Asunto(s)
Antirreumáticos/efectos adversos , Dermatitis Alérgica por Contacto/patología , Tiosulfato Sódico de Oro/efectos adversos , Enfermedades de Inicio Tardío/patología , Adulto , Dermatitis Alérgica por Contacto/etiología , Femenino , Humanos , Enfermedades de Inicio Tardío/etiología , Pruebas del Parche , Adulto JovenRESUMEN
We report a 68-year-old Japanese female patient with subepidermal blistering disease with autoantibodies to multiple laminins, who subsequently developed membranous glomerulonephropathy. At skin disease stage, immunofluorescence demonstrated IgG anti-basement membrane zone antibodies reactive with dermal side of NaCl-split skin. Immunoblotting of human dermal extract, purified laminin-332, hemidesmosome-rich fraction and laminin-521 trimer recombinant protein (RP) detected laminin γ-1 and α-3 and γ-2 subunits of laminin-332. Three years after skin lesions disappeared, nephrotic symptoms developed. Antibodies to α-3 chain of type IV collagen (COL4A3) were negative, thus excluding the diagnosis of Goodpasture syndrome. All anti-laminin antibodies disappeared. Additional IB and ELISA studies of RPs of various COL4 chains revealed reactivity with COL4A5, but not with COL4A6 or COL4A3. Although diagnosis of anti-laminin γ-1 (p200) pemphigoid or anti-laminin-332-type mucous membrane pemphigoid could not be made, this case was similar to previous cases with autoantibodies to COL4A5 and/or COL4A6.
Asunto(s)
Autoanticuerpos/análisis , Enfermedades Autoinmunes/inmunología , Vesícula/inmunología , Colágeno Tipo IV/inmunología , Glomerulonefritis Membranosa/inmunología , Riñón/inmunología , Laminina/inmunología , Piel/inmunología , Anciano , Autoanticuerpos/sangre , Enfermedades Autoinmunes/sangre , Enfermedades Autoinmunes/diagnóstico , Enfermedades Autoinmunes/terapia , Biopsia , Vesícula/sangre , Vesícula/diagnóstico , Vesícula/terapia , Femenino , Técnica del Anticuerpo Fluorescente , Glomerulonefritis Membranosa/sangre , Glomerulonefritis Membranosa/diagnóstico , Glucocorticoides/uso terapéutico , Humanos , Riñón/ultraestructura , Intercambio Plasmático , Valor Predictivo de las Pruebas , Subunidades de Proteína , Piel/efectos de los fármacos , Piel/patología , Factores de TiempoAsunto(s)
Síndrome de Hipersensibilidad a Medicamentos/patología , Seudolinfoma/diagnóstico , Piel/patología , Ácido Valproico/efectos adversos , Adulto , Antimaníacos/efectos adversos , Carbamazepina/efectos adversos , Diagnóstico Diferencial , Síndrome de Hipersensibilidad a Medicamentos/etiología , Humanos , MasculinoAsunto(s)
Cloruros/uso terapéutico , Pomadas/uso terapéutico , Neoplasias Cutáneas/tratamiento farmacológico , Compuestos de Zinc/uso terapéutico , Anciano , Antígenos CD34/análisis , Resultado Fatal , Femenino , Hemangiosarcoma/tratamiento farmacológico , Hemangiosarcoma/patología , Humanos , Linfangiosarcoma/tratamiento farmacológico , Linfangiosarcoma/patología , Molécula-1 de Adhesión Celular Endotelial de Plaqueta/análisis , Neoplasias Cutáneas/patologíaRESUMEN
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) may be fatal. Although classified by body surface area skin detachment, initial stages of both may present with erythema multiforme (EM)-like lesions. To diagnose and predict disease activity adequately as early as possible for patients revealing EM-like lesions, we performed frozen-section diagnosis. Thirty-five patients clinically diagnosed as EM, SJS or TEN were biopsied to diagnose and predict disease progression within the initial-visit day. Half of a histological section taken from a lesion was snap-frozen and immediately cryostat-sectioned, acetone-fixed and stained with hematoxylin-eosin. Specimens were examined with light microscopy for presence of epidermal necrosis. A section from unaffected sites was also examined for 11 patients. Specimens were examined with light microscopy for presence of graft-versus-host reaction (GVHR)-like findings: apoptotic keratinocytes and satellite cell necrosis. Epidermal necrosis was seen in nine patients. Initial diagnosis of the nine was one of overlap SJS-TEN, four of SJS and four of EM, and final diagnosis of those was one of TEN, one of overlap SJS-TEN, four of SJS and three of EM. Dissociation between initial and final diagnosis was seen in three cases. GVHR-like findings in the epidermis were observed in two patients finally diagnosed as overlap SJS-TEN and TEN. Frozen sections are useful not only to make a diagnosis of erythema multiforme but to assess a potential to exhibit more aggressive clinical behaviors (SJS or TEN).