An unusual presentation of Whipple’s disease: adenopathies, polyarthralgia and dermatomyositis-like symptoms

Abstract

Whipple’s disease (WD) is a rare systemic disease caused by gram-positive bacillus bacteria that invades multiple organs mainly the intestinal epithelium. Its manifestation is not only limited to the gastrointestinal tract but it also affects the joints, muscle and skin. This is a case of a 54-year-old male patient with a medical history of chronic arthritis presenting with bilateral progressive calves pain, anterior tibial hyperpigmentation, joints pain, anemia and weight loss. He was misdiagnosed as rheumatoid arthritis, for which he was treated by immunosuppressors for several years with no amelioration. After advanced investigations, he was found to have multiple retroperitoneal and mesenteric adenopathies, with an incidental finding of a mesojejunal mass during laparoscopy, from which the biopsies revealed the presence of histiocytosis and numerous intra-cytoplasmic particles with positive periodic acid–Schiff (PAS) suggesting the diagnosis of WD. Endoscopy was done and intestinal histology with polymerase chain reaction (PCR) test confirmed the diagnosis of WD. The patient was then treated with antibiotics (ceftriaxone and trimethoprim-sulfamethoxazole) with a remarkable clinical amelioration. To be aware of WD as a potential etiology behind malabsorption, musculoskeletal and skin abnormalities, is the first step in order to establish the diagnosis and provide adequate treatment, thus, improving the patient’s quality of life. WD is a rare, without antibiotic treatment deadly systemic infectious disease caused by the ubiquitary Gram-positive bacterium Tropheryma whipplei. This article aims to report a case marked with dermatomyositis like presentation that had a missed and delayed diagnosis.