Kikuchi‐Fujimoto’s Disease with Skin Manifestations

A fourteen‐year‐old female presented with persistent fever, abdominal pain, splenomegaly and multiple intra‐abdominal lymphadenopathies. She underwent an exploratory laparotomy. Biopsies of the mesenteric lymph nodes revealed necrotizing histiocytic lymphadenitis, characterized by para‐cortical fibrinous necrosis with karyorrhectic debris and histiocytic palisading, and paucity of the polymorphic neutrophils. She also developed concurrent skin lesions with scattered small non‐itching vesicles and blisters over her back and lower legs. A punch biopsy revealed vacuolar interface changes, sub‐epidermal blister formation, mild superficial perivascular infiltration, and follicle necrosis. Karyorrhectic debris and CD68‐positive cells are noted around the follicle. A direct immunofluorescent study was negative for complement or immunoglobulin depositions. The patient was diagnosed as Kikuchi‐Fujimoto’s disease, or necrotizing histiocytic lymphadenitis, a benign self‐limited lymph node disorder most commonly occurred in young females. Patients with Kikuchi’s disease are typically presented with isolated cervical lymphadenopathy with or without accompanying fever. Multiple lymph node involvement, splenomegaly and/or cutaneous lesions are rare occurrences. The present patient experienced a severe inflammatory response syndrome manifested by respiratory failure, pancytopenia, and disseminated intravascular coagulopathy. She recovered completely after two weeks of intensive medical treatment. She has had no respiratory symptoms, residual lymphadenopathies or documented skin lesions in her follow‐up three months after discharge.