Graft Versus Host Disease-Like: A New Paraneoplastic Syndrome
March 2010
in “
Journal of The American Academy of Dermatology
”
chronic graft versus host disease GVHD thymoma thymectomy erythematous scaling patches pruritus erythrodermia hyperpigmentation sclerodermoid changes generalized hair loss oral mucous ulcerations autoimmune regulator gene AIRE chronic GVHD thymus gland tumor thymus gland removal itching red skin patches skin darkening skin hardening hair loss mouth ulcers
TLDR A woman with thymoma developed a rare chronic condition similar to graft versus host disease after surgery.
The document reports on a case of a 39-year-old woman with a history of myasthenia gravis who developed chronic graft versus host disease-like (GVHD-like) symptoms following a thymoma diagnosis and subsequent thymectomy. Despite the rarity of thymoma, with an incidence of 0.15 cases per 100,000 people, it is associated with various neoplastic syndromes, including myasthenia gravis. The patient initially presented with erythematous scaling patches and pruritus, which progressed to erythrodermia, and later developed hyperpigmentation, sclerodermoid changes, generalized hair loss, oral mucous ulcerations, and episodes of self-limited diarrhea. Laboratory tests showed normocytic normochromic anemia and elevated levels of alkaline phosphatase and lactate dehydrogenase. A skin biopsy confirmed chronic GVHD. This case is notable as it represents the first described instance of a chronic GVHD-like presentation associated with thymoma, expanding the understanding of autoimmune diseases potentially linked to thymoma and the role of the autoimmune regulator gene (AIRE) in these conditions.
