Worsening rash in female with history of cord blood transplant

CORRECT DIAGNOSIS:

Chronic Plaque Psoriasis

DISCUSSION:

Psoriasis is a well-regarded, complex T-cell mediated autoimmune disorder. The interplay between host predisposition and the environmental trigger is a home-run, cover-our-bases type of explanation that has been capitalized on through the exploration of various interleukin inhibitors and immune system suppressors. Clearly, the successful clearance of psoriatic plaques with cyclosporine and IL-17 and 17R inhibitors highlights the role of the regulatory T cell in the development of psoriasis. The development of psoriasis after stem-cell transplant in a patient without psoriasis also confers the possibility of direct T-cell transfer of psoriasis from the mature donor T-cells. However, here we report a case of psoriasis that developed after umbilical cord blood transplant, which contains naive T cells, for a patient with an uncommon inherited immunodeficiency disorder. Our case draws attention to the chorus of immunologic events that may be required in addition to T lymphocyte abnormality in the production of psoriasis.

We describe the second case report of psoriasis developing after cord blood transplantation. Eight other cases of psoriasis onset after hematopoietic stem cell transplant have been reported, as well as fifty reported cases of other autoimmune disorders occurring after cord blood transplantation. The development and remission of autoimmune diseases, including psoriasis, has been reported in peripheral blood stem cell transplantation as well as bone marrow transplantation, and it is thought that the mature T lymphocytes transfer the donor’s disease, or lack of disease, to the recipient. That autoimmune disorders, including psoriasis, occur after cord blood transplantation, which transfers naive T lymphocytes to the recipient, supports the idea that the expression of autoimmune disease involves more than the transferred T lymphocytes. Patients with a history of stem cell transplantation who present with a new complaint should prompt physicians to consider autoimmune disorders as part of the differential.

TREATMENT:

We report a very unique case of a patient with a genodermatosis, namely hypohidrotic ectodermal dysplasia with immunodeficiency (AED-ID) inherited from a mother with signs of incontinentia pigmenti (IP), who developed severe chronic plaque psoriasis 11-years following cord blood transplant. Within 8 weeks of initiating etanercept, the patient’s psoriasis has mostly resolved. 

 

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