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A case of oral lichen planus with Good syndrome

  • Author(s): Lolis, Margarita
  • Levitt, Jacob
  • et al.
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A case of oral lichen planus with Good syndrome
Margarita Lolis, Jacob Levitt MD
Dermatology Online Journal 12 (6): 28



In 1954 Dr. Robert Good described an immunodeficient state with thymoma and hypogammaglobulinemia [1]. Subsequently, many cases have been reported associated with myasthenia gravis, chronic diarrhea, anemia from pure red cell aplasia, or chronic mucocutaneous candidiasis. A patient with Good syndrome was recently treated in our office for oral lichen planus. A review of the literature revealed two reported cases of Good syndrome associated with oral lichen planus [2, 3]. Our patient is a 50-year-old white woman with biopsy-proven oral lichen planus, Good syndrome, and biopsy-proven bronchiolitis obliterans pneumonia (BOOP)—presumably because of chronic inflammation from recurrent pulmonary infections. The patient was healthy until early 2000 when she developed a persistent cough and painful mouth sores. In May 2000, a chest x-ray revealed a large thymoma, which was resected (pathology not available). The thymoma coupled with recurrent pulmonary infections prompted hematologic work-up revealing hypogammaglobulinemia, lack of B cells, normal T cell and T helper cell populations, and increased CD8 (T cytotoxic/suppressor) subset values. Treatment initially with prednisone and then with cyclosporine (3mg/kg/day) has been effective in controlling both her BOOP and oral lichen planus.

Good Syndrome occurs in 7 percent of adults with primary immunoglobulin deficiency. Although the accepted defining features of Good syndrome are immunodeficiency involving thymoma with hypogammaglobulinemia, we suggest that other rare, yet associated disease states, should prompt strong suspicion for this syndrome. Specifically, oral lichen planus (as highlighted in the current case) [2, 3], chronic mucocutaneous candidiasis (diagnosed in 24 percent of cases) [1, 4], and pure red cell aplasia (diagnosed in 35 percent of cases) [1, 2, 5] are aberrantly common in this syndrome. Although epithelioid cell thymoma has been reported, spindle cell thymoma is overwhelmingly more frequent and is independently suggestive of Good syndrome [1]. The average interval between the onset of initial symptoms and the diagnosis of Good Syndrome is 6 years [1]. These associations can be very helpful for its early recognition given that they may predate the onset of symptomatic hypogammaglobulinemia. Expanding our current clinical picture of Good syndrome to include the aforementioned diseases may not only avoid diagnostic delay, but also lead to studies that will help us better understand its underlying pathophysiology.

References

1. Kelleher, P., Misbah, S.A. What is Good's syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol 2003;56:12-16.

2. Grendelmeier, P, Conen, D. Thymoma with pure red cell aplasia (PRCA), Good's syndrome and lichen ruber planus. Schweiz Rundsch Med Prax. 2003;92(13):606-8.

3. Idogawa, M., Yuji H., Toshiaki H., Tadao I., Kohzoh, I. A case of thymoma and hypogammaglobulinemia (Good's syndrome) with lichen planus, Nihon Rinsho Meneki Gakkai Kaishi. 1999;22(3):137-43.

4. Kaneko, F., Tsuchiya, K., Miura, Y., Kishiyama, K., Kusakabe, Y., Matsumoto, S., et al. Clinical observations on a case of immuno-deficiency and thymoma (Good's syndrome) associated with chronic mucocutaneous candidiasis. J Dermatol. 1982;9(5):355-65.

5. Sato, M., Nagai, H., Kurasima, A., Yotsumoto, H., Mohri, M., Tanaka, K., et al. Lung abscess in a patient with Good's syndrome and pure red cell aplasia. Nihon Kokyuki Gakkai Zasshi.1998;36(2):187-91.

© 2006 Dermatology Online Journal