Interact CardioVasc Thorac Surg 2009;9:345-346. doi:10.1510/icvts.2009.207308
© 2009 European Association of Cardio-Thoracic Surgery
Brief communication - Thoracic oncologic |
Lichen planus in a case of Good's syndrome (thymoma and immunodeficiency)
Sujoy Khana,*,
Anne Campbellb,
Carol Huntc and
W.A. Carrock Sewella,d
a Path Links Immunology, Scunthorpe General Hospital, DN15 7BH, UK
b Department of Histopathology, Hull Royal Infirmary, Hull, HU3 2JZ, UK
c Path Links Histopathology, Scunthorpe General Hospital, DN15 7BH, UK
d Hull York Medical School, Hull, HU6 7RX, UK
Received 13 March 2009;
received in revised form 20 April 2009;
accepted 5 May 2009
 Conflict of interest: The authors declare that they have no conflict of interest.
*Corresponding author. Tel.: +44-1724-387820; fax: +44-1724-865680.
E-mail address: sujoykhan{at}gmail.com (S. Khan).
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Abstract
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Patients with thymoma are mostly investigated for autoimmunity but a few patients may have underlying immunodeficiency that is referred to as Good's syndrome (GS). Cardiothoracic surgeons must always consider this diagnosis when undertaking thymectomy, as immunoglobulin levels can be easily measured and is readily available. The immunodeficiency in GS can be life-threatening and more importantly, it is not reversed by thymectomy. Collaborative care with an Immunologist for these patients is strongly recommended.
Key Words: Thymoma; Immunodeficiency; Good's syndrome; Lichen planus
Patients with thymoma may have underlying immunodeficiency, a condition referred to as Good's syndrome (GS). Although this syndrome has now been described for more than five decades [1], lack of formal diagnostic criteria and absence of confirmatory genetic testing are perhaps the reasons for diagnostic delay in most cases. The World Health Organization (WHO)/International Union of Immunological Societies (IUIS) on primary immunodeficiencies have classified GS as a distinct entity [2]. The likely incidence of GS is considered to be 1–2% [3]. The immunodeficiency may precede or follow the diagnosis of a thymoma [4], and mortality in GS is higher than in other primary immunodeficiencies that present in a similar age group to GS [4]. Some patients have low CD4+ T cells with abnormal CD4:CD8 ratio and defective T cell proliferation [3]. We would like to share our experience of managing a case of GS who developed lichen planus three years after thymectomy and is presently on immunoglobulin replacement therapy.
The patient was 58 years old when he was referred to chest physicians with a three-year history of recurrent chest infections. Radiological investigations revealed bilateral basal bronchiectasis and a mediastinal shadow, which was histologically confirmed to be thymoma of a mixed (composite) type or type AB according to the WHO classification (Fig. 1a). He underwent thymectomy and was discharged. He continued to suffer from chest infections and antibody levels that were requested more than a year after thymectomy showed panhypogammaglobulinaemia (Table 1). Lymphocyte subsets showed complete B cell lymphopenia. A diagnosis of GS was made and the patient was started on intravenous immunoglobulin (IVIG) replacement therapy. Three years later, he developed a rash on his back with histological features of lichen planus (Fig. 1b, c). He was prescribed a potent topical steroid cream (betamethasone valerate 0.1%) and the rash slowly settled in 6 months. Fungal nail infections have remained resistant to therapy (Fig. 1d) suggesting a T cell defect that remains uncharacterized in this disorder. Although his IgG levels have remained >8.0 g/l for over two years (Table 1), he continues to have chest infections and remains almost constantly on antibiotics.
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Fig. 1. (a) CD45 immunohistochemistry stains of the thymus that highlights the lymphocyte infiltration of the thymus (dark brown) – type AB WHO classification. (b) Macroscopic view of resolving lichen planus. (c) Skin biopsy showed mild superficial chronic inflammation with focal interface dermatitis, melanophages and ecstatic capillaries, features of late-stage lichen planus. (d) Persistent fungal nail infections.
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The Journal had a case report by Akio Hayashi and colleagues on a patient with resistant lichen planus and thymoma [5]. The patient was on treatment with itraconazole for oral candidiasis for two years, which leads us to speculate as to whether the patient had GS with an underlying T cell defect. The authors have not reported on the serum immunoglobulin levels or lymphocyte subsets (CD4+/CD8+ T cells or CD19+ B cell numbers), that could have made the diagnosis more certain as patients with GS have antibody deficiency (hypogammaglobulinaemia).
Cardiothoracic surgeons must always consider the diagnosis of GS when undertaking thymectomy, as the resulting immunodeficiency is life-threatening and not always reversed by thymectomy; collaborative care with an immunologist is strongly recommended.
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References
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- Good RA, Varco RL. A clinical and experimental study of agammaglobulinemia. J Lancet 1955;75:245–271.[Medline]
- Primary Immunodeficiency Diseases. Report of an IUIS scientific committee. International Union of Immunological Societies. Clin Exp Immunol 1999;118:1–28.[Medline]
- Kelleher P, Misbah SA. What is Good's syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol 2003;56:12–16.[Free Full Text]
- Blore J, Haeney MR. Primary antibody deficiency and diagnostic delay. Br Med J 1989;298:516–517.[Free Full Text]
- Hayashi A, Shiono H, Okumura M. Thymoma accompanied by lichen planus. Interact CardioVasc Thorac Surg 2008;7:347–348.[Abstract/Free Full Text]
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