Sclerodermia accompanied with interstitial cystitis: a case report
Interstitial cystitis/bladder pain syndrome (IC/BPS) is a chronic condition that has been defined as an unpleasant suprapubic sensation associated with the urinary bladder (pain, pressure, discomfort) accompanied by lower urinary tract symptoms of more than 6 weeks duration not due to infection or other causes.Although IC/BPS is more prevalent in women, it may affect men. Etiology is poorly defined and thus hampers both classification and treatment. Current treatments are not uniformly effective. Mast cell degranulation within the bladder wall has been proposed as a contributing factor in the inflammatory process, possibly related to histamine release.
We present a case report of a patient with Sclerodermia syndrome accompanied with interstitial cystitis. A 54-year-old woman complained of symptoms of the underlying disease from 1992 and urinary irritation symptoms in the past 2 years. Several courses of different types of antibiotics could not relieve her urinary irritation symptoms. Bladder hydraulic dilatation had only transient effects. Urine sediment contained neither erythrocytes nor leukocytes, and urine cultures yielded no growth. Cystoscopy revealed redness, edema, angiectasis, and extensive ecchymosis of the mucosal surface and several small floating cruor entities in the bladder. Urinary bladder biopsy specimens revealed the absence of urothelium and edematous lamina propria and submucosa, with diffuse or multiple focal chronic inflammatory cell infiltration. Based on these findings, she was diagnosed with Sclerodrmia syndrome accompanied with interstitial cystitis. Therapy with corticosteroids relieved the symptoms significantly.