RESUMEN
A 58-year-old Japanese male visited us with painful lesions on the lower lip, oral mucosa and genital region of an 8-month duration. Histological features of the genital lesion were almost consistent with lichenoid tissue reaction. A few intraepidermal acantholytic keratinocytes were also seen in the suprabasal clefts. Direct immunofluorescence exhibited cell surface immunoglobulin (Ig)G deposition and linear deposition of fibrinogen at the dermoepidermal junction. IgG anti-desmoglein (Dsg)3 antibody, but not anti-Dsg1 antibody, was detected in the patient's serum by enzyme-linked immunosorbent assay. Immunoblotting using normal human epidermal extract detected the 210-kD envoplakin, 190-kD periplakin and 130-kD Dsg3. The diagnosis of paraneoplastic pemphigus (PNP) was made. Subsequent investigation revealed a large space-occupying lesion in the liver. Histological findings from liver biopsy specimen were consistent with hepatocellular carcinoma. The patient has been alive 38 months after the diagnosis of PNP was made, although the liver mass has slowly enlarged. Our case is clinically and histologically similar to erosive mucosal lichen planus. Immunological studies confirmed the diagnosis of PNP. The results of negative Dsg1 and positive Dsg3 were consistent with clinical features showing severe mucosal involvement without cutaneous erosion. In PNP, the association with non-hematological solid tumor is extremely rare.
Asunto(s)
Carcinoma Hepatocelular/diagnóstico , Liquen Plano Oral/diagnóstico , Liquen Plano/diagnóstico , Enfermedades de los Labios/diagnóstico , Neoplasias Hepáticas/diagnóstico , Síndromes Paraneoplásicos/diagnóstico , Pénfigo/diagnóstico , Enfermedades del Pene/diagnóstico , Biomarcadores de Tumor/análisis , Desmogleína 1/análisis , Desmogleína 3/análisis , Desmosomas/ultraestructura , Fibrinógeno/análisis , Técnica del Anticuerpo Fluorescente Directa , Humanos , Inmunoglobulina G/análisis , Queratinocitos/patología , Masculino , Proteínas de la Membrana/análisis , Persona de Mediana Edad , Plaquinas/análisis , Precursores de Proteínas/análisisAsunto(s)
Piodermia Gangrenosa/complicaciones , Piodermia Gangrenosa/diagnóstico , Arteritis de Takayasu/complicaciones , Arteritis de Takayasu/diagnóstico , Adulto , Femenino , Glucocorticoides/uso terapéutico , Humanos , Cuello/patología , Prednisolona/uso terapéutico , Piodermia Gangrenosa/tratamiento farmacológico , Piel/patología , Arteritis de Takayasu/tratamiento farmacológicoAsunto(s)
Dermatitis Alérgica por Contacto/etiología , Dermatitis Profesional/etiología , Níquel/inmunología , Estetoscopios/efectos adversos , Adulto , Dermatitis Alérgica por Contacto/diagnóstico , Dermatitis Profesional/diagnóstico , Femenino , Estudios de Seguimiento , Humanos , Cuello , Enfermería , Pruebas del ParcheAsunto(s)
Condiloma Acuminado/patología , Neoplasias del Pene/patología , Tumor de Buschke-Lowenstein , Condiloma Acuminado/virología , Papillomavirus Humano 11/genética , Papillomavirus Humano 11/aislamiento & purificación , Papillomavirus Humano 6/genética , Papillomavirus Humano 6/aislamiento & purificación , Humanos , Hibridación in Situ , Masculino , Persona de Mediana Edad , Neoplasias del Pene/virologíaRESUMEN
A 75-year-old Japanese male visited us with bullous eruptions on the extremities. Physical examination revealed large bullae on the hands, lower legs and feet. The oral mucosa was also involved. Histology disclosed subepidermal blister with inflammatory cell infiltrates in the dermis. Direct immunofluorescence showed deposits of IgG and IgA at the cutaneous basement membrane zone. Indirect immunofluorescence on 1 M NaCl-split human skin sections demonstrated that the patient's IgG antibodies reacted with the dermal side of the split, while IgA antibodies reacted with the epidermal side. Immunoblotting showed that the patient's serum reacted with the NC1 domain of type VII collagen (290-kDa epidermolysis bullosa acquisita antigen) as well as the 120-kDa linear IgA bullous dermatosis antigen, LAD-1. Systemic prednisolone resulted in a favorable response. From the clinicopathological findings, the present case is not consistent with either epidermolysis bullosa acquisita or IgA bullous dermatosis. Therefore, we regarded the case as mixed bullous disease of epidermolysis bullosa acquisita and linear IgA bullous dermatosis. Such a case has not been previously reported.