Overlap between @entity2631 , progressive XXXX and immune-inflammatory @entity198 in a paediatric cohort.

@entity1 @entity198 @entity2631 @entity5043 @entity14752 @entity2704 @entity302 @entity10
UNASSIGNED: @entity2631 en coup the sabre (LSCS), progressive @entity5043 (PFH) and @entity14752 are distinct clinical entities, although @entity1 with overlapping features have been reported. We performed a multicenter retrospective review of a series of @entity1 with LSCS and/or PFH to explore the relation between these entities. The files of 16 @entity1 were reviewed, 11 presented with LSCS, 5 with PFH, with time overlapping cutaneous features were seen. Extracutaneous signs were found in both groups. ANA were present in more than 50  % of @entity1 . Almost half of our @entity1 presented with CNS manifestations comprising @entity10 , @entity2704 and @entity302 with or without abnormalities on MRI. Brain biopsy in one @entity1 was consistent with @entity198 . In two other @entity1 , associated autoimmune manifestations were present. CONCLUSION: Our @entity1 cohort brings more arguments to consider LSCS and PFH as a single disease entity with LSCS and superficial skin involvement at one end of the spectrum and PFH with involvement of subcutaneous deep tissue at the other end. In both entities, @entity198 can be observed. Our findings of circulating ANA, intradermal lymphocytes and IgG, intrathecal IgG production and clinical improvement with immunosuppressive therapy endorse the concept of a possible common immune-inflammatory pathogenesis. What is known:     LSCS, PFH and immune-inflammatory @entity198 are distinct clinical entities, but @entity1 with overlapping features have been reported. What is new:     We present a unique paediatric cohort with LSCS, PFH and/or @entity198 .     We endorse the concept of a common immune-inflammatory disease process.
