Introduction:Eruptive cutaneous collagenoma is non familial connective tissue nevi of unknown etiology presented with an abrupt onset.To date,the literatures on eruptive cutaneous collagenoma are extremely rare in chi...Introduction:Eruptive cutaneous collagenoma is non familial connective tissue nevi of unknown etiology presented with an abrupt onset.To date,the literatures on eruptive cutaneous collagenoma are extremely rare in china.Case present:Two women presented with multiple asymptomatic,skin-colored papules and nodules on the trunk and extremities with no systemic involvement.Histopathology revealed dense,coarse collagen fibers by hematoxylin-esoin stain and decreased,fragmented elastic fibers by Elastic stain compared with the normal skin.Basing on these findings,the diagnosis of eruptive cutaneous collagenoma was made.No specific treatment was given.Discussion:Eruptive cutaneous collagenoma is a very rare dermatosis that is often misdiagnosed as other connective tissue nevi.The pathogenesis is unclear,and also no efficient treatment is available.It is usually diagnosed based on clinical and histopathological findings.Conclusion:The present cases are relatively rare type of eruptive cutaneous collagenoma that provide more experience for clinician,and may be helpful for them to make correct diagnosis for suspicious cases.展开更多
This review will encompass definition, pathogenesis, renal clinical manifestations and treatment of immunoglobulin G4-related diseases( IgG4-RDs). IgG4-RD is a recently recognized clinical entity that often involves ...This review will encompass definition, pathogenesis, renal clinical manifestations and treatment of immunoglobulin G4-related diseases( IgG4-RDs). IgG4-RD is a recently recognized clinical entity that often involves multiple organs and is characterized by high levels of serum immunoglobulins G4, dense infiltration of IgG4+ cells and storiform fibrosis. Cellular immunity, particularly T-cell mediated immunity, has been implicated in the pathogenesis of IgG4-RDs. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulopathy and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis. IgG4-RD diagnosis should be based on specific histopathological findings, confirmed by tissue immunostaining, typical radiological findings and an appropriate clinical context. The first line treatment is the steroids with two warnings: Steroid resistance and relapse after discontinuation. In the case of steroid resistance, B cell depleting agents as rituximab represent the secondline treatment. In the case of relapse after discontinuation, steroid treatment may be associated with steroid sparing agents. Since the disease has been only recently identified, more prospective, long-term studies are needed to an improved understanding and a more correct and safe treatment.展开更多
基金Youth Science and technology project in science and education of Suzhou(KJXW2016026)。
文摘Introduction:Eruptive cutaneous collagenoma is non familial connective tissue nevi of unknown etiology presented with an abrupt onset.To date,the literatures on eruptive cutaneous collagenoma are extremely rare in china.Case present:Two women presented with multiple asymptomatic,skin-colored papules and nodules on the trunk and extremities with no systemic involvement.Histopathology revealed dense,coarse collagen fibers by hematoxylin-esoin stain and decreased,fragmented elastic fibers by Elastic stain compared with the normal skin.Basing on these findings,the diagnosis of eruptive cutaneous collagenoma was made.No specific treatment was given.Discussion:Eruptive cutaneous collagenoma is a very rare dermatosis that is often misdiagnosed as other connective tissue nevi.The pathogenesis is unclear,and also no efficient treatment is available.It is usually diagnosed based on clinical and histopathological findings.Conclusion:The present cases are relatively rare type of eruptive cutaneous collagenoma that provide more experience for clinician,and may be helpful for them to make correct diagnosis for suspicious cases.
文摘This review will encompass definition, pathogenesis, renal clinical manifestations and treatment of immunoglobulin G4-related diseases( IgG4-RDs). IgG4-RD is a recently recognized clinical entity that often involves multiple organs and is characterized by high levels of serum immunoglobulins G4, dense infiltration of IgG4+ cells and storiform fibrosis. Cellular immunity, particularly T-cell mediated immunity, has been implicated in the pathogenesis of IgG4-RDs. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulopathy and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis. IgG4-RD diagnosis should be based on specific histopathological findings, confirmed by tissue immunostaining, typical radiological findings and an appropriate clinical context. The first line treatment is the steroids with two warnings: Steroid resistance and relapse after discontinuation. In the case of steroid resistance, B cell depleting agents as rituximab represent the secondline treatment. In the case of relapse after discontinuation, steroid treatment may be associated with steroid sparing agents. Since the disease has been only recently identified, more prospective, long-term studies are needed to an improved understanding and a more correct and safe treatment.
