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Paraneoplastic pemphigus - Oral Mucosal Lesion
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Paraneoplastic pemphigus - Oral Mucosal Lesion

See also in: Overview
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Contributors: Marvin Turck MD, Carl Allen DDS, MSD, Sook-Bin Woo MS, DMD, MMSc
Other Resources UpToDate PubMed

Synopsis

Paraneoplastic pemphigus is a severe, immunologically mediated mucocutaneous disease that typically develops in the setting of current or past history of a malignancy (usually hematologic, but not always). Both humoral and cell-mediated responses are involved, and the presentation is likely to depend on which predominates. Some prefer the term "paraneoplastic autoimmune multiorgan syndrome," since the lungs and other organs may also be involved, and the findings are not always identical to pemphigus.

The etiopathogenesis is complex, with cellular and humoral systems involved. One mechanism may be that tumors produce autoantibodies against the skin and mucosa.

Symptoms include painful cutaneous and oral lesions secondary to the vesicles and bullae that form and subsequently rupture. Severe eye irritation may also be seen with conjunctival involvement, and pulmonary involvement, which is frequent, takes the form of bronchiolitis obliterans leading to dyspnea.

Most patients who develop paraneoplastic pemphigus are adults and have a history of lymphoma or chronic lymphocytic leukemia, although a small percentage of patients have been documented with other malignancies. Castleman's disease (angiofollicular lymph node hyperplasia) and thymoma have also been associated with paraneoplastic pemphigus. These patients tend to be younger, in the second or third decades of life. In a small but significant percentage of patients, the onset of paraneoplastic pemphigus will be the first clue that a tumor is present.

Codes

ICD10CM:
L10.81 – Paraneoplastic pemphigus

SNOMEDCT:
402718003 – Pemphigus paraneoplastica

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Pemphigus vulgaris – Oral erosions and ulcerations are usually the dominant feature for weeks to months before cutaneous vesicles and bullae begin to appear. Skin lesions in paraneoplastic pemphigus are more pleomorphic than in pemphigus vulgaris.
  • Pemphigus foliaceus does not usually show oral involvement.
  • Erythema multiforme – Patients may have a history of previous episodes that resolved within 2-4 weeks, which is usually not seen in paraneoplastic pemphigus. Patients may have a history of preceding herpetic infection. If classic "target" lesions of the skin are present, this would be more consistent with erythema multiforme.
  • Bullous pemphigoid – Cutaneous vesicles and bullae are the predominant feature of this condition, with only 20% of patients having oral involvement. Hemorrhagic crusting of the lips would be unusual.
  • Mucous membrane pemphigoid – This condition mostly affects the mucosa at various sites, with only 20% of patients showing cutaneous bullae. The relatively chronic, slow evolution of cicatricial pemphigoid would not be consistent with paraneoplastic pemphigus.
  • Lichen planus – The erosive and ulcerative lesions of oral lichen planus are usually surrounded by radiating keratotic striae, a feature that is typically not evident in the oral lesions of paraneoplastic pemphigus.
  • Herpetic stomatitis – The oral ulcerations may be diffuse but are comprised of crops of small ulcers (1-2 mm) that coalesce. Unlike paraneoplastic pemphigus, significant cutaneous involvement in herpetic stomatitis would be unusual.
  • Stevens-Johnson syndrome / toxic epidermal necrolysis – Drug-induced high fevers, skin tenderness, mucosal erosions, and skin detachment (necrolysis) develop 1-3 weeks after the inciting medication is started.
  • Drug hypersensitivity syndrome (drug reaction with eosinophilia and systemic symptoms [DRESS]) – Look for facial edema (hallmark of DRESS), eosinophilia, hepatitis, and other visceral involvement.
  • Drug-induced erythroderma

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Last Updated: 05/20/2019
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Paraneoplastic pemphigus - Oral Mucosal Lesion
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Paraneoplastic pemphigus : Bullae, Lip mucosa hemorrhagic crust, Oral erosions, Painful oral ulcers, Dyspnea
Clinical image of Paraneoplastic pemphigus
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