paraneoplastic pemphigus


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paraneoplastic pemphigus

painful mucosal erosions and polymorphous skin eruptions with biopsy findings resembling pemphigus vulgaris, associated with neoplasm and serum antibodies reactive with intercellular substance of all epithelia; usually rapidly fatal.

par·a·ne·o·plas·tic pemph·i·gus

(par'ă-nē-ō-plas'tik pem'fi-gŭs)
Painful mucosal erosions and polymorphous skin eruptions with biopsy findings resembling pemphigus vulgaris, associated with neoplasm and serum antibodies reactive with intercellular substance of all epithelia; usually rapidly fatal.

paraneoplastic pemphigus

Pemphigus that arises together with or sometimes before malignancy, particularly B-cell lymphomas and chronic lymphocytic leukemia.
See also: pemphigus
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References in periodicals archive ?
IgG autoantibodies directed against desmoglein 3 cause dissociation of keratinocytes in canine pemphigus vulgaris and paraneoplastic pemphigus. Vet Immunol Immunop 117: 209-21.
Mediastinal follicular dendritic cell sarcoma with paraneoplastic pemphigus. Asian Cardiovasc Thorac Ann.
Paraneoplastic pemphigus associated with fatal bronchiolitis obliterans and intractable mucosal erosions: Treatment with cyclosporinin addition to steroid, rituximab immunoglobulin.
Castleman's tumours and production of autoantibody in paraneoplastic pemphigus. Lancet 2004; 363:525-31.
Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia.
Classification, clinical manifestations, and immunopathological mechanisms of the epithelial variant of paraneoplastic autoimmune multiorgan syndrome: a reappraisal of paraneoplastic pemphigus. Arch Dermatol 2001;137:193-206.
* Rule out look-alike conditions, including erythema multiforme, sunburn, toxic shock syndrome, staphylococcal scalded skin syndrome, and paraneoplastic pemphigus.
The first category is referred to as pemphigus and entails 4 disease entities: pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, and IgA pemphigus.
The differential diagnosis for EM includes urticaria, Stevens-Johnson syndrome, fixed drug eruption, bullous pemphigoid, paraneoplastic pemphigus, acute febrile neutrophilic dermatosis (Sweet's syndrome), polymorphous light eruption (PMLE), and cutaneous small-vessel vasculitis.
Additionally, there may be an association between FDCS and autoimmune disease: paraneoplastic pemphigus and myasthenia gravis.
According to this new classification, pemphigus group diseases include, pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceus, pemphigus erythematosus, endemic pemphigus, IgA pemphigus, pemphigus herpetiformis, paraneoplastic pemphigus, and drug-induced pemphigus.

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