A middle-aged man presenting with unexplained mucosal erosions and progressive dyspnoea

Healy, William; Peters, Sara; Nana‐Sinkam, S. Patrick

doi:10.1136/bcr-2014-208677

Cited by 9 publications

(18 citation statements)

References 7 publications

(13 reference statements)

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“…The mechanism of how PNP and CD cause BO has not been completely elucidated. Previous studies have shown that CD8+ T lymphocytes mediate cytotoxic immunity, IgG autoantibody deposition, and acantholysis, which further induces airway inflammation, with subsequent irreversible fibrotic change . In a previous study in China, BO was reported in 10 of 37 patients with PNP and CD (27%) .…”

Section: Discussionmentioning

confidence: 92%

Masked pemphigus among pediatric patients with Castleman’s disease

Han

Chen

2018

Int J of Rheum Dis

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Anhalt in 1990. He described 5 patients with underlying neoplasms who presented with clinical mucocutaneous lesions resulting from an autoantibody-mediated immune response. 1 The patients exhibited conjunctiva erosion, oral sores, genital ulcers, skin eruption, vesicles, and blisters. The histopathological findings of these lesions were characterized by suprabasilar acantholysis, keratinocytes necrosis, and vacuolar interface change. The coexisting tumors were Abstract Aim: Paraneoplastic pemphigus (PNP) is a mucocutaneous autoimmune disorder accompanied with a neoplasm. Castleman's disease (CD), although rare, is the most common cause of PNP in children. It can be life-threatening when pulmonary involvement occurs. Our study aimed to describe the features of PNP resulting from CD and to find clues for the early diagnosis in pediatric patients. Method: We report the case of a 13-year-old girl who initially presented with oral ulcers and lichen planus, with progression to respiratory failure. A literature review of PNP and CD in children between 1997 and 2016 was performed. The clinical manifestations, pathological findings, treatment, and outcome were analyzed. Results: Thirty-two children were included in our study: 16 boys and 16 girls. Intractable mucocutaneous lesions developed early before CD was diagnosed. The clinical manifestations comprised oral ulcers (100%), polymorphous skin rash (86.7%) and genital (62.5%) erosion. Histopathological findings revealed lymphoplasmacytic cells infiltration (92%), vacuolar interface change (72%), acantholysis (68%), and keratinocytes necrosis (36%). Thirty patients underwent tumor resection. These patients mainly had unicentric CD, with the hyaline-vascular variant dominant. Twenty-six patients (81.2%) exhibited pulmonary involvement. The mortality rate was 70.0%. Among them, 90.5% exhibited pulmonary involvement, and 81.0% died of respiratory failure. Conclusion: Intractable mucocutaneous lesions with a concurrent tumor in children strongly indicate PNP resulting from CD. Because stomatitis or skin erosion may be the first presentation, mucocutaneous tissue biopsy and early detection of the underlying tumor are important. Earlier diagnosis is mandatory for the effective treatment of PNP and pulmonary involvement. K E Y W O R D S bronchiolitis obliterans, Castleman's disease, intravenous immunoglobulin, paraneoplastic pemphigus, pediatric

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Section: Discussionmentioning

confidence: 92%

Masked pemphigus among pediatric patients with Castleman’s disease

Han

Chen

2018

Int J of Rheum Dis

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“…Ulceration may involve the entire oral mucosal surfaces and may represent the sole manifestation of this condition. A painful stomatitis is also commonly shown caused by massive erosion in the oropharynx [ 2 , 21 , 53 , 55 ].…”

Section: Clinical Featuresmentioning

confidence: 99%

Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy

Paolino

Didona

Magliulo

et al. 2017

IJMS

111

195

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Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by paraneoplastic pemphigus patients. The pathogenesis of paraneoplastic pemphigus is not yet completely understood, although some immunological aspects have been recently clarified. Because of its rarity, several diagnostic criteria have been proposed. Besides, several diagnostic procedures have been used for the diagnosis, including indirect immunofluorescence, direct immunofluorescence, and ELISA. We reviewed the most recent literature, searching on PubMed “paraneoplastic pemphigus”. We included also papers in French, German, and Spanish. We found 613 papers for “paraneoplastic pemphigus”. Among them, 169 were review papers. Because of its varying clinical features, paraneoplastic pemphigus still represents a challenge for clinicians. Furthermore, diagnosis and management of paraneoplastic pemphigus requires close collaboration between physicians, including dermatologist, oncologist, and otorhinolaryngologist.

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“…Lesions can also affect regions such as the oropharynx, esophagus, stomach, duodenum, large intestine, conjunctiva, and anogenital region [2,3,7,39,41,44,45]. The involvement of the oropharynx and esophagus commonly triggers painful sensations and dysphagia [4].…”

Section: Secondary Mucosal Lesionsmentioning

confidence: 99%

“…The use of otorhinolaryngological examination is very important to differentiate the diseases that affect the mucous membranes. Well-done physical examination of the oral cavity, histopathological analysis characteristics, cutaneous involvement, and the presence of IIF strongly suggest for the diagnosis of PNP [40,44,67].…”

Section: Differential Diagnosismentioning

confidence: 99%

Paraneoplastic Pemphigus Is a Life-Threatening Disease

Konichi-Dias¹

2019

Current Trends in Cancer Management

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Paraneoplastic pemphigus is a multiorganic autoimmune disease, usually triggered by neoplasias, mainly of lymphoproliferative origin such as chronic lymphocytic leukemia, multiple myeloma, non-Hodgkin's lymphoma, Castleman disease, and thymoma. This disorder is characterized by the presence of autoantibodies that react against proteins, such as desmoplakins, desmocollins, and others existing in cell junctions. The prognosis is reserved, and the mortality rate of the disease is very high, thus proving to be an additional challenge in the therapeutic management of onco-hematological diseases. The objective of this chapter is to solve the main clinical aspects of paraneoplastic pemphigus in lymphoproliferative hematological diseases, anatomopathological and immunofluorescence characteristics, as well as associations with the main differential diagnoses and therapeutic management. We will also describe the main differential diagnoses of paraneoplastic pemphigus, such as various types of pemphigus including induced drug, bullous pemphigoid, drug eruption, lichen planus, graft versus host disease, erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis. In addition, the prognosis and quality of life will be mentioned.

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A middle-aged man presenting with unexplained mucosal erosions and progressive dyspnoea

Cited by 9 publications

References 7 publications

Masked pemphigus among pediatric patients with Castleman’s disease

Masked pemphigus among pediatric patients with Castleman’s disease

Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy

Paraneoplastic Pemphigus Is a Life-Threatening Disease

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