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 T regs . 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.Thymomas are tumors of thymic epithelial cell origin and are the most common type of thymic neoplasm. They are often diagnosed incidentally on imaging, but can also present with thoracic mass effects such as cough, chest pain, and phrenic nerve palsy. Additionally, thymomas are frequently associated with paraneoplastic autoimmune disorders, the most common of which are myasthenia gravis, pure red cell aplasia, and hypogammaglobulinemia. While rare, several case studies 1-13 have also reported a condition termed thymoma-associated multiorgan autoimmunity (TAMA) affecting the skin, liver, and colon. Interestingly, the histopathologic findings in damaged organs resemble those of graftversus-host disease (GVHD) despite the lack of transplanted lymphocytes. GVHD-like erythroderma is rare and little is understood about its pathogenesis. Additionally, its

Thymoma-associated multiorgan autoimmunity with exclusive gastrointestinal tract involvement: case report and review of the literature

“…The 21 TAMA cases documented to date have shown a female predominance, with a mean age of 47 years at presentation [2 -5]. Clinical features of paraneoplastic autoimmunity have manifested before, after or synchronously with the diagnosis of thymoma [10]. Most cases were associated with malignant or incompletely excised tumor, although complete thymoma removal does not preclude subsequent development of TAMA (as in our patient).…”

“…GIT symptoms and / or pathology were reported in 10 of the 21 TAMA patients [10]. Of these, one patient had chronic intractable diarrhea which was not investigated endoscopically and another patient was found to have esophageal candidiasis, without any other specific GIT microscopic pathology [1,6].…”

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 …”

“…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.…”

Progressive Erythroderma without Malignancy in the Skin: A Quiz

“…Cutaneous manifestations of TAMA are described as keratotic papules, scaly plaques, morbilliform eruptions, psoriasiform lesions, or erythroderma (3)(4)(5). In histopathology, TAMA is characterized by interface and perivascular dermatitis with necrotic keratinocytes throughout the epidermis (6). Imaging of the chest is indispensable for the diagnosis of TAMA.…”