Open AccessLaryngeal involvement with fatal outcome in progressive nodular histiocytosis: A rare case report
Author(s) -
Aarti S. Salunke,
Vasudha Abhijit Belgaumkar,
Ravindranath B. Chavan,
Rinkesh Dobariya
Publication year - 2016
Publication title -
indian dermatology online journal
Language(s) - English
Resource type - Journals
eISSN - 2249-5673
pISSN - 2229-5178
DOI - 10.4103/2229-5178.193913
Subject(s) - medicine , histiocytosis , histopathology , langerhans cell histiocytosis , pathology , dermatology , histiocytosis x , disease
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.