Graft‐versus‐host disease‐like erythroderma: a manifestation of thymoma‐associated multiorgan autoimmunity
- 28 October 2015
- journal article
- case report
- Published by Wiley in Journal of Cutaneous Pathology
- Vol. 42 (10), 663-668
- https://doi.org/10.1111/cup.12642
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.Keywords
This publication has 23 references indexed in Scilit:
- Diminished regulatory T cells in cutaneous lesions of thymoma-associated multi-organ autoimmunity: a newly described paraneoplastic autoimmune disorder with fatal clinical courseClinical and Experimental Immunology, 2011
- Chemotherapy improves thymoma-associated graft-versus-host-disease-like erythrodermaBMJ Case Reports, 2011
- An unusual case of malignant thymoma associated graft-versus-host diseaseBMJ Case Reports, 2009
- Thymoma-associated Graft-versus-Host Disease-like ErythrodermaJournal of Thoracic Oncology, 2007
- Graft-versus-host-like disease complicating thymoma: Lack of AIRE expression as a cause of non-hereditary autoimmunity?Immunology Letters, 2007
- Thymoma-associated multiorgan autoimmunity: A graft-versus-host–like diseaseJournal of the American Academy of Dermatology, 2007
- Full-blown graft-versus-host disease presenting with skin manifestations, jaundice and diarrhoeaEuropean Journal of Gastroenterology & Hepatology, 2003
- Graft-versus-Host Disease-like Syndrome in Malignant ThymomaScandinavian Journal of Gastroenterology, 2000
- Thymoma-associated cutaneous graft-versus-host-like reactionClinical and Experimental Dermatology, 1997
- The histological diagnosis of cutaneous graft versus host disease: relationship of skin changes to marrow purging and other clinical variablesHistopathology, 1987