Paraneoplastic pemphigus in children and adolescents

D. Mimouni, Grant James Anhalt, Z. Lazarova, S. Aho, S. Kazerounian, D. J. Kouba, J. M. Mascaro, H. C. Nousari

Research output: Contribution to journalArticle

Abstract

Background: Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease associated with specific B-cell lymphoproliferative neoplasms. There has been an increasing number of individual reports in the childhood and adolescent population. Objectives: To examine the clinical and immunopathological features of PNP occurring in children and adolescents. Patients and methods: We analysed the clinical and immunopathological findings of 14 patients under the age of 18 years with a confirmed diagnosis of PNP. Sera from all patients were analysed by indirect immunofluorescence (IF) and immunoprecipitation for plakin autoantibodies, immunoblotting for detection of plectin autoantibodies, and enzyme-linked immunosorbent assay (ELISA) for the detection of desmoglein (Dsg) 1 and Dsg3 autoantibodies. Results: Severe oral mucositis was observed in all patients, and lichenoid cutaneous lesions in eight of 14 patients. The average age at presentation was 13 years. Striking findings included: pulmonary destruction leading to bronchiolitis obliterans in 10 patients, association with Castleman's disease in 12 patients, and a fatal outcome in 10 patients. The underlying neoplasm was occult in 10 patients. Histological findings include lichenoid and interface dermatitis with variable intraepithelial acantholysis. Deposition of IgG and C3 in the mouth and skin by direct IF was not found in some cases, but indirect IF detected IgG autoantibodies in all cases. Immunoprecipitation revealed IgG autoantibodies against desmoplakin I, envoplakin and periplakin in all cases, and against desmoplakin II and the 170-kDa antigen in 13 and 10 patients, respectively. Dsg3 and Dsg1 autoantibodies were present in 10 and three patients, respectively, and plectin autoantibodies in 13 patients. Conclusions: PNP in children and adolescents is most often a presenting sign of occult Castleman's disease. It presents with severe oral mucositis and cutaneous lichenoid lesions. Serum autoanti-bodies against plakin proteins were the most constant diagnostic markers. Pulmonary injury appears to account for the very high mortality rates observed.

Original languageEnglish (US)
Pages (from-to)725-732
Number of pages8
JournalBritish Journal of Dermatology
Volume147
Issue number4
DOIs
StatePublished - 2002

Fingerprint

Pemphigus
Autoantibodies
Plectin
Desmoplakins
Giant Lymph Node Hyperplasia
Stomatitis
Immunoglobulin G
Indirect Fluorescent Antibody Technique
Immunoprecipitation
Skin
Desmoglein 1
Acantholysis
Bronchiolitis Obliterans
Direct Fluorescent Antibody Technique
Fatal Outcome
Lung Injury
Dermatitis
Serum
Immunoblotting
Autoimmune Diseases

Keywords

  • Castleman's disease
  • Children
  • Lichenoid dermatitis
  • Paraneoplastic syndrome
  • Pemphigus
  • Plakin protein

ASJC Scopus subject areas

  • Dermatology

Cite this

Mimouni, D., Anhalt, G. J., Lazarova, Z., Aho, S., Kazerounian, S., Kouba, D. J., ... Nousari, H. C. (2002). Paraneoplastic pemphigus in children and adolescents. British Journal of Dermatology, 147(4), 725-732. https://doi.org/10.1046/j.1365-2133.2002.04992.x

Paraneoplastic pemphigus in children and adolescents. / Mimouni, D.; Anhalt, Grant James; Lazarova, Z.; Aho, S.; Kazerounian, S.; Kouba, D. J.; Mascaro, J. M.; Nousari, H. C.

In: British Journal of Dermatology, Vol. 147, No. 4, 2002, p. 725-732.

Research output: Contribution to journalArticle

Mimouni, D, Anhalt, GJ, Lazarova, Z, Aho, S, Kazerounian, S, Kouba, DJ, Mascaro, JM & Nousari, HC 2002, 'Paraneoplastic pemphigus in children and adolescents', British Journal of Dermatology, vol. 147, no. 4, pp. 725-732. https://doi.org/10.1046/j.1365-2133.2002.04992.x
Mimouni, D. ; Anhalt, Grant James ; Lazarova, Z. ; Aho, S. ; Kazerounian, S. ; Kouba, D. J. ; Mascaro, J. M. ; Nousari, H. C. / Paraneoplastic pemphigus in children and adolescents. In: British Journal of Dermatology. 2002 ; Vol. 147, No. 4. pp. 725-732.
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AU - Lazarova, Z.

AU - Aho, S.

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AU - Kouba, D. J.

AU - Mascaro, J. M.

AU - Nousari, H. C.

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N2 - Background: Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease associated with specific B-cell lymphoproliferative neoplasms. There has been an increasing number of individual reports in the childhood and adolescent population. Objectives: To examine the clinical and immunopathological features of PNP occurring in children and adolescents. Patients and methods: We analysed the clinical and immunopathological findings of 14 patients under the age of 18 years with a confirmed diagnosis of PNP. Sera from all patients were analysed by indirect immunofluorescence (IF) and immunoprecipitation for plakin autoantibodies, immunoblotting for detection of plectin autoantibodies, and enzyme-linked immunosorbent assay (ELISA) for the detection of desmoglein (Dsg) 1 and Dsg3 autoantibodies. Results: Severe oral mucositis was observed in all patients, and lichenoid cutaneous lesions in eight of 14 patients. The average age at presentation was 13 years. Striking findings included: pulmonary destruction leading to bronchiolitis obliterans in 10 patients, association with Castleman's disease in 12 patients, and a fatal outcome in 10 patients. The underlying neoplasm was occult in 10 patients. Histological findings include lichenoid and interface dermatitis with variable intraepithelial acantholysis. Deposition of IgG and C3 in the mouth and skin by direct IF was not found in some cases, but indirect IF detected IgG autoantibodies in all cases. Immunoprecipitation revealed IgG autoantibodies against desmoplakin I, envoplakin and periplakin in all cases, and against desmoplakin II and the 170-kDa antigen in 13 and 10 patients, respectively. Dsg3 and Dsg1 autoantibodies were present in 10 and three patients, respectively, and plectin autoantibodies in 13 patients. Conclusions: PNP in children and adolescents is most often a presenting sign of occult Castleman's disease. It presents with severe oral mucositis and cutaneous lichenoid lesions. Serum autoanti-bodies against plakin proteins were the most constant diagnostic markers. Pulmonary injury appears to account for the very high mortality rates observed.

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