Volume 172, Issue 3 pp. 774-777
Case Report

Three difficult cases: the challenge of autoimmunity, immunodeficiency and recurrent infections in patients with Good syndrome

S.J. Arnold

Corresponding Author

S.J. Arnold

Department of Dermatology, Churchill Hospital, Old Road, Headington, Oxford, OX3 7LE U.K

Correspondence

Stephanie J. Arnold.

E-mail: [email protected]

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T. Hodgson

T. Hodgson

Department of Oral Medicine and Special Care Dentistry, Eastman Dental Hospital, London, U.K

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S.A. Misbah

S.A. Misbah

Department of Clinical Immunology, John Radcliffe Hospital, Oxford, U.K

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S.Y. Patel

S.Y. Patel

Department of Clinical Immunology, John Radcliffe Hospital, Oxford, U.K

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S.M. Cooper

S.M. Cooper

Department of Dermatology, Churchill Hospital, Old Road, Headington, Oxford, OX3 7LE U.K

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V.A. Venning

V.A. Venning

Department of Dermatology, Churchill Hospital, Old Road, Headington, Oxford, OX3 7LE U.K

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First published: 24 July 2014
Citations: 10
Funding sources None.
Conflicts of interest None declared.

Summary

Good syndrome (GS) is a rare, adult-acquired primary combined immunodeficiency syndrome arising in the context of previous or current thymoma. Patients with GS frequently develop recurrent sinopulmonary infections and are also at high risk of autoimmune manifestations, including skin conditions such as lichen planus. We report three middle-aged patients with GS complicated by multiple autoimmune and infectious manifestations. The combination of immunodeficiency, autoimmunity and recurrent infections seen in patients with GS continues to present a management challenge, particularly in patients with oral mucosal disease and recurrent candidiasis. Clinicians should be prompted to investigate an underlying immunodeficiency in patients with multiple autoimmune conditions and recurrent sinopulmonary infections.

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