Thursday, November 19, 2015

STORY ABOUT PEMPHIGOID GESTATIONIS OR AS IT WAS KNOWN BEFORE-HERPES GESTATIONIS

This story is about Mary a young mother of 26 years of age. She had a normal first pregnancy, two years ago. There was no history of any problems associated with that pregnancy. Her current pregnancy was going well. When she presented at 32 weeks for her routine antenatal visit she complained of severe itching and puritis around the belly button.  She also had a few blisters also around the belly button. There was some redness in her thighs as well. She said she was starting to itch very severely when I looked as these blisters; I immediately thought of a condition called herpes gestations or pemphigoid gestationis (PG). This is a very rare skin disorder which occurs during pregnancy only. It has nothing to do with ordinary Herpes. It’s frequency is quoted to be 1 in 70 000, or 1 in 2 million. I had never seen a case before, or ever since, but her very intense puritis and blistering around the belly button made me think of this diagnosis.


I urgently referred her to a colleague of mine, a skin specialist. He soon confirmed the diagnosis. The diagnosis is confirmed by skin biopsy and a technique called immunoflorescence test to look for antibodies. This may be a bit technical for you, please do not worry about it. PG is an autoimmune disease of pregnancy. There are many other diseases in our body which are autoimmune. Autoimmune diseases are a range of disorders, when an individual’s immune system produces an inappropriate response to its own cells resulting in damage to the tissues for example:  Rheumatoid arthritis. In PG, antibodies form in the skin, against a protein between the epidermis (the outer layer of skin) and dermis (the inner layer of skin) and then destroy the skin. What triggers this reaction is not understood. This diagnosis was confirmed by my skin specialist colleague. He advised the usual treatment of local Corticosteroids for itching. She also had some systemic Oral cortisol. To suppress the severity of the problems, she was also given some intravenous Immunoglobulin’s. These help to make the disease a bit milder.  There are many other drugs on the market which can be used for this purpose but they were not required in her case. The other complications in this situation are; premature delivery, foetal growth restriction, secondary infection and scarring. I watched her for all these. An ultrasound was performed every two weeks to make sure that the baby was growing well. She managed well with the treatment; however she did get some rashes on her thighs. She did not have any associated disorders. The main disorder that PG is associated with is autoimmune disorders of the thyroid gland, Graves’s disease (overactive thyroid) or underactive thyroid such as Hashimotos disease. Another condition that can happen and make pregnancy difficult is pernicious anaemia. Other skin disorders are not often confused with PG as the nature of the rash is different and the symptoms are different.

An ultrasound at 36 weeks suggested that the foetal growth was being compromised as the fluid around the baby was decreased more than expected. I induced her at the first opportunity by normal induction method. Her labour was easy; a male baby weighing 2.63Kg was born in good condition. The baby had a few rashes on the abdomen. This can happen in 5-10 % of babies, as the antibodies can pass through the placenta .She did not have any trouble in the post partum period as the rash had cleared within 3 weeks. She was discharged home happily. I did advise her not to use oral contraceptives as PD can recur with oral contraceptive. It can also recur during menstrual cycle. I fitted her with an IUCD for contraception. To the best of my knowledge she never had any further problems or another baby. I was very pleased with myself for making this very rare diagnosis in a flash and had a comfortable mother and a healthy and happy baby.

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