Call me Behcet’s Syndrome

Tiffany* and Kayla* could not wait to get to grandma’s house. The long Covid-19 lockdown measures had separated the family for two years, connecting only online. The girls, nine and seven, looked forward to the freedom to roam the expansive farm, inhale the crisp Nandi Hills air and climb trees to pick fresh fruits. 

From the moment they alighted from grandma’s green jalopy, they did not waste any time. They were out checking on grandma’s newborn lambs, the delicate chicks and the kittens that had barely opened their eyes. They had eyes only for the young ones. Their mother, Sheila*, was happy to sit under the tree all day catching up with grandma on village gossip while regaling her with stories of the dramatic life in West Africa. 

The idyllic time was brought to a rude halt on the fourth day when Tiffany woke up with angry pink lesions on her shins and ankles. It did not help that her skin was extremely fair. The real scare though, resulted from the painful, itchy rash around her vulva that caused her to yelp in pain when she passed urine. Her groin was on fire and this was only calmed down by washing with cold water. 

Breakfast was forgotten and they all piled into the car and drove 35km to the nearest paediatrician. Tiffany was otherwise well, save for the rashes and her lab tests were all normal. She was given antibiotics and pain medications because the paediatrician wasn’t quite sure what was going on, and was scheduled for review within 48 hours.

The next morning when Tiffany woke up, the rash had now spread to her mouth and the rash in the vulva was breaking down into shallow ulcers that were even more painful. Sheila sprang into action and they were all on a flight back to the city to seek a second opinion.

Things moved very fast and by nightfall, Tiffany was admitted in the hospital with a urinary catheter in place to prevent urine from coming into contact with the vulval lesions. She was on strong pain medications, antibiotics, mouthwash, intravenous fluids and sitz baths every four hours to relieve her discomfort. She was under the care of a team of doctors trying to decipher what was ailing her. When I met her, Tiffany had only one request to make, she wanted to go home, sleep in her bed and drink oxtail soup. She had barely been in the ward for 24 hours and she was already tired, yet we still did not have a diagnosis.

The lesions in her vulva had now turned purple while the ones on her shins progressively healed. She wasn’t able to eat much because of the mouth ulcers, just bland soups and lots of ice cream. I was grossly challenged. All her tests remained normal, save for indicating that she had ongoing inflammation. I ate humble pie and went back to the books. Tiffany was suffering from Behcet’s Syndrome! 

Back to the bedside and further discussions with the family elicited some symptoms that had failed to raise a red flag in the past. Tiffany had recurrent eye inflammation that was mild and would clear off on its own. They’d thought it was an allergy. Thankfully it had never progressed to involve the inner eye, a serious complication that could affect her vision.

Tiffany was also spared the other more serious manifestations such as involvement of the joints in form of arthritis; irritable bowel syndrome arising from gut involvement; blood vessels inflammation and clot formation that could cause pulmonary embolism; or, more seriously, brain and peripheral nerve inflammation that could cause meningitis, stroke, seizures or paralysis. 

It was extremely difficult for the family. Tiffany’s dad flew in and we held a family conference to explain the illness. The hardest part for them to accept was that this was a lifelong condition that Tiffany would have to deal with. It was harder to accept that the symptoms could recur, get worse or that there was nothing to prevent development of new ones not manifested so far. 

Tiffany made a full recovery in a week, with the help of steroid treatment, and was able to salvage what was left of their holiday. She may suffer from a condition belonging to the rare diseases category but for now, she is just a lively nine-year old living a full life!

Dr Bosire is an obstetrician/gynaecologist