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LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY.

Authors :
Afsar, Fatma Sule
Ergin, Malik
Ozek, Gulcihan
Vergin, Canan
Karakuzu, Ali
Seremet, Sila
Source :
Revista Paulista de Pediatria. Jan-Mar2017, Vol. 35 Issue 1, p115-119. 5p.
Publication Year :
2017

Abstract

Objective: To report a case of late-onset self-healing Langerhans cell histiocytosis. Case description: A 4½-month-old female patient presenting with an eythematopurpuric eruption underwent a skin biopsy for histopathology and was first diagnosed with isolated cutaneous Langerhans cell histiocytosis. Her lesions regressed within a few months and she was retrospectively diagnosed with late-onset self-healing Langerhans cell histiocytosis after being without skin or systemic involvement in a follow-up four years later. Comments: Self-healing Langerhans cell histiocytosis, which is characterized by clonal proliferation of Langerhans cells and presents with cutaneous lesions, is a rare self-limited variant of histiocytosis and can only be diagnosed retrospectively, after the patient remains free from systemic involvement for several years. Although it presents at birth or during the neonatal period, only a few cases of its late-onset type regarding the age of onset have been reported. Purpuric lesions that appear after the neonatal period serve as a clue for late-onset self-healing Langerhans cell histiocytosis and the patients should be monitored regularly for systemic involvement if the diagnosis is confirmed by a cutaneous biopsy. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
01030582
Volume :
35
Issue :
1
Database :
Academic Search Index
Journal :
Revista Paulista de Pediatria
Publication Type :
Academic Journal
Accession number :
122537334
Full Text :
https://doi.org/10.1590/1984-0462/;2017;35;1;00015