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LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY
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...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Sociedade de Pediatria de São Paulo
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5417814/ https://www.ncbi.nlm.nih.gov/pubmed/28977321 http://dx.doi.org/10.1590/1984-0462/;2017;35;1;00015 |
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author | Afsar, Fatma Sule Ergin, Malik Ozek, Gulcihan Vergin, Canan Karakuzu, Ali Seremet, Sila |
author_facet | Afsar, Fatma Sule Ergin, Malik Ozek, Gulcihan Vergin, Canan Karakuzu, Ali Seremet, Sila |
author_sort | Afsar, Fatma Sule |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-5417814 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Sociedade de Pediatria de São Paulo |
record_format | MEDLINE/PubMed |
spelling | pubmed-54178142017-05-24 LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY Afsar, Fatma Sule Ergin, Malik Ozek, Gulcihan Vergin, Canan Karakuzu, Ali Seremet, Sila Rev Paul Pediatr Case Reports 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. Sociedade de Pediatria de São Paulo 2017 /pmc/articles/PMC5417814/ /pubmed/28977321 http://dx.doi.org/10.1590/1984-0462/;2017;35;1;00015 Text en http://creativecommons.org/licenses/by/4.0/ Este é um artigo publicado em acesso aberto sob uma licença Creative Commons |
spellingShingle | Case Reports Afsar, Fatma Sule Ergin, Malik Ozek, Gulcihan Vergin, Canan Karakuzu, Ali Seremet, Sila LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY |
title | LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY |
title_full | LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY |
title_fullStr | LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY |
title_full_unstemmed | LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY |
title_short | LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY |
title_sort | late-onset self-healing langerhans cell histiocytosis: report of a very rare entity |
topic | Case Reports |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5417814/ https://www.ncbi.nlm.nih.gov/pubmed/28977321 http://dx.doi.org/10.1590/1984-0462/;2017;35;1;00015 |
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