Recurrent migratory angioedema as cutaneous manifestation in a familiar case of TRAPS: dramatic response to Anakinra.
Dermatol Online J
; 19(11): 20405, 2013 Nov 15.
Article
en En
| MEDLINE
| ID: mdl-24314780
ABSTRACT
BACKGROUND:
Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) is a hereditary autoinflammatory syndrome characterized by recurrent episodes of fever and localized inflammation. Clinical presentation can be very variable in terms of duration of fever attacks, periodicity, and accompanying manifestations. One of the most characteristic symptoms is the occurrence of migrating skin rash with myalgia that is sustained by monocytic inflammation. OBSERVATIONS We herein present the case of a family suffering from TRAPS who had been misdiagnosed for a long period of time and whose main symptom was migrating angioedema. Skin biopsy from one of the patients documented a monocytic panniculitis. All the living patients responded dramatically to anakinra treatment.CONCLUSIONS:
The classic symptom of migratory angioedema with myalgia in TRAPS can be produced by monocytic panniculitis.This manifestation is so characteristic of TRAPS that its occurrence, even in the absence of other manifestations, should prompt genetic analysis. Our patient's condition responded promptly to anakinra treatment.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Antirreumáticos
/
Proteína Antagonista del Receptor de Interleucina 1
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Enfermedades Autoinflamatorias Hereditarias
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Angioedema
Tipo de estudio:
Diagnostic_studies
Límite:
Adult
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Female
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Humans
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Male
Idioma:
En
Revista:
Dermatol Online J
Asunto de la revista:
DERMATOLOGIA
Año:
2013
Tipo del documento:
Article
Pais de publicación:
EEUU
/
ESTADOS UNIDOS
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ESTADOS UNIDOS DA AMERICA
/
EUA
/
UNITED STATES
/
UNITED STATES OF AMERICA
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US
/
USA