PITYRIASIS LICHENOIDES - REPORT OF TWO CASES SHOWING THE WIDE SPECTRUM OF PRESENTATION OF THIS RARE DISEASE
Autor(a) principal: | |
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Data de Publicação: | 2015 |
Outros Autores: | , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://doi.org/10.29021/spdv.73.3.463 |
Resumo: | Pityriasis lichenoides is a rare skin disease of unknown etiology, whose spectrum of clinical presentation varies from an acute form, pityriasis lichenoides et varioliformis acuta (PLEVA) to a more insidious and recurrent one, pityriasis lichenoides chronica (PLC). PLEVA, or Mucha-Habermann disease, has an acute onset of papules on the trunk, buttocks and proximal extremities that rapidly progress to vesicles or form hemorrhagic crusts, and may leave depressed scars. PLC, by contrast, has small scaly papules, asymptomatic or pruritic, which can develop over days, on the same distribution. The lesions may continue appearing and disappearing over months, sometimes causing post-inflammatory hypopigmentation. The patient does not usually evolve from a clinical form to another, tending to spontaneous healing or the cure comes after treatment. It is believed that pityriasis lichenoides is a hypersensitivity reaction to various infectious agents and with self-limiting clonal proliferation of T cells, wherein the immune response to the clone leads to clinical and pathological manifestations. It has been detected T-cell receptor gamma gene rearrangement (TCR-gamma) by PCR. Pathological exam shows typical changes of each presentation. This paper presents two very representative cases of this uncommon disease, showing the main features of each form of pityriasis lichenoides. Both had resolution with the use of Doxycycline. |
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PITYRIASIS LICHENOIDES - REPORT OF TWO CASES SHOWING THE WIDE SPECTRUM OF PRESENTATION OF THIS RARE DISEASEPITYRIASIS LICHENOIDES - RELATO DE DOIS CASOS QUE EVIDENCIAM O VARIADO ESPECTRO DE APRESENTAÇÃO DESTA RARA DOENÇAPityriasis lichenoidesPitiríase liquenoidePityriasis lichenoides is a rare skin disease of unknown etiology, whose spectrum of clinical presentation varies from an acute form, pityriasis lichenoides et varioliformis acuta (PLEVA) to a more insidious and recurrent one, pityriasis lichenoides chronica (PLC). PLEVA, or Mucha-Habermann disease, has an acute onset of papules on the trunk, buttocks and proximal extremities that rapidly progress to vesicles or form hemorrhagic crusts, and may leave depressed scars. PLC, by contrast, has small scaly papules, asymptomatic or pruritic, which can develop over days, on the same distribution. The lesions may continue appearing and disappearing over months, sometimes causing post-inflammatory hypopigmentation. The patient does not usually evolve from a clinical form to another, tending to spontaneous healing or the cure comes after treatment. It is believed that pityriasis lichenoides is a hypersensitivity reaction to various infectious agents and with self-limiting clonal proliferation of T cells, wherein the immune response to the clone leads to clinical and pathological manifestations. It has been detected T-cell receptor gamma gene rearrangement (TCR-gamma) by PCR. Pathological exam shows typical changes of each presentation. This paper presents two very representative cases of this uncommon disease, showing the main features of each form of pityriasis lichenoides. Both had resolution with the use of Doxycycline.Pityriasis lichenoides é uma dermatose rara, de etiologia desconhecida, cujo espectro de apresentações clínicas varia de uma forma aguda, a pityriasis lichenoides et varioliformis acuta (PLEVA) a uma forma mais insidiosa e recorrente, a pityriasis lichenoides chronica (PLC). A PLEVA, ou doença de Mucha-Habermann, tem um quadro abrupto de pápulas no tronco, nádegas e extremidades proximais que progridem rapidamente para vesículas ou formam crostas hemorrágicas, podendo deixar cicatrizes deprimidas. Já a PLC apresenta pequenas pápulas descamativas, assintomáticas ou pruriginosas, que podem se desenvolver ao longo de dias, também com distribuição ao tronco, nádegas e extremidades proximais. As lesões podem continuar surgindo e involuindo ao longo de meses, algumas vezes causando hipopigmentação pós-inflamatória. O doente não costuma evoluir de um polo de apresentação para outro, tendendo a cura espontânea ou após tratamento. Acredita-se que a pitiríase liquenóide seja uma reação de hipersensibilidade a agentes infecciosos diversos, com proliferação clonal autolimitada de células T, em que a resposta imunológica ao clone provoca as manifestações clínicas e histopatológicas. Já foi identificado rearranjo dos receptores do gene gama de células T (TCR-gama) por técnicas de PCR. O anatomopatológico mostra alterações típicas de cada forma de apresentação. O artigo apresenta dois casos bem representativos desta rara doença, evidenciando as principais características de cada um dos polos da pitiríase liquenóide. Ambos tiveram resolução com o uso de Doxiciclina.Sociedade Portuguesa de Dermatologia e Venereologia2015-09-29T00:00:00Zjournal