@entity915 is a heterogeneous group of disorders characterized by defective T cell and B cell function. Eczematous and morbilliform @entity203 are common, and @entity2132 ( @entity2132 ) due to maternal engraftment has been documented. We sought to better characterize @entity915 -related cutaneous disease observed prior to BMT and to compare the @entity203 to conventional @entity2132 . Medical records of 51 @entity1 with @entity915 treated between 1982 and 1999 were reviewed. Ten of 51 (20%) had @entity1910 and evidence of maternal engraftment prior to BMT (study group). Eleven of 51 (22%) had no @entity1910 or evidence of engraftment pre-BMT but developed @entity2132 following transplant (control group). Skin biopsies were available for review for 8/10 of the study group and for 8/11 of the control group. Cutaneous findings consisted of a scaling, erythematous maculopapular @entity203 spread widely over the trunk and extremities, with near- @entity5658 in some @entity1 . Microscopically, biopsies from the study group differed significantly from controls. Key differences included @entity8286 (P < or = 0.01), @entity1373 (P < or = 0.04) and spongiosis (P < or = 0.04). The dermatopathologic findings of transplacental @entity2132 differ from the pattern of post-transplant @entity2132 . A 'psoriasiform-lichenoid-spongiotic' pattern with @entity39 keratinocytes should trigger consideration of @entity915 and maternal engraftment in the dermatopathologic evaluation of @entity203 of infancy.

Cutaneous manifestations of maternal engraftment in XXXX with severe combined immunodeficiency: a clinicopathologic study.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity1910 :: ('MESH:D005076', 'Disease') :: ['rash'] @entity39 :: ('MESH:D009336', 'Disease') :: ['necrotic'] @entity1373 :: ('MESH:D006965', 'Disease') :: ['psoriasiform hyperplasia'] @entity203 :: ('MESH:D003875', 'Disease') :: ['eruptions', 'eruption'] @entity8286 :: ('MESH:D010241', 'Disease') :: ['parakeratosis'] @entity915 :: ('MESH:D053632', 'Disease') :: ['SCID'] @entity2132 :: ('MESH:D006086', 'Disease') :: ['GVHD', 'graft-versus-host disease'] @entity5658 :: ('MESH:D003873', 'Disease') :: ['erythroderma']
@entity1 :: (9606,Species) :: ['patients']
