Paraneoplastic pemphigus appearing as bullous pemphigoid-like eruption after palliative radiation therapy (original) (raw)

Lesions of pemphigus vulgaris on irradiated skin

Clinical and Experimental Dermatology, 2009

Pemphigus vulgaris (PV) is an autoimmune blistering disease produced by IgG autoantibodies against desmoglein (Dsg)3. Lesions on the skin and mucosa can, in rare cases, be induced by radiotherapy. We report a patient with a history of microprolactinoma and PV, who had only oral lesions from the beginning of her illness but 2 months after treatment with radiotherapy for a breast neoplasia, developed skin lesions limited to the irradiated area. Over the following few months, she also developed autoantibodies against Dsg1.

A Case of Bullous Pemphigoid Exacerbated by Irradiation After Breast Conservative Radiotherapy

Japanese Journal of Clinical Oncology, 2011

We present a case, considered to be a form of the Koebner phenomenon, of bullous pemphigoid that was exacerbated mainly within the irradiated field after breast conservative radiotherapy. In May 2009, a 60-year-old woman was diagnosed with bullous pemphigoid, which was treated with steroid therapy. The following month, she was diagnosed with breast cancer (invasive ductal carcinoma, pT1cN0M0). After breast conservative surgery in December 2009, conservative radiotherapy to the right breast was performed (50 Gy in 25 fractions). Portal skin showed no serious change (up to grade 1 skin erythema) and no bullous neogenesis during conservative radiotherapy. However, 2 months after conservative radiotherapy, new blisters became exacerbated mainly within the irradiated field but also in the area outside the irradiated field. Increasing the dosage of oral steroid and minocycline resulted in relief of bullous pemphigoid, although patchy skin pigmentation remained especially in the irradiated skin.

Localized bullous pemphigoid as an unusual complication of radiation therapy

Journal of the European Academy of Dermatology and Venereology, 2002

1 Behrens GM, Meyer D, Stoll M, Schmidt RE. Immune reconstitution syndromes in human immuno-deficiency virus infection following effective antiretroviral therapy. Immunobiology 2000; 201 : 186 -193. 2 Collazos J, Mayo J, Martinez E, Blanco MS. Contrast-enhancing progressive multifocal leukoencephalopathy as an immune reconstitution event in AIDS patients. AIDS 1999; 13 : 1426 -1428. 3 Bouscarat F, Maubec E, Matheron S, Descamps V. Immune recovery inflammatory folliculitis. AIDS 2000; 14 : 617-618. 4 Handa S, Bingham JS. Dermatological immune restoration syndrome: does it exist? J Eur Acad Derm Ven 2001; 15 (5): 430 -432. 5 Domingo P, Torres OH, Ris J, Vasquez G. Herpes zoster as an immune reconstitution disease after initiation of combination antiretroviral therapy in patients with human immunodeficiency virus type-1 infection. Am J Med 2001; 110 : 605 -609. 16 Letter Letter Letter xxxxx Report of a case of Muir-Torre syndrome To the Editor Muir-Torre syndrome (MTS) is an autosomal dominant genodermatosis, characterized by at least one single sebaceous gland tumour and a minimum of one internal malignancy. 1 In a recent review of the literature, Akhtar et al . found only 205 cases of MTS. 2 The most common presentation is sebaceous tumours with a low-grade visceral malignancy. 1

Paraneoplastic Pemphigus Case Report

Paraneoplastic pemphigus is a rare autoimmune bullous dermatosis. It occurs more frequently around the age of 60 and is most commonly associated with lymphomas and leukaemia. This particular form of pemphigus has issues that are clinically, histopathologically and immunopathologically distinct from other forms of pemphigus. Severe mucosal erosions and a polymorph rash are the clinical manifestations. We report a case of paraneoplastic pemphigus diagnosed in the Clinical Hospital of Sibiu which allowed us to present a literature update of this subject. Keywords: pemphigus, chronic lymphocytic leukaemia, paraneoplastic Rezumat: Pemfigusul paraneoplazic este o dermatoză buloasă autoimună rar întâlnită. Apare mai frecvent în jurul vârstei de 60 de ani şi se asociază cel mai frecvent cu limfoame şi leucemii. Această formă particulară de pemfigus are aspecte clinice, histopatologice şi imunopatologice distincte de alte forme de pemfigus. Clinic se manifestă prin eroziuni severe ale mucoas...

Radiation induced pemphigoid disease

Obstetrics & Gynecology Science, 2020

Among the possible complications of radiation therapy, acute and chronic side effects on the skin can be induced by percutaneous radiotherapy in the target site. Common skin lesions include radiation dermatitis, which can be treated by topical application of dressing and ointment. Pemphigoid disease, which displays similar clinical features as other skin diseases such as recurrent cancer and herpes zoster, rarely occurs in the site of radiotherapy; therefore, care must be taken during diagnosis for a timely treatment. The present report is a case of pemphigoid disease that had developed in a patient with endometrioid/clear cell carcinoma after radiation therapy, and the time between onset and radiotherapy was more than 6 months.

Recurrence of pemphigus vulgaris after bilateral breast irradiation: a case report and review of the literature

2021

Background Pemphigus is a serious and rare chronic bullous autoimmune disease. It is characterized by mucocutaneous erosions secondary to autoantibodies directed against desmogleins 1 and 3, proteins involved in intercellular adhesion mechanisms. The occurrence of pemphigus is based on the triggering of genetic and external environmental factors such as drugs, infection and more rarely radiotherapy. To date, only 16 cases of radiation-induced pemphigus are described in a context of breast cancer treatment. Case presentation: We present the case of a 76-year-old woman who had a recurrence of pemphigus vulgaris limited to the irradiation field after exposure to an adjuvant radiotherapy treatment for a bilateral triple negative breast cancer. The onset was bilateral limited to the irradiation area and was treated effectively with local and systemic corticosteroids. After a rigorous review of the literature, only 16 cases of breast cancer radiation-induced pemphigus appeared. In contras...

Paraneoplastic pemphigus with a pemphigus vegetans-like plaque as the only cutaneous manifestation

Journal of the American Academy of Dermatology, 1998

Paraneoplastic pemphigus (PNP) is defined by 5 diagnostic criteria: (1) painful mucosal erosions and polymorphous skin eruption in the context of an occult or confirmed neoplasm; (2) histopathologic changes of keratinocyte necrosis, intraepidermal acantholysis, and vacuolar-interface dermatitis; (3) direct immunofluorescence (DIF) findings of intercellular IgG and complement and often linear or granular complement at the dermalepidermal junction (DEJ); (4) indirect immunofluorescence (IIF) testing that demonstrates circulating antibodies binding to simple, columnar, and transitional epithelia in addition to the typical pemphigus pattern of binding to skin and mucosa; (5) circulating autoantibodies that immunoprecipitate a high-molecular weight complex of polypep-tides from keratinocyte extracts weighing 250, 230, 210, 190, and 170 kd. Although refractory stomatitis is a consistent clinical feature, the cutaneous manifestations are characteristically variable. We describe a patient with PNP who presented with severe oral, laryngeal, and esophageal involvement. A pemphigus vegetans-like plaque was the only cutaneous manifestation.

Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview

Anais Brasileiros de Dermatologia, 2019

Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and α-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively.