Cutaneous lesions in a CLIPPERS patient: Further confusion between CLIPPERS and grade I lymphomatoid granulomatosis

摘要

We read with great interest the recent article entitled 'Neurotrophic T-cell lymphocytosis: a cutaneous expression of CLIPPERS' by Smith et al.Criteria for CLIPPERS include (1) brainstem signs and symptoms, (2) brain-stem punctuate and curvilinear gadolinium enhancing lesions, (3) steroid sensitivity with possible clinical and radiological sequelae, (4) relapsing-remitting course with steroid dependence, (5) a lymphohistiocytic infiltrate around and sometimes in vessel walls with predominance of CD4-cells and scarce B-cells on brainstem biopsy, and (6) absent evidence of an alternative diagnosis. The underlying patho-physiology of CLIPPERS remains unknown. An autoimmune hypothesis has been proposed.