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Manifestations of chronic cutaneous lupus erythematosus are variable. Periorbital and facial swelling occurs in dermatomyositis and systemic lupus, but it has been rarely reported as a manifestation of exclusively cutaneous lupus. A 48-year-old woman presented with a 16-year history of asymptomatic, bilateral swelling and erythema of her face with marked worsening after sun exposure. No systemic symptoms were associated. A complete evaluation did not reveal other findings. Cutaneous biopsy showed features of lupus erythematosus. She was treated with photoprotection, topical tacrolimus, hydroxychloroquine and azathioprine with a partial response. Facial swelling with erythema represents quite an unusual manifestation of chronic cutaneous lupus erythematosus. Dermatomyositis, systemic lupus and Morbihan disease are the main differential diagnoses., Learning Points: Periorbital and facial swelling with erythema are clinical manifestations of dermatomyositis and systemic lupus erythematosus. However, these manifestations represent quite an unusual presentation of chronic cutaneous lupus erythematosus.The periorbital area is most frequently affected, while extensive facial involvement is much more unusual.A complete evaluation and cutaneous biopsy are essential to make the diagnosis and to rule out other disorders such as dermatomyositis, systemic lupus erythematosus and Morbihan disease., Competing Interests: Conflicts of Interests: The Authors declare that there are no competing interest, (© EFIM 2020.)

Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a syndrome characterized by the presence of usually painful cervical lymphadenopathy with fever and night sweats. It is a rare clinical entity that mainly affects young Asian women, although it found worldwide. It is a benign and self-limiting condition; however, its importance lies in its differential diagnosis with other clinical entities such as lymphoma or histiocytoma. We present a case of Kikuchi-Fujimoto disease, with special emphasis on its clinicopathological significance and its evolution during outbreaks. Antimalarial therapy causes remission and we discuss the use of hydroxychloroquine for reoccurring cases., (Copyright © 2016 Sociedad Española de Anatomía Patológica. Publicado por Elsevier España, S.L.U. All rights reserved.)