Mucositis in a patient from the mediterrranean; More than just Behçet’s disease.
S.M.W. Koop
Voorzitter(s): prof. dr. J.W. de Fijter, LUMC, Leiden & dr. W.G. Meijer, WestFries Gasthuis, Hoorn
Woensdag 22 april 2015
15:00 - 16:00u
in Auditorium 1
Categorieën: parallelsessie (case reports/research)
Parallel sessie: Parallelsessie 1: Case reports/research
A 59 year old Turkish man presented himself with complaints of severe painful mucositis since 3 months. A trial treatment with prednisone had had some effect, however after treatment was discontinued symptoms worsened again. The patient had also recently developed skin lesions complained of a painful red eye. Physical examination showed severe ulcerations on lips, tong and palate. Chest, abdomen, hand palms and glans penis showed multiple erythematous and vesiculous lesions with a diameter up to 5mm. His eyes showed signs of conjunctivitis. The combination of oral and genital ulcers and eye symptoms made us suspect Behçet’s disorder as a possible explanation. However the criteria for Behçet require the absence of other systemic disorders. Infectious diseases like Lues and HIV were excluded. Systemic inflammatory disorders like SLE and Crohn’s disease were deemed less likely due to the absence of gastro-intestinal disorders and arthritis. Auto-immune markers like ANA and ANCA were negative.
A skin biopsy was taken for further analysis of the skin lesions. This showed evidence of pemphigus vulgaris, with a preference for paraneoplastic pemphigus. Imaging of thorax and abdomen was obtained showing multiple masses around thoracal and abdominal aorta, around the spleen and in the retroperitoneum. Biopsy of the retroperitoneal lesion showed a low grade B-cellymphoma stage III, most likely marginal zone lymphoma. Treatment with R-CVP has been initiated.
Oral ulceration in patient from the Mediterranean can point to more diseases than just Behcet’s disease and excluding these disorders is a viable part in the diagnostic process.
