Pemphigus Vulgaris and Paraneoplastic Pemphigus

Edgin, Wendell A.; Pratt, Taylor C.; Grimwood, Ronald E.

doi:10.1016/j.coms.2008.06.008

Cited by 12 publications

(14 citation statements)

References 53 publications

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“…Contrary to pemphigus vulgaris, in which DIF shows only intercellular deposition in epithelial cells, the basement membrane is also affected in PNP. 2,8,9,34 …”

Section: Paraneoplastic Pemphigusmentioning

confidence: 99%

See 1 more Smart Citation

Paraneoplastic cutaneous manifestations: concepts and updates*

Silva

Mesquita

Igreja

et al. 2013

An. Bras. Dermatol.

151

170

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The skin often signals systemic changes. Some neoplastic diseases that affect internal organs may trigger several cutaneous manifestations. Although these dermatoses are relatively unusual, the recognition of some typical paraneoplastic dermatoses may lead to the early diagnosis of a neoplasm and determine a better prognosis. In this review article, we discuss the paraneoplastic cutaneous manifestations strongly associated with neoplasms, which include acanthosis nigricans maligna, tripe palms, erythema gyratum repens, Bazex syndrome, acquired hypertrichosis lanuginosa, necrolytic migratory erythema, Leser-Trélat sign and paraneoplastic pemphigus. We also review the clinical manifestations of each condition and include updated knowledge on disease pathogenesis.

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“…Contrary to pemphigus vulgaris, in which DIF shows only intercellular deposition in epithelial cells, the basement membrane is also affected in PNP. 2,8,9,34 …”

Section: Paraneoplastic Pemphigusmentioning

confidence: 99%

“…PNP is associated with high mortality rate secondary to sepsis, bleeding and respiratory failure. 2,9,34,35 …”

Section: Paraneoplastic Pemphigusmentioning

confidence: 99%

Paraneoplastic cutaneous manifestations: concepts and updates*

Silva

Mesquita

Igreja

et al. 2013

An. Bras. Dermatol.

151

170

View full text Add to dashboard Cite

show abstract

“…Presence of intractable stomatitis and anti-plakin protein autoantibodies is a constant feature; however, there have been reports of antibody negative PNP in literature. [2345]…”

Section: Discussionmentioning

confidence: 99%

Castleman′s disease with paraneoplastic pemphigus

et al. 2014

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Castleman's disease is a rare autoimmune disorder with varied clinical presentations. Castleman's commonly involves mediastinum and hence it is thoracic in most of the reported cases. Paraneoplastic pemphigus (PNP) and myasthenia gravis can be associated with multicentric Castleman's disease. Its association with HIV, Kaposi sarcoma, and lymphoma is also well known. We report a rare combination of unicentric, extrathoracic Castleman's disease with PNP and myasthenia gravis.

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“…Prior to the advent of corticosteroid therapy in the 1950s, the disease was in most cases fatal within 5 years 4. Systemic corticosteroids (prednisolone) rapidly brought the disease under control and up until recently have been the mainstay of treatment 12.…”

Section: Discussionmentioning

confidence: 99%

“…Patients with high-serum antidesmoglein 3 antibodies but low-antidesmoglein 1 antibodies will have only mucosal involvement. This is because the function of desmoglein 1 is preserved preventing the development of skin lesions 4. Loss of cell-to-cell adhesion results in the development of intraepithelial blisters that rupture following minimal trauma, leaving behind painful sloughing eroded areas of skin and/or mucosa.…”

Section: Introductionmentioning

confidence: 99%

Pemphigus vulgaris: a multidisciplinary approach to management

2013

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Pemphigus vulgaris (PV) is a rare but potentially life-threatening autoimmune disease affecting the mucosa and the skin. The disease is caused by circulating antibodies to desmosomes (important adhesion proteins linking cells together). Disruption of these intercellular connections results in a loss of cohesion between cells (acantholysis). The clinical result of this process is the development of multiple blisters that easily rupture, leaving behind painful sloughing eroded areas of mucosa and/or skin. We report a case of severe PV in a 56-year-old man presenting with widespread, painful, eroded mucocutaneous lesions. The severity of the disease demanded a range of medical and surgical specialties to successfully manage the problem. This paper highlights the importance of an early multidisciplinary team approach to improve the outcome of patients suffering with this disease.

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Pemphigus Vulgaris and Paraneoplastic Pemphigus

Cited by 12 publications

References 53 publications

Paraneoplastic cutaneous manifestations: concepts and updates*

Paraneoplastic cutaneous manifestations: concepts and updates*

Castleman′s disease with paraneoplastic pemphigus

Pemphigus vulgaris: a multidisciplinary approach to management

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