ORAL LESIONS IN A PATIENT WITH HYPER IMMUNOGLOBULIN M: DIFFERENTIAL DIAGNOSIS AND MANAGEMENT

ABSTRACT

Introduction: Hyper immunoglobulin M (HIGM) syndromes are a group of rare inherited disorders characterized by deficiencies in immunoglobulin class switch recombination (CSR). Patients with HIGM are predisposed to infectious, autoimmune, inflammatory, and malignant complications. We describe a case of a 21-year-old male with HIGM presenting with fever and severe oral ulcers. Case Description A 21-year-old male with HIGM due to a CD40L mutation on immunoglobulin therapy was admitted for 4-week history of painful erosive oral lesions, sore throat, and fever. He had deep ulcers on his hard palate, tonsils, and tongue that worsened despite treatment with valacyclovir and azithromycin as an outpatient. Neutrophil counts were normal. Blood and respiratory cultures were negative;one out of two throat fungal cultures showed Candida albicans. Biopsy of the ulcers was negative for fungal elements and HSV. COVID-19 PCR was negative, but COVID-19 IgM to the receptor-binding domain was positive (180). IgG was negative, which was expected with HIGM. The patient was treated with acyclovir, fluconazole, ceftriaxone, and metronidazole while undergoing infectious work-up. He continued to have fevers with no improvement in stomatitis. After adding prednisone, his ulcers and fever quickly resolved. Discussion: This remains an unclear case of chronic fevers and severe oral ulcers in a patient with HIGM. Candida albicans was unlikely to be the cause. Surprisingly, COVID-19 IgM was positive, suggesting that an infection had occurred. Mucosal involvement has been increasingly reported with COVID-19, and lesions may be more severe in patients with primary immunodeficiency. Steroids may be an effective therapy once other infections are excluded. [Formula presented]