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Laryngeal involvement with fatal outcome in progressive nodular histiocytosis: A rare case report

Progressive nodular histiocytosis (PNH) represents a very rare type of non-Langerhans cell histiocytosis. It is characterized by progressive appearance of papules and nodules without spontaneous resolution. We report a 60-year-old patient with novel clinical features in the form of extensive nodulou...

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Detalles Bibliográficos
Autores principales: Salunke, Aarti, Belgaumkar, Vasudha, Chavan, Ravindranath, Dobariya, Rinkesh
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5134168/
https://www.ncbi.nlm.nih.gov/pubmed/27990389
http://dx.doi.org/10.4103/2229-5178.193913
Descripción
Sumario:Progressive nodular histiocytosis (PNH) represents a very rare type of non-Langerhans cell histiocytosis. It is characterized by progressive appearance of papules and nodules without spontaneous resolution. We report a 60-year-old patient with novel clinical features in the form of extensive noduloulcerative lesions, ichthyotic patches, and laryngeal involvement culminating in fatal outcome prior to therapeutic intervention. Although the presenting features were baffling, histopathology and immunohistochemistry clinched the diagnosis of PNH.