RESUMEN
OBJECTIVE: Pemphigus is an autoimmune blistering disease of skin and mucous membranes. Pemphigus vulgaris (PV) is a major subtype of pemphigus, which is histologically characterized by suprabasal acantholysis. The major antigen in PV is desmosomal glycoproteins desmoglein (Dsg) 3. The autoantibodies against Dsg3 cause loss of adhesion between keratinocytes. Some PV patients also have circulating anti-Dsg1 autoantibodies. Enzyme-linked immunosorbent assays (ELISAs) with recombinant Dsg3 and Dsg1 are highly sensitive and specific for detecting anti-Dsg3 and anti-Dsg1 autoantibodies in PV patients. To evaluate the role of desmosomal glycoproteins desmoglein (Dsg3) ELISA and Dsg1 ELISA for detecting anti-Dsg3 and anti-Dsg1 autoantibodies in monitoring disease activity in Pemphigus vulgaris patients. METHODS: Twenty PV patients with long-term follow-up were included. We tested their serial sera with modified Dsg3 ELISA (MESACUP Desmoglein TEST "Dsg3", Medical & Biological Laboratories Co. LTD.), Dsg1 ELISA(MESACUP Desmoglein TEST "Dsg1", Medical & Biological Laboratories Co. LTD.) and indirect immunofluorescence (IIF). Then we analyzed the correlation between Dsg3 ELISA index values, Dsg1 ELISA index values, IIF titres and disease activity scores (ABSIS) along the time course. RESULTS: There were significant correlations between Dsg3 ELISA index values, Dsg1 ELISA index values, IIF titres and disease activity scores (both skin scores and oral scores) (P<0.01) along the time course. Significant differences of Dsg3 ELISA index values, Dsg1 ELISA index values and IIF titres between active time-point group and clinical remission time-point group were also observed (P<0.01). We found that Dsg3 ELISA index values, Dsg1 ELISA index values and IIF titres fluctuated in parallel with disease activity, and ELISA index values were superior to IIF titres. CONCLUSION: Dsg3 ELISA index values fluctuating in parallel with disease activity are useful to monitor disease activity, predict flares or relapses and plan the schedules for tapering the drugs.
Asunto(s)
Autoanticuerpos/sangre , Desmogleína 3/inmunología , Pénfigo/diagnóstico , Pénfigo/inmunología , Adulto , Anciano , Biomarcadores/sangre , Desmogleína 1/inmunología , Ensayo de Inmunoadsorción Enzimática , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
BACKGROUND: Paraneoplastic pemphigus (PNP) is an autoimmune-related acquired bullous disease. Delayed diagnosis and treatment of this clinically rare disease often result in poor prognosis. METHODS: Between January 1999 and December 2009, 22 patients with confirmed PNP who underwent surgical resection of underlying tumors were enrolled in this study. Clinicopathologic characteristics, treatment options, and perioperative and long-term results were analyzed. RESULTS: Among 22 patients, 2 patients died of severe infection several weeks after surgery. Postoperative major complications included pulmonary infections (n = 10) and septicemia (n = 4). Respiratory symptoms persisted in 13 patients. Tumors were completely resected in 20 patients. Two patients whose tumors were not completely resected died of relapse 2 and 32 months after surgery. Two patients with completely resected tumors died of respiratory failure 10 and 24 months after surgery, respectively. One patient whose pathological result was follicular dentritic cell sarcoma had a relapse recently. The remaining 15 patients have survived till now. CONCLUSIONS: Early detection, prompt treatment, and complete resection of PNP can effectively decrease the mortality and speed up the recovery.
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Neoplasias/cirugía , Síndromes Paraneoplásicos/patología , Pénfigo/patología , Adolescente , Adulto , Enfermedad de Castleman/cirugía , China , Células Dendríticas , Femenino , Humanos , Linfoma no Hodgkin/cirugía , Masculino , Persona de Mediana Edad , Pénfigo/etiología , Análisis de Supervivencia , Timoma/cirugía , Neoplasias del Timo/cirugía , Resultado del TratamientoRESUMEN
OBJECTIVES: To investigate the clinical and pathological subtypes of Castleman's disease (CD) and their relationship with complications. METHODS: The clinical complications of 53 patients with CD and the relationship of these complications with clinical and pathological subtypes were analyzed retrospectively. RESULTS: Among 53 CD patients, 32 (60.4%) were classified as uni-centric type and 21 (39.6%) multicentric type. Histopathological examination showed that 37 cases (69.8%) were hyaline vascular variants (HV), 9 (17.0%) plasmacytic variants (PC), and 7 (13.2%) mixed cellular variants (Mix). Complications were identified in 32 (60.4%) patients, including the involvements of skin, internal organs and hematopoietic system. Some complications were closely associated with the clinical subtype of CD: the majority of complications in the 32 uni-centric CDs were paraneoplastic pemphigus (PNP) and bronchiolitis obliterans (BO), and those in 21 multi-centric CDs were the involvements of kidney and hematopoietic system. The complications were different among the three kinds of histopathological subtypes: PNP and BO were the predominant complications of HV variants, while the internal organ and hematopoietic system involvements were those of PC and Mix variants. The clinical and histopathological classification of CD patients with PNP were different obviously from other subtypes of CDs. In Kaplan-Meier survival analysis, the survival rate of those with complications was significantly lower than those without complication (P = 0.028). CONCLUSION: The clinical complications of CDs are related to their clinical and histopathological subtypes. CD patients with PNP should be considered as a unique entity to tailor the therapy. The presence of clinical complications is an independent prognostic factor in CD patients.
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Enfermedad de Castleman/complicaciones , Enfermedad de Castleman/patología , Adolescente , Adulto , Anciano , Enfermedad de Castleman/diagnóstico , Niño , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Adulto JovenRESUMEN
Research in the field of genetic skin disease has grown rapidly over the past two decades. Even though the fundamental molecular pathways are still not fully understood, there have prominent advances in our understanding of the underlying mechanisms involved in the pathogenesis of genodermatosis. Dermatologists in China contributed to this field in recent years. They found the causative genes involved in primary erythermalgia and familial trichoepithelioma. Different gene mutations involved in more than twenty kinds of genodermatosises have been detected. This review will synthesize recent findings of the genodermatosises in China.