• Print ISSN: 2395 - 1400, Online ISSN: 2455 - 8923

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Case Report

Autoimmune lymphoproliferative syndrome presenting as facial panniculitis in a young boy - A case report

*1Professor, Department of Pathology, 2Assistant professor, 3Professor and Head, Department of Dermatology and Venereology, Kamineni Academy of Medical Sciences and Research Centre, LB Nagar, Hyderabad, Telangana State, India.


A fourteen year old boy presented with gradually enlarging pigmented cutaneous lesions of face and cervical lymphadenopathy for six months. He was evaluated at other hospitals with fine needle aspiration cytology (FNAC) of the parotid gland and later with a guided FNAC of the right external iliac lymph node. He came to our institute due to persistence of the lesions and was evaluated with a skin biopsy and cervical lymph node biopsy. The skin biopsy showed features of panniculitis with emperipolesis. The lymph node revealed prominent emperipolesis and follicular hyperplasia, suggestive of Rosai-Dorfman disease (RDD). Considering the patient’s clinical presentation, previous serological and imaging findings, and the present biopsy reports, a diagnosis of autoimmune lymphoproliferative syndrome was given, and genetic studies for the same were suggested. This case report is presented to draw attention to this rare entity and more so to its unusual presentation as facial panniculitis.

Key Words: Autoimmune lymphoproliferative syndrome, Panniculitis, Rosai-Dorfman disease.