A case of paraneoplastic pemphigus associated with other iatrogenic immunodeficiency‐associated lymphoproliferative disorders

Paraneoplastic pemphigus (PNP) is a rare autoimmune bullous disease associated with underlying neoplasms. Targetoid lesions, intractable stomatitis and refractory course of disease are some of the characteristic features of paraneoplastic pemphigus. It is usually associated with lymphoproliferative disorders, and rarely with solid tumors. We present a case of a 35 years old female with a six-months history of recurrent, severe, recalcitrant stomatitis and widespread blistering and erosions with involvement of palms and soles. A provisional diagnosis of PNP was made based on clinical features. Investigations for an underlying neoplasm revealed presence of solid lesion of size 6.7 x 6.4 cm in left adnexa, likely an ovarian tumor. PNP should be considered in the differential diagnoses in severe atypical mucocutaneous manifestations of pemphigus vulgaris and diagnostic screening for search for an underlying tumor should be performed.

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

Journal of Palliative Care ◽

10.1177/0825859720946504 ◽

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.

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Lymphoproliferative disorders (LPD) involving lungs: T and B cell subsets within pulmonary infiltrates and in peripheral blood

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s042482010011338x ◽

1984 ◽

Vol 42 ◽

pp. 700-701

Author(s):

Irene Stachura ◽

Milton H. Dalbow ◽

Michael J. Niemiec ◽

Matias Pardo ◽

Gurmukh Singh ◽

...

Keyword(s):

B Cells ◽

B Cell ◽

Lymphoproliferative Disorders ◽

Mixed Population ◽

Lymphoid Cells ◽

Lymphomatoid Granulomatosis ◽

Cell Type ◽

Cell Surface Antigens ◽

Pulmonary Infiltrates ◽

B Cell Subsets

Lymphoid cells were analyzed within pulmonary infiltrates of six patients with lymphoproliferative disorders involving lungs by immunofluorescence and immunoperoxidase techniques utilizing monoclonal antibodies to cell surface antigens T11 (total T), T4 (inducer/helper T), T8 (cytotoxic/suppressor T) and B1 (B cells) and the antisera against heavy (G,A,M) and light (kappa, lambda) immunoglobulin chains. Three patients had pseudolymphoma, two patients had lymphoma and one patient had lymphomatoid granulomatosis.A mixed population of cells was present in tissue infiltrates from the three patients with pseudolymphoma, IgM-kappa producing cells constituted the main B cell type in one patient. In two patients with lymphoma pattern the infiltrates were composed exclusively of T4+ cells and IgG-lambda B cells predominated slightly in the patient with lymphomatoid granulomatosis.

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