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18 July 2024

Dubai woman treated for rare disorder

By Staff

A Dubai hospital diagnosed and treated a rare blistering disorder in a 31-year-old female patient in just 48 hours.

The patient was presented with sudden onset of fever, severe oral ulcers and multiple blisters over the face and both arms and legs since a week. She neither had such episodes in the past nor had similar disorders in the family. She was the mother of two children born normally and had never experienced any rashes during pregnancy. She denied taking any drugs, allopathic, homeopathic or ayurvedic in the recent past for any illness.

Dermatological examination by Dr. Ramachandran Rajagopal, Specialist Dermatologist at Thumbay Hospital in Dubai, revealed a febrile, ill patient with swelling and redness of both eyes, swelling of face and lips with multiple small blisters around the mouth and large intact blisters over both arms, back and legs.

She was unable to walk due to the blisters on the thighs. She was also unable to open her mouth and eat normally.  Blisters were painful and the surrounding skin was red and burning.

The patient was investigated with a skin biopsy for the diagnosis of a rare disorder named “Linear IgA Dermatoses (Adult type)”.

Skin biopsy was sent for special tests like histopathology and immunofluorescence with the help of pathologist Dr. Ankur Malik of Thumbay Hospital. Treatment was initiated pending results.

The patient was admitted and treated with injectable steroids. There was dramatic response to the treatment and the patient became well in 48 hours and was discharged with advice to follow up in the OPD.

Steroids are being gradually reduced now. Blisters on skin and mouth have fully subsided and the patient is back to her routine office work.

The skin biopsy results confirmed the diagnosis of Linear IgA dermatoses (Adult type). The case is rare as it is found in only 1 in 2 million cases in Europe, though slightly more common in China, Malaysia, Thailand and Sri Lanka. Very few cases have been reported from India.  It usually occurs in children and when it rarely occurs in adults, the patients affected are over 60 year of age. The present case is unique in that it occurred in an adult just 31 years old.

This skin disorder is challenging to diagnose, as it can resemble infectious disorders like chickenpox in the early stages and other blistering disorders in the late stages.

Successful outcome depends on accurate diagnosis which is supported by skin biopsy and immunofluorescence results. The treatment was successful as there was immediate response to steroids, relieving the patient of the distressing skin and mouth blisters and ulcers.