Graft‐versus‐host disease‐like erythroderma: a manifestation of thymoma‐associated multiorgan autoimmunity

Abstract

Thymoma associated multiorgan autoimmunity is a rare paraneoplastic disorder, clinicopathologically similar to graft versus host disease, which is thought to be mediated by dysfunctional negative thymocyte selection and abnormally low levels of Tregs. We report a 50 year old Chinese women with a history of malignant thymoma and myasthenia gravis who developed graft versus host disease- like erythroderma after instituting chemotherapy and undergoing myasthenia crisis. Clinically her rash presented as erythematous scaly papules, which evolved to psoriasiform patches and plaques with foci of vitiligo. Histopathologically the biopsy showed a predominantly interface dermatitis with necrotic keratinocytes extending to the upper levels of the epidermis, and florid basket weave orthokeratosis. Clinical and laboratory work-up ruled out common inflammatory or infectious causes, eventually favoring the diagnosis of TAMA with GVHD-like erythroderma. Unfortunately, the patient underwent multi-organ compromise and death due to respiratory failure from myasthenia crisis. Patients with TAMA have a poor clinical outlook; rare successful treatments include high dose oral steroids and additional modalities including bone marrow transplant and chemotherapeutic or biologic agents. As the predominant findings are in the skin, dermatologists and dermatopathologists are in a unique position to enable the early diagnosis and treatment of this unusual disease.

Thymoma‐associated multi‐organ autoimmunity: A case of graft‐versus‐host disease‐like erythroderma complicated by Good syndrome successfully treated by thymectomy

“…It is usually distinguished from other thymoma‐associated paraneoplastic disorders such as myasthenia gravis (MG) and pure red cell aplasia . TAMA is often lethal; half of cases die from serious infectious diseases subsequent to immunosuppression induced by chemotherapy for unresectable thymoma or systemic use of steroids for autoimmune reactions …”

“…7 TAMA is often lethal; half of cases die from serious infectious diseases subsequent to immunosuppression induced by chemotherapy for unresectable thymoma or systemic use of steroids for autoimmune reactions. 2,3 The pathophysiology of TAMA remains unclear. In normal thymus, immature T cells undergo "negative selection", where T cells that interact strongly with "self-antigens" presented by the medullary epithelium cells are deleted.…”

“…Thymoma‐associated multi‐organ disease (TAMA) is a rare paraneoplastic disorder, clinicopathologically similar to graft‐versus‐host disease (GVHD) . The patients generally have an unfavorable clinical course, undergoing intensive immunosuppressant therapies for unresectable thymoma, GVHD‐like symptoms and accompanying autoimmune diseases . We herein report a case complicated by Good syndrome, who was successfully treated by resection of thymoma, without systemic steroids or any other immunosuppressant therapies.…”

Graft-versus-host disease-like erythroderma

“…[ 5 ] Of the 25 patients with TAMA with cutaneous manifestation, 80.0% (20/25) resulted in a fatal course, which agrees with the current understanding that cutaneous manifestations of TAMA are associated with a poor prognosis. [ 4 , 6 , 7 ]…”

“…Only necrotic keratinocytes were thinly scattered in the epidermis, but the immunohistochemical evaluation showed infiltration of more CD8+ than CD4+ T cells and a marked decrease in CD1a+ Langerhans cells in the epidermis, which correspond to observed characteristics of TAMA in our case. [ 6 , 7 ]…”