spine MRI, normal EMG & NCV more than than weeks after symptoms appeared, normal EEG, negative cultures, normal CSF, normal blood tests including thyroid function and negative serological and immunological panels, we see no signs for an organic disease. We suggest to consider a psychology evaluation.
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Source: Israel in the News