Cutaneous Manifestations of Thymoma-associated Multi-organ Autoimmunity: A Fatal Sign

Muramatsu, Ken; Fujita, Yasuyuki; Inokuma, Daisuke; Tsukinaga, Ichiro; Ito, Kenichiro; Nishiura, Youichi; Shimizu, Hiroshi

doi:10.2340/00015555-2367

Cited by 14 publications

(22 citation statements)

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“…The patient’s GVHD-like erythroderma promptly improved after thymectomy and the administration of intravenous methylprednisolone, which, however, was complicated by CMV pneumonitis and respiratory failure 1 month after operation and immunosuppression. Current treatment options for GVHD-like skin lesions related to TAMA include thymectomy, systemic glucocorticoids, intravenous immunoglobulin, cyclosporine and narrow-band ultraviolet B phototherapy, with varying results 3–5. Two case reports have described the resolution of skin lesions with complete resection of thymoma without the use of systemic glucocorticoids 6 7.…”

Section: Discussionmentioning

confidence: 99%

Thymoma-associated multiorgan autoimmunity

et al. 2019

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Thymoma-associated multiorgan autoimmunity is a relatively new term to describe the rare paraneoplastic syndrome that complicates thymoma, which can involve the thyroid, liver and intestine in addition to the skin. The pathology often indicates a graft-versus-host-like pattern commonly observed in recipients of an allogeneic haematopoietic cell transplant. We report a case of type B2 and B3 thymoma with invasion to the lung and pleura in a patient who presented with oral lichen planus and graft-versus-host-like erythroderma. The cutaneous lesions improved after complete resection of the thymoma in combination with systemic glucocorticoids, which was subsequently complicated by cytomegalovirus pneumonitis.

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Section: Discussionmentioning

confidence: 99%

Thymoma-associated multiorgan autoimmunity

et al. 2019

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“…[ 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 ]…”

Section: Discussionmentioning

confidence: 99%

“…The differential diagnosis can be wide—including dermatitis herpetiformis, psoriasis, lichen planus, pityriasis rosea, lichenoid drug eruption, viral infections, paraneoplastic pemphigus, and persistent pruritic eruption of adult-onset Still disease. [ 6 – 8 ] The most common pathologic findings of GVHD-like erythroderma are parakeratosis, necrotic keratinocytes, and interface and perivascular dermatitis. 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.…”

Section: Discussionmentioning

confidence: 99%

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Graft-versus-host disease-like erythroderma

et al. 2017

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Rationale:Thymomas are associated with numerous autoimmune disorders, such as myasthenia gravis (MG), pure red cell aplasia (PRCA), and systemic lupus erythematosus (SLE). However, graft-versus-host disease (GVHD)-like erythroderma is a relatively uncommon paraneoplastic disorder associated with thymomas and signifies a poor prognosis.Patient concerns:A 35-year-old woman with medical history significant for stage IVa type AB thymoma presented with patchy erythema over face, trunk, and extremities that failed to respond to topical steroids.Diagnosis:A contrast-enhanced computerized tomography (CECT) scan of the chest demonstrated tumors in the right mediastinum and right pleura. Percutaneous right mediastinal pleural biopsy confirmed recurrent thymoma (WHO type B3, Masaoka stage IVb). Histopathologic examination of her skin lesions revealed GVHD-like erythroderma.Interventions:The patient received chemotherapy and local thoracic radiotherapy, as well as corticosteroids.Outcomes:The eruptions gradually subsided with hyperpigmentation; however the patient eventually died of multiple organ failure.Lessons:GVHD-like erythroderma is an uncommon paraneoplastic disorder associated with thymomas. Though its pathogenesis still needs further research, prompt diagnosis and appropriate treatment can improve survival rate in patients.

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“…Treatments for TAMA have not yet been established: surgical resection, chemotherapies, and radiotherapies for thymoma, and immunosuppressive therapies and phototherapies for the skin have previously been reported (3,5,(7)(8)(9). Despite these treatments, however, patients have a progressive course with fatal outcome in some cases (3,10,11).…”

Section: Progressive Erythroderma Without Malignancy In the Skin: A Quizmentioning

confidence: 99%