Cargando…
Paraneoplastic Pemphigus: An Indication for Treatment in Chronic Lymphocytic Leukemia
Paraneoplastic disorders are rare multiorgan diseases associated with hematological malignancies such as chronic lymphocytic leukemia (CLL). Some of these paraneoplasms manifest as cutaneous lesions, appearing as a simple rash, ulcers or skin thickening. The pathogenesis for this process has been de...
Autores principales: | , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2020
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7320649/ https://www.ncbi.nlm.nih.gov/pubmed/32607299 http://dx.doi.org/10.7759/cureus.8316 |
_version_ | 1783551284982317056 |
---|---|
author | Onukogu, Ifeanyichukwu Ramachandran, Preethi Narh, Joshua Sahni, Sonu Joseph, Gardith |
author_facet | Onukogu, Ifeanyichukwu Ramachandran, Preethi Narh, Joshua Sahni, Sonu Joseph, Gardith |
author_sort | Onukogu, Ifeanyichukwu |
collection | PubMed |
description | Paraneoplastic disorders are rare multiorgan diseases associated with hematological malignancies such as chronic lymphocytic leukemia (CLL). Some of these paraneoplasms manifest as cutaneous lesions, appearing as a simple rash, ulcers or skin thickening. The pathogenesis for this process has been described as development of certain autoimmune reactions against cell wall antigens and proteins. An example is paraneoplastic pemphigus (PNP) which manifests as cutaneous bullae. Bullae may occur anytime during the course of the malignancy i.e. acute phase or remission. Diagnosis involves evaluation of clinical findings, serology and presence of characteristic histological findings. Its pathogenesis is described as development of auto-antibodies against cell junctional and basement membrane proteins. Presence of paraneoplasms has been associated with poorer prognosis and increased mortality in hematological malignancies including CLL. Currently, there are established indications for the treatment of CLL; however, presence of paraneoplasms as an indication for treatment is unclear. Patients with paraneoplasms who underwent expeditious treatment have exhibited better clinical outcomes. Herein we describe a case of a CLL patient in remission presenting with PNP and its response to treatment. |
format | Online Article Text |
id | pubmed-7320649 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
spelling | pubmed-73206492020-06-29 Paraneoplastic Pemphigus: An Indication for Treatment in Chronic Lymphocytic Leukemia Onukogu, Ifeanyichukwu Ramachandran, Preethi Narh, Joshua Sahni, Sonu Joseph, Gardith Cureus Dermatology Paraneoplastic disorders are rare multiorgan diseases associated with hematological malignancies such as chronic lymphocytic leukemia (CLL). Some of these paraneoplasms manifest as cutaneous lesions, appearing as a simple rash, ulcers or skin thickening. The pathogenesis for this process has been described as development of certain autoimmune reactions against cell wall antigens and proteins. An example is paraneoplastic pemphigus (PNP) which manifests as cutaneous bullae. Bullae may occur anytime during the course of the malignancy i.e. acute phase or remission. Diagnosis involves evaluation of clinical findings, serology and presence of characteristic histological findings. Its pathogenesis is described as development of auto-antibodies against cell junctional and basement membrane proteins. Presence of paraneoplasms has been associated with poorer prognosis and increased mortality in hematological malignancies including CLL. Currently, there are established indications for the treatment of CLL; however, presence of paraneoplasms as an indication for treatment is unclear. Patients with paraneoplasms who underwent expeditious treatment have exhibited better clinical outcomes. Herein we describe a case of a CLL patient in remission presenting with PNP and its response to treatment. Cureus 2020-05-27 /pmc/articles/PMC7320649/ /pubmed/32607299 http://dx.doi.org/10.7759/cureus.8316 Text en Copyright © 2020, Onukogu et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Dermatology Onukogu, Ifeanyichukwu Ramachandran, Preethi Narh, Joshua Sahni, Sonu Joseph, Gardith Paraneoplastic Pemphigus: An Indication for Treatment in Chronic Lymphocytic Leukemia |
title | Paraneoplastic Pemphigus: An Indication for Treatment in Chronic Lymphocytic Leukemia |
title_full | Paraneoplastic Pemphigus: An Indication for Treatment in Chronic Lymphocytic Leukemia |
title_fullStr | Paraneoplastic Pemphigus: An Indication for Treatment in Chronic Lymphocytic Leukemia |
title_full_unstemmed | Paraneoplastic Pemphigus: An Indication for Treatment in Chronic Lymphocytic Leukemia |
title_short | Paraneoplastic Pemphigus: An Indication for Treatment in Chronic Lymphocytic Leukemia |
title_sort | paraneoplastic pemphigus: an indication for treatment in chronic lymphocytic leukemia |
topic | Dermatology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7320649/ https://www.ncbi.nlm.nih.gov/pubmed/32607299 http://dx.doi.org/10.7759/cureus.8316 |
work_keys_str_mv | AT onukoguifeanyichukwu paraneoplasticpemphigusanindicationfortreatmentinchroniclymphocyticleukemia AT ramachandranpreethi paraneoplasticpemphigusanindicationfortreatmentinchroniclymphocyticleukemia AT narhjoshua paraneoplasticpemphigusanindicationfortreatmentinchroniclymphocyticleukemia AT sahnisonu paraneoplasticpemphigusanindicationfortreatmentinchroniclymphocyticleukemia AT josephgardith paraneoplasticpemphigusanindicationfortreatmentinchroniclymphocyticleukemia |