Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome

Sehgal, Virendra; Srivastava, Govind

doi:10.1111/j.1365-4632.2009.03995.x

Cited by 98 publications

(94 citation statements)

References 131 publications

(285 reference statements)

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“…varied, typically begins with a florid, painful eruption of mucous membrane lesions, which may involve the oropharynx, nasopharynx, tongue, vermillion of the lips (Figure 1), conjunctiva, anogenital region (Figure 2), or esophagus [1,4,10,11]. Oral mucosal lesions were usually more extensive than that of pemphigus vulgaris or erythema multiforme.…”

Section: Histopathologymentioning

confidence: 99%

Paraneoplastic Pemphigus

Zhong-Shuai¹,

Li²

2017

J Dermatol Res Ther

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Paraneoplastic Pemphigus (PNP) is an autoimmune-related acquired bullous disease associated with neoplasia. Both humoral and cellular immunity are involved in the pathogenesis of PNP. Characteristically, PNP has a diverse spectrum of clinical and immunopathological features. Suprabasal acantholysis and clefts with scattered necrotic keratinocytes are the unique histopathological features. PNP patient sera recognize multiple antigens, which have been identified as the plakin protein family. Non-Hodgkin's lymphoma, Castleman's disease, thymoma, follicular dendritic cell sarcoma and chronic lymphocytic leukemia are the commonly associated neoplasms in PNP. The treatment of PNP to date has been rather disappointing. For patients with Castleman's disease, the early detection and removal of the tumor are critical for the treatment of PNP.

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Section: Histopathologymentioning

confidence: 99%

Paraneoplastic Pemphigus

Zhong-Shuai¹,

Li²

2017

J Dermatol Res Ther

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“…Lymphoproliferative disorders predominate, with non-Hodgkin's lymphoma, chronic lymphocytic leukemia, Castelman's tumor, and thymoma being the most common. 7 Of note, PNP with monoclonal gammopathy has been reported as well. 8 Non-hematologic neoplasms associated with PNP are liposarcoma, leiomyosarcoma, adenocarcinoma of the breast, colon, pancreas and other organs, basal cell carcinoma, and malignant melanoma.…”

Section: Discussionmentioning

confidence: 96%

Paraneoplastic Pemphigus in a Patient with Carcinosarcoma of the Uterus

Giebel¹,

Shah²

2010

TMF

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The patient is a 68-year-old female with hypertension, chronic obstructive pulmonary disease, without medical follow-up for several years, who initially presented with skin blisters and oral lesions, which were biopsied by her dermatologist and found to be bullous pemphigoid. She was treated with a course of oral prednisone and had resolution of her skin and oral lesions. Three months later, she presented with a swollen leg and was diagnosed with a deep venous thrombosis, for which she was started on coumadin and had an inferior vena cava filter placed. She then had hemoptysis a few days later, after which the coumadin was held. A chest computed tomography (CT) done at that time found a right middle lobe consolidation consistent with aspiration pneumonia and a cavitary lesion in the left upper lobe of her lung, which was biopsied and found to be a necrotizing cavitary lung lesion. After being ruled out for tuberculosis, she had an outpatient positron emission tomography (PET) scan which was positive for a 2.7cm left lower lobe cavitary lesion and a 9 cm pelvic mass with hypermetabolic activity, as well as small liver lesions. Concurrently, she complained of dysphagia and 30-lbs of weight loss over the prior six months, so the decision was made to undergo an outpatient esophagogastroduodenoscopy (EGD). The EGD could not be completed secondary to a stricture in the distal esophagus; however, pemphigus ulcers were seen in the mouth and esophagus. Given the patient's degree of malnourishment and dehydration, she was admitted to the outside hospital. On admission, she was found to have leukocytosis with a white blood cell count of 37,000/L, hypercalcemia with a calcium of 13mg/dL, hypotension, and acute renal failure with a creatinine of 4.4mg/dL. Her parathyroid hormone-related protein (PTHrP) was 105 pmol/L (normal <1.3 pmol/L), while her intact parathyroid hormone (PTH) was <3pg/mL (10-55 pg/ml). A serum protein electrophoresis with immunoelectrophoreis was positive for an IgG lambda paraprotein. On bone marrow biopsy, there were 25% plasma cells consistent with multiple myeloma, though this diagnosis was questioned after transfer. For treatment of her hypercalcemia, she was given intravenous fluids and calcitonin with minimal improvement, but with resolution of her renal failure.The esophageal biopsy showed squamous mucosa with necrotic ulcer and chronic inflammation with fibrinous exudate. The pelvic mass was also biopsied which was consistent with carcinosarcoma with massive necrosis. The pathology revealed myxoid high-grade spindle cell sarcoma with pleomorphosis and extensive mitosis and poorly differentiated adenocarcinoma with papillary serous differentiation, as well as some smooth muscle differentiation. During her hospitalization, the skin blisters reappeared and were biopsied. The biopsy was consistent with paraneoplastic pemphigus. She was treated with three days of intravenous immunoglobulin (IVIg). She had an open jejunostomy tube placed for nutrition. At this point, she was transferred to Thomas Jef...

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“…However, PNP can affect every age group, including children and adolescents [8][9][10] . PNP appears to affect males and females equally [2] .…”

Section: Epidemiologymentioning

confidence: 94%

“…PNP is always associated with neoplasm, among which B-cell lymphomas and other hematological malignant diseases are most common [2] . In 2001, Nguyen et al suggested the term "paraneoplastic autoimmune multiorgan syndrome" (PAMS) as several organs are affected and autoantibodies bind several tissues [3] .…”

Section: Receivedmentioning

confidence: 99%

Paraneoplastic pemphigus: A trait d’union between dermatology and oncology

Didona¹,

Didona²,

Richetta³

et al. 2015

Adv Mod Oncol Res

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Paraneoplastic pemphigus is a rare autoimmune disease of the skin associated with neoplasm. Nowadays, the pathogenesis of paraneoplastic pemphigus is not fully understood. Due to its rarity, various criteria have been proposed for the diagnosis. For this reason, several diagnostic methods have been considered useful for the diagnosis of paraneoplastic pemphigus including indirect immunofluorescence, direct immune of fluorescence, immunoprecipitation, immunoblotting, and enzyme-linked immunosorbent assay (ELISA). However, the polymorphic clinical features and the various results of laboratory tests and pathological evaluation present a challenge for the clinicians.

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Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome

Cited by 98 publications

References 131 publications

Paraneoplastic Pemphigus

Paraneoplastic Pemphigus

Paraneoplastic Pemphigus in a Patient with Carcinosarcoma of the Uterus

Paraneoplastic pemphigus: A trait d’union between dermatology and oncology

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