Kikuchi-Fujimoto disease is a rare necrotizing histiocytic lymphadenitis that typically affects young women and presents with febrile cervical lymphadenopathy. Because of its clinical and histopathological overlap with lupus adenitis and lymphoma, diagnosis may be challenging. We report the case of a 14-year-old girl admitted for prolonged fever and painful cervical lymphadenopathy associated with striking mucocutaneous manifestations. Dermatologic examination revealed massive bilateral periorbital edema leading to near-complete eyelid closure, diffuse centrofacial erythema forming infiltrated plaques, a morbilliform maculopapular eruption, malar butterfly erythema sparing the nasolabial folds, and painful erosive crusted cheilitis. Laboratory investigations showed lymphopenia, anemia, thrombocytopenia, hyperferritinemia, and elevated lactate dehydrogenase levels. Autoimmune workup revealed positive antinuclear antibodies and anti-double-stranded DNA antibodies with low complement levels. Imaging demonstrated cervical and axillary polyadenopathy associated with moderate ascites. Lymph node biopsy confirmed Kikuchi-Fujimoto disease, while skin biopsy supported the diagnosis of systemic lupus erythematosus with cutaneous manifestations. The clinical course was complicated by encephalopathy requiring treatment with high-dose corticosteroids, intravenous immunoglobulins, hydroxychloroquine, and etoposide, leading to progressive improvement. This case highlights a possible association between Kikuchi-Fujimoto disease and systemic lupus erythematosus with cutaneous manifestations. It emphasizes the key diagnostic value of dermatologic examination in identifying associated connective tissue disease and guiding early multidisciplinary management.
Hakim et al. (Wed,) studied this question.