Thymoma-associated multiorgan autoimmunity with cutaneous only presentation: A case report

Abstract

Thymoma-associated multiorgan autoimmunity disease can be seen in patients with thymomas and presents with features of graft versus host disease. Here, we report a case of a 52-year-old woman with a complex medical history including malignant thymoma, myasthenia gravis, Good’s syndrome, and T cell large granular lymphocytic leukemia who presented with a diffuse pruritic rash ultimately found to be compatible with a cutaneous presentation of thymoma-associated multiorgan autoimmunity disease. The eruption heralded the recurrence of the malignant thymoma and the rash was ultimately found to be resistant to a multitude of therapies except for oral corticosteroids.

Erythroderma in a Patient With Thymoma-Associated Myasthenia Gravis

“…3 TAMA is a very JAMA Dermatology Clinicopathological Challenge rare disease, and its cutaneous manifestations are even less reported. [4][5][6][7] GVHD-like dermatitis with diffuse erythroderma with or without scaling located on the face, trunk, extremities, palms, and soles. Although the prevalence of TAMA is unknown, it appears to be higher among Asian populations.…”

“…[6][7][8][9] Resection of the thymoma is the most important way to improve the prognosis and is recommended whenever possible. 5 However, more than half of reported patients died from serious infectious diseases secondary to the use of high-dose systemic steroids 10 or chemotherapy for unresectable thymoma, 8 especially after GVHD-like erythroderma onset. 8 TAMA also has been associated with increased risk of opportunistic infections as well as progressive multifocal leukoencephalopathy.…”

“…TAMA is defined as an autoinflammatory disorder of the liver, intestines, or skin in patients with malignant thymoma who have not undergone hematopoietic stem cell transplant . TAMA is a very rare disease, and its cutaneous manifestations are even less reported . GVHD-like dermatitis with diffuse erythroderma with or without scaling located on the face, trunk, extremities, palms, and soles.…”

Cutaneous graft‐versus‐host‐like disease in a patient with fatal thymoma

“…3 It is hypothesized that self-reactive lymphocytes leave the thymus and attack the skin, liver, and colon in a manner that is almost histologically identical to graft-versus-host disease. 4 A 44-year-old man presented to the emergency department with a 1-year history of worsening skin lesions, previously diagnosed clinically as psoriasis, under topical therapy with a combination of corticosteroid and vitamin D analogs. The lesions were moderately pruritic and he presented no other complaints.…”

Kutane Graft‐versus‐Host‐ähnliche Erkrankung bei einem Patienten mit letal verlaufendem Thymom

“…3 Es wird angenommen, dass autoreaktive Lymphozyten den Thymus verlassen und die Haut, die Leber und den Dickdarm auf eine Weise angreifen, die histologisch fast identisch mit der Graft-versus-Host-Krankheit ist. 4 Ein 44-jähriger Mann stellte sich in der Notaufnahme mit sich seit einem Jahr verschlimmernden Hautläsionen…”