Background:Kikuchi disease (Kikuchi-Fujimoto disease, histiocytic necrotizing lymphadenitis) is a systemic illness of unkown etiology. It is characterized by cervical lymphadenopathy and fever. The skin is the most frequently affected extranodal organ. Cuta­neous Kikuchi-Fujimoto disease can mimic both clinically and histolo­gically skin lesions in lupus erythema­tosus, a disorder with which it seems to be closely related. A close follow up is required as systemic lupus erythema­tosus develop­ment has been reported.Main observation:We report a case of a 23-year-old woman, who was admitted to our depart­ment with high fever, skin lesions and arthralgia. Scaly and erythematous plaques were noted on both cheeks and earlobes. In pads of feet and hands and periungual area, multiple purple papules with a perilesional erythematous halo were evident. A thoraco­abdominal computed tomography scan revealed lateral cervical, mediastinal, paratracheal, subcarinal and submandi­bular lympha­denopathy. Excisional node biopsy was consisten with Kikuchi disease with skin involvement.Conclusion:It has to be kept in mind that Kikuchi disease is a differential diag­nosis in case of fever, lymphade­nopathy and lupus-like skin lesions. Skin lesions in this disease and may resemble clinically and histologically to those of subacute lupus erythema­tosus or systemic erythema­tosus lupus.