Paraneoplastic Pemphigus. A Life-Threatening Autoimmune Blistering Disease

2017 ◽  
Vol 108 (10) ◽  
pp. 902-910 ◽  
Author(s):  
A. Tirado-Sánchez ◽  
A. Bonifaz
Keyword(s):  
Paraneoplastic Pemphigus ◽  
Autoimmune Blistering Disease ◽  
Life Threatening ◽  
Blistering Disease

A Novel Use of Topical Ketamine for the Treatment of Oral Pemphigus: A Case Report

2020 ◽  
pp. 082585972094650
Author(s):  
Elizabeth A. Higgins ◽  
Julia Arana West
Keyword(s):  
Palliative Care ◽  
Case Report ◽  
Standard Treatment ◽  
Stage Iv ◽  
Lymphoproliferative Disorders ◽  
Pain Level ◽  
Paraneoplastic Pemphigus ◽  
Autoimmune Blistering Disease ◽  
Blistering Disease ◽  
Topical Ketamine

Background: Paraneoplastic pemphigus (PNP) is a rare autoimmune blistering disease often associated with lymphoproliferative disorders. PNP causes hemorrhagic stomatitis of the lips, tongue, and cheeks which can be painful and difficult to treat. Case History: A 54-year-old man was referred to our hospital for treatment of Stage IV lymphoma and paraneoplastic pemphigus. Due to pain from oral stomatitis, he required a gastrostomy tube for delivery of nutritional tube feeds. He was placed on steroids and received both lidocaine mouthwash and opioids, but his pain persisted. Case Management and Outcome: A palliative care consult was obtained for management of his pain. The palliative team recommended topical ketamine for the treatment of the painful lip lesions. The patient felt immediate relief with the application of ketamine and his pain level was reduced to a 5/10. The following day, his pain was 0/10. Five days later, the opioids were discontinued and the pain relief continued. Conclusion: Painful stomatitis from PNP may be refractory to standard treatment. We found that the application of topical ketamine was very effective in this patient with hemorrhagic stomatitis from PNP.

scholarly journals Paraneoplastic pemphigus associated with Castleman’s disease in a 13-year-old boy

2020 ◽  
Vol 0 ◽  
pp. 1-4
Author(s):  
Neema Joy ◽  
Anuja Elizabeth George ◽  
Lissy Skaria
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Clinical Remission ◽  
Castleman’S Disease ◽  
Skin Lesions ◽  
Treatment Evaluation ◽  
Castleman's Disease ◽  
Paraneoplastic Pemphigus ◽  
Autoimmune Blistering Disease ◽  
Underlying Malignancy ◽  
Blistering Disease

Paraneoplastic pemphigus is an autoimmune blistering disease associated with an underlying malignancy. This is a case of a 13-year-old boy initially presenting with isolated oral erosions – managed as a case of pemphigus, who one and a half years later developed toxic epidermal necrolysis like rash, both refractory to treatment. Evaluation for an underlying malignancy revealed a retroperitoneal Castleman’s tumor, following the excision of which there was clinical remission of the oral and skin lesions.

scholarly journals Pemphigus Vulgaris as Oral Mucosal Ulcer Progressive to Skin Lesion:

2014 ◽  
Vol 3 (2) ◽  
pp. 43-47
Author(s):  
SM Anwar Sadat ◽  
Akhter Imam
Keyword(s):  
Public Health ◽  
Pemphigus Vulgaris ◽  
Health It ◽  
Major Threat ◽  
Floor Of The Mouth ◽  
Autoimmune Blistering Disease ◽  
Life Threatening ◽  
History Of ◽  
Blistering Disease ◽  
One Year

Among all the life threatening diseases, autoimmune diseases are among most notorious and complicated diseases. As these diseases have no specific cause or responsible organism, it is therefore very difficult to distinguish and diagnose and thus stands as a major threat for public health. It is assumed that the reason for these diseases may be from genetic to environmental factors, from stress to idiopathic origin. Pemphigus Vulgaris(PV) is such an autoimmune blistering disease that frequently affects the mucous membrane and skin. Very often it starts from oral cavity and Dentists therefore the first to recognize. This paper describes the case of a patient presenting with a one-year history of painful ulcerated gingiva, tongue, floor of the mouth and even on GIT who is finally diagnosed as having PV spreading to generalized skin. DOI: http://dx.doi.org/10.3329/updcj.v3i2.17999 Update Dent. Coll. j: 2013; 3 (2): 43-47

Autoimmune Blistering Disease with Concurrent IgG Antibodies to Desmoglein 1 and BP180 : Two Cases

2013 ◽  
Vol 75 (5) ◽  
pp. 415-418
Author(s):  
Miki ICHIKAWA ◽  
Maya TANAKA ◽  
Kazunori URABE ◽  
Masutaka FURUE
Keyword(s):  
Igg Antibodies ◽  
Autoimmune Blistering Disease ◽  
Blistering Disease

Sweet Syndrome and Pemphigus Vulgaris

2012 ◽  
Vol 16 (2) ◽  
pp. 128-130 ◽  
Author(s):  
Ashley O'toole ◽  
Maureen O'malley
Keyword(s):  
Clinical Presentation ◽  
Pemphigus Vulgaris ◽  
Neutrophilic Dermatosis ◽  
Rare Presentation ◽  
Sweet Syndrome ◽  
Autoimmune Blistering Disease ◽  
Mucous Membranes ◽  
Blistering Disease ◽  
Erythematous Plaques ◽  
New Onset

Background: Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucous membranes. Sweet syndrome is an uncommon reactive neutrophilic dermatosis. Objective: This case report describes the rare presentation of a 58-year-old female patient with newly diagnosed pemphigus vulgaris who later presented with erythematous plaques and leukocytosis highly suggestive of Sweet syndrome. Methods: Review of the literature using the PubMed and Medline databases. Results: The clinical presentation of new-onset Sweet syndrome in a patient with pemphigus has been reported in the literature only on one other occasion. Conclusion: The observation of an association between Sweet syndrome and autoimmune diseases, such as pemphigus vulgaris, may ultimately lead to better understanding of the pathophysiology of this disease.

scholarly journals Pemphigus herpetiformis : A rare clinical variant of pemphigus

2016 ◽  
Vol 13 (1) ◽  
pp. 61-65
Author(s):  
P Shrestha ◽  
R B Tajhya ◽  
A Pokharel
Keyword(s):  
Inflammatory Infiltrate ◽  
Autoimmune Blistering Disease ◽  
Neutrophilic Dermatoses ◽  
Clinical Variant ◽  
Blistering Disease ◽  
Anti Inflammatory

Pemphigus herpetiformis is an autoimmune blistering disease. We report a case of pemphigus herpetiformis, a generalized form, manifested as pruritic grouped vesicles at extremities and trunk, in a seventy years old man. Neutrophil dominant inflammatory substrate was seen in histopathology. Direct immunofluorescene revealed IgG reactivity in net like pattern at upper epidermis, confirming pemphigus. The patient responded well to prednisolone and colchicine combination initially and maintained remission later on colchicine alone. Colchicine has anti-mitotic, anti-inflammatory and immunosuppressive mechanism of actions and proven benefits in neutrophilic dermatoses. Therefore we recommend colchicine as mono therapy or in combination with immunosuppressive, for the treatment of pemphigus herpetiformis where neutrophil is predominant inflammatory infiltrate histologically.NJDVL Vol. 13, No. 1, 2015 Page: 61-65

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