articleinfo:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://doi.org/10.29021/spdv.73.3.463oai:ojs.revista.spdv.com.pt:article/463Journal of the Portuguese Society of Dermatology and Venereology; Vol 73 No 3 (2015): Julho / Setembro; 383-387Revista da Sociedade Portuguesa de Dermatologia e Venereologia; v. 73 n. 3 (2015): Julho / Setembro; 383-3872182-24092182-2395reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPporhttps://revista.spdv.com.pt/index.php/spdv/article/view/463https://doi.org/10.29021/spdv.73.3.463https://revista.spdv.com.pt/index.php/spdv/article/view/463/361Sabino Sisnando, AlexandreAgonio, BárbaraMadeira, LaryssaFrancesconi, Fabioinfo:eu-repo/semantics/openAccess2022-10-06T12:34:54Zoai:ojs.revista.spdv.com.pt:article/463Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:10:54.149524Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
PITYRIASIS LICHENOIDES - REPORT OF TWO CASES SHOWING THE WIDE SPECTRUM OF PRESENTATION OF THIS RARE DISEASE PITYRIASIS LICHENOIDES - RELATO DE DOIS CASOS QUE EVIDENCIAM O VARIADO ESPECTRO DE APRESENTAÇÃO DESTA RARA DOENÇA |
title |
PITYRIASIS LICHENOIDES - REPORT OF TWO CASES SHOWING THE WIDE SPECTRUM OF PRESENTATION OF THIS RARE DISEASE |
spellingShingle |
PITYRIASIS LICHENOIDES - REPORT OF TWO CASES SHOWING THE WIDE SPECTRUM OF PRESENTATION OF THIS RARE DISEASE Sabino Sisnando, Alexandre Pityriasis lichenoides Pitiríase liquenoide |
title_short |
PITYRIASIS LICHENOIDES - REPORT OF TWO CASES SHOWING THE WIDE SPECTRUM OF PRESENTATION OF THIS RARE DISEASE |
title_full |
PITYRIASIS LICHENOIDES - REPORT OF TWO CASES SHOWING THE WIDE SPECTRUM OF PRESENTATION OF THIS RARE DISEASE |
title_fullStr |
PITYRIASIS LICHENOIDES - REPORT OF TWO CASES SHOWING THE WIDE SPECTRUM OF PRESENTATION OF THIS RARE DISEASE |
title_full_unstemmed |
PITYRIASIS LICHENOIDES - REPORT OF TWO CASES SHOWING THE WIDE SPECTRUM OF PRESENTATION OF THIS RARE DISEASE |
title_sort |
PITYRIASIS LICHENOIDES - REPORT OF TWO CASES SHOWING THE WIDE SPECTRUM OF PRESENTATION OF THIS RARE DISEASE |
author |
Sabino Sisnando, Alexandre |
author_facet |
Sabino Sisnando, Alexandre Agonio, Bárbara Madeira, Laryssa Francesconi, Fabio |
author_role |
author |
author2 |
Agonio, Bárbara Madeira, Laryssa Francesconi, Fabio |
author2_role |
author author author |
dc.contributor.author.fl_str_mv |
Sabino Sisnando, Alexandre Agonio, Bárbara Madeira, Laryssa Francesconi, Fabio |
dc.subject.por.fl_str_mv |
Pityriasis lichenoides Pitiríase liquenoide |
topic |
Pityriasis lichenoides Pitiríase liquenoide |
description |
Pityriasis lichenoides is a rare skin disease of unknown etiology, whose spectrum of clinical presentation varies from an acute form, pityriasis lichenoides et varioliformis acuta (PLEVA) to a more insidious and recurrent one, pityriasis lichenoides chronica (PLC). PLEVA, or Mucha-Habermann disease, has an acute onset of papules on the trunk, buttocks and proximal extremities that rapidly progress to vesicles or form hemorrhagic crusts, and may leave depressed scars. PLC, by contrast, has small scaly papules, asymptomatic or pruritic, which can develop over days, on the same distribution. The lesions may continue appearing and disappearing over months, sometimes causing post-inflammatory hypopigmentation. The patient does not usually evolve from a clinical form to another, tending to spontaneous healing or the cure comes after treatment. It is believed that pityriasis lichenoides is a hypersensitivity reaction to various infectious agents and with self-limiting clonal proliferation of T cells, wherein the immune response to the clone leads to clinical and pathological manifestations. It has been detected T-cell receptor gamma gene rearrangement (TCR-gamma) by PCR. Pathological exam shows typical changes of each presentation. This paper presents two very representative cases of this uncommon disease, showing the main features of each form of pityriasis lichenoides. Both had resolution with the use of Doxycycline. |
publishDate |
2015 |
dc.date.none.fl_str_mv |
2015-09-29T00:00:00Z |
dc.type.driver.fl_str_mv |
journal article info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://doi.org/10.29021/spdv.73.3.463 oai:ojs.revista.spdv.com.pt:article/463 |
url |
https://doi.org/10.29021/spdv.73.3.463 |
identifier_str_mv |
oai:ojs.revista.spdv.com.pt:article/463 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
https://revista.spdv.com.pt/index.php/spdv/article/view/463 https://doi.org/10.29021/spdv.73.3.463 https://revista.spdv.com.pt/index.php/spdv/article/view/463/361 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Sociedade Portuguesa de Dermatologia e Venereologia |
publisher.none.fl_str_mv |
Sociedade Portuguesa de Dermatologia e Venereologia |
dc.source.none.fl_str_mv |
Journal of the Portuguese Society of Dermatology and Venereology; Vol 73 No 3 (2015): Julho / Setembro; 383-387 Revista da Sociedade Portuguesa de Dermatologia e Venereologia; v. 73 n. 3 (2015): Julho / Setembro; 383-387 2182-2409 2182-2395 reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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