Pyoderma gangrenosum and chronic granulomatous disease treated with adalimumab: Case-based review

Abstract

BackgroundChronic granulomatous disease (CGD) is a rare immunodeficiency disorder that manifested by recurrent severe bacterial and fungal infection. It may be associated with non-infectious inflammatory complications that affect gastrointestinal tract, lung and skin. Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterizes by painful cutaneous ulceration. The coexistence of PG and CGD is very rare and the treatment is challenging; only few cases have been reported. Aim of the workTo describe the features and management of a challenging case of PG associated with CGD. Case reportA 20-year old male with a long history of recurrent bacterial and fungal infections due to CGD presented to the Rheumatology and Immunology outpatient clinic at Mansoura University hospital with an ulcerative cutaneous lesion with exudation and crusting on the left lateral malleolus. There were elevated inflammatory markers: erythrocyte sedimentation rate (ESR) 130 mm/1st h and C-reactive protein (CRP) 52 mg/dl. Histological examination of the lesion revealed diffuse dermal infiltrate of neutrophils and nuclear dust admixed with some histiocytes, lymphocytes and plasma cells, a picture suggestive of PG. Systemic and musculoskeletal examination were unremarkable except for low grade fever (37.7 °C). Conventional immunosuppressives were not satisfactory and adalimumab (40 mg/2 weeks) was added showing significant improvement of the lesion (ESR 7 mm/h, CRP 3 mg/dl). ConclusionCGD is a rare primary immunodeficiency disorders that can be associated with PG. The coexistence of these two conditions requires judicious use of immunosuppressive agents. However; adalimumab may be an effective and safe treatment option.