Pénfigo paraneoplásico/síndrome multiorgánico autoinmune paraneoplásico. Presentación de dos casos en la edad infantil. Revisión de la literatura / Paraneoplastic Pemphigus or Paraneoplastic Autoimmune Multiorgan Syndrome. Report of 2 Cases in Children and a Review of the Literature
Actas dermo-sifiliogr. (Ed. impr.)
; 101(10): 879-886, dic. 2010. ilus, tab
Article
in Spanish
| IBECS
| ID: ibc-83503
Responsible library:
ES1.1
Localization: BNCS
RESUMEN
El pénfigo paraneoplásico es una enfermedad ampollosa autoinmune asociada a una neoplasia oculta o previamente diagnosticada, con manifestaciones clínicas, histológicas e inmunológicas bien definidas. Se caracteriza por erosiones de la mucosa orogenital refractarias a los tratamientos convencionales y la presencia de lesiones cutáneas polimorfas. La histología puede ser variada, con presencia de acantólisis o dermatitis liquenoide. La presencia de anticuerpos circulantes es un hallazgo constante que confirma el diagnóstico. Presentamos dos niñas con pénfigo paraneoplásico asociado a linfoma de Hodgkin y enfermedad de Castleman respectivamente. Presentaban compromiso orogenital refractario a tratamientos convencionales. En la histopatología se observó acantólisis y reacción liquenoide respectivamente. Los estudios inmunológicos confirmaron el diagnóstico. Ambas realizaron tratamiento quimioterápico para su enfermedad de base conjuntamente con altas dosis de corticosteroides para sus lesiones cutáneo-mucosas falleciendo por fallo respiratorio. Es de nuestro interés destacar que si bien es infrecuente la presencia de pénfigo paraneoplásico en la edad infantil y en la adolescencia, debe ser tenido en cuenta entre los diagnósticos diferenciales de las dermatosis erosivas periorificiales facilitando, de esta manera, el hallazgo de una neoplasia oculta (AU)
ABSTRACT
Paraneoplastic pemphigus is an autoimmune blistering disease associated with an occult or previously diagnosed tumor. Its clinical, histological, and immunological features have been clearly defined. It is characterized by the presence of polymorphic skin lesions and by erosions of the oral and genital mucosas that are refractory to conventional treatments. The histology can be variable and includes acantholysis or lichenoid dermatitis. Circulating autoantibodies are a constant feature and confirm the diagnosis. We describe 2 girls with paraneoplastic pemphigus associated with Hodgkin lymphoma in one and Castelman disease in the other. Both children had oral and genital lesions that did not respond to conventional treatments. Biopsy revealed acantholysis in one and a lichenoid reaction in the other, and immunoassays confirmed the diagnosis. Chemotherapeutic treatment of the underlying disease was performed in both cases, together with high-dose corticosteroids for the skin and mucosal lesions. Both patients died due to respiratory failure. We suggest that paraneoplastic pemphigus, although rare in childhood and adolescence, should be included in the differential diagnosis of periorificial erosive dermatitis; this may assist in the detection of an occult neoplasm (AU)
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Collection:
National databases
/
Spain
Database:
IBECS
Main subject:
Hodgkin Disease
/
Castleman Disease
/
Pemphigus
Limits:
Child
/
Female
/
Humans
Language:
Spanish
Journal:
Actas dermo-sifiliogr. (Ed. impr.)
Year:
2010
Document type:
Article
Institution/Affiliation country:
Hospital Carlos G. Durand/Argentina
/
Hospital de Pediatría Dr. Prof. Juan P. Garrahan/Argentina
/
Institute for Immunofluorescence Dermpath Diagnostic/EE.UU