9- Ampollares
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ENFERMEDADES AMPOLLARES
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ENFERMEDADES AMPOLLARES:
1. PENFIGOS
• VULGAR
• VEGETANTE
• SEBORREICO
• FOLIÁCEO
• PARANEOPLÁSICO
2. PENFIGOIDE AMPOLLAR
3. PENFIGOIDE CICATRIZAL
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4. DERMATITIS POLIMORFA DOLOROSA O DERMATITIS HERPETIFORME (DURHING)
OTRAS :
•ERUPCIONES AMPOLLARES DE CAUSA EXTERNA
•ERUPCIONES AMPOLLARES MEDICAMENTOSAS
•ERUPCIONES AMPOLLARES DE ETIOLOGÍA INDECCIOSA
•PENFIGOIDE GESTATIONIS
•ENFERMEDADES AMPOLLARES HEREDITARIAS
•PÉNFIGO CRONICO FAMILIAR BENIGNO
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PENFIGOS:PENFIGOS:
ENFERMEDADES AUTOINMUNES ANTICUERPOS CIRCULANTES
COMPROMISO CUTÁNEO - MUCOSO
LESIÓN CUTÁNEA CARACTERÍSTICA: AMPOLLA ACANTOLISIS
INCIDENCIA: BAJA
EDAD: 4° - 5° DÉCADA DE LA VIDA
SEXO: MUJERES = VARONES
CURSO: CRONICO
I
ENFERMEDADES AMPOLLARES
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ETIOLOGÍA DESCONOCIDA
MULTIFACTORIAL
BASE GENÉTICA
PATOGENIA AUTOINMUNE
ANTICUERPOS CONTRA MOLÉCULAS DE ADHESIÓN INTERCELULAR
DESMOGLÉINAS DG 1 (P. SUPERFICIAL)
DG 3 (P. PROFUNDO)
ACTIVACIÓN DEL COMPLEMENTO
ENFERMEDADES AMPOLLARES
IFD: IgG Y COMPLEMENTO INTERCELULAR
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FORMAS CLÍNICAS:
PÉNFIGOS PROFUNDOS (SUPRABASALES)
PÉNFIGO VULGAR
PÉNFIGO VEGETANTE
PÉNFIGOS SUPERFICIALES (SUBCÓRNEOS)
PÉNFIGO SEBORREICO
PÉNFIGO FOLIÁCEO
ENFERMEDADES AMPOLLARES
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PENFIGO VULGAR
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PÉNFIGO VULGAR:
•MÁS FECUENTE: 80 %
•LESIONES CUTÁNEAS AMPOLLAS FLÁCIDAS
EROSIONES
COSTRAS
•LESIONES MUCOSAS 50 % MANIFESTACIÓN INICIAL
•SIGNO DE NIKOLSKY
•EVOLUCIÓN: BROTES AGRAVACIÓN DE ESTADO GENERAL
•COMPLICACIONES: MÚLTIPLES ORGANOS
MÁS GRAVE
EROSIONES
LESIONES RESIDUALES
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DIAGNÓSTICO:
•CLÍNICA
•BIOPSIA CUTÁNEA
•CITODIAGNÓSTICO
•INMUNOFLUORESCENCIA DIRECTA
INDIRECTA
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AMPOLLA SUPRABASAL
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IFD:
IgG Y COMPLEMENTO INTERCELULAR
IFI:
AC CIRCULANTESCONTRA DG 3
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PÉNFIGO VEGETANTE:
AMPOLLAS DE GRAN TAMAÑO
UBICACIÓN GRANDES PLIEGUES
BOCA
GENITALES
LABIOS
POCO FRECUENTE: MENOR DEL 2 %
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PÉNFIGO SEBORREICO, ERITEMATODES O SÍNDROME DE SENEAR-USHER:
AMPOLLAS SUPERFICIALES Y FLÁCIDAS
UBICACIÓN CARA
TRONCO
COMPROMISO MUCOSO: POCO FRECUENTE
ESTADO GENERAL: BUENO
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PÉNFIGO FOLIÁCEO:
•FORMA ERITRODERMICA DEL PÉNFIGO SEBORREICO
•ESCAMAS HÚMEDAS Y MACERADAS
•MUCOSAS GENERALMENTE RESPETADAS
•BRAZIL: PENFIGO FOLIÁCEO ENDÉMICOPENFIGO FOLIÁCEO ENDÉMICO FAMILIAR
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AMPOLLA SUBCORNEA
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PÉNFIGO PARANEOPLÁSICO:
ENFERMEDAD MUCOCUTÁNEA AMPOLLAR EROSIVA
ESTOMATITIS ULCEROSA DOLOROSA
COMPROMISO MULTIORGÁNICO
TRATAMIENTO:
CORTICOESTEROIDES
TRATAMIENTO DE LA ENFERMEDAD DE BASE
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TRATAMIENTO PENFIGOS:
SISTÉMICO:
•ESTRICTO CONTROL DEL MEDIO INTERNO
• CORTICOSTEROIDES
• TERAPEÚTICAS COMBINADAS: CORTICOIDES + IS
MTX
CF
AZT•PLASMAFÉRESIS
LOCAL
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PENFIGOIDE AMPOLLAR (LEVER):
AMPOLLAS GRANDES Y TENSAS
INGLES AXILAS SUPERFICIES DE FLEXIÓN
ESCASO COMPROMISO DE MUCOSAS
ESTADO GENERAL GENERALMENTE BUENO
HISTOPATOLOGÍA: AMPOLLA SUBEPIDERMICA IFD: + : MB
IFI : + : Ac anti MB
TRATAMIENTO: CORTICOESTEROIDES
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DIAGNÓSTICO:
•CLÍNICA
•BIOPSIA CUTÁNEA
•CITODIAGNÓSTICO
•INMUNOFLUORESCENCIA DIRECTA
INDIRECTA
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AMPOLLA SUBEPIDÉRMICA
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IFD: IgG y COMPLEMENTO A LO LARGO DE LA MEMBRANA BASAL
IFI: AC ANTI MB
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PENFIGOIDE CICATRIZAL:
•COMROMISO MUCOSO PREDOMINANTE
•AMPOLLAS CUTÁNEAS
•SINEQUIAS
•CICATRICES IRREVERSIBLES
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DIAGNÓSTICOS DIFERENCIALES:
•PENFIGOS
•PENFIGOIDE AMPOLLAR
•ERITEMA POLIMORFO AMPOLLAR
•TOXIDERMIAS AMPOLLARES
•PÉNFIGO CRÓNICO BENIGNO FAMILIAR
CITODIAGNÓSTICO DE TZANCK
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ENFERMEDAD DE DUHRING:
•INCIDENCIA: POCO FRECUENTE
•DOLOR, PRURITO, ARDOR
•ERUPCIÓN POLIMORFA CON TENDENCIA A LA SIMETRÍA
•COMPROMISO MUCOSO
•EVOLUCIÓN: BROTES
•CONSERVACIÓN DEL BUEN ESTADO GENERAL
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AMPOLLAS SUBEPIDÉRMICAS
ETIOLOGÍA: ENTEROPATÍA SENSIBLE AL GLUTEN
HISTOPATOLOGÍA:
TRATAMIENTO: DAPSONA
OTROS: SULFAPIRIDINA, CORTICOESTEROIDES DIETA LIBRE DE TAAC
DERMATITIS HERPETIFORME
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IFD CUTÁNEA: IgG A GRANULAR Y COMPLEMENTO
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