Regauer, Sigrid MD; Beham-Schmid, Christine MD
To the Editor:
We would like to challenge Drs. Kerl and Cerroni’s reply to our letter regarding monoclonality in lichen sclerosus. It was never our intention to label lichen sclerosus as a pseudolymphoma or even lymphoma, as stated by Drs. Cerroni and Kerl in their reply. It was the suggestion of Dr. Citarella and coauthors to include lichen sclerosus into the group of dermatological disorders called pseudolymphoma. 1 We are WELL (!) aware of Hallopeau’s original historic publication 2 from 1889 as well as a plenitude of other, more recent, excellent publications with long-term clinical follow-up of LS. 3–5 Without doubt, however, there is a lack of knowledge and long-term follow-up of patients with documented monoclonal T-cells in their infiltrates. All investigators of LS agree on the non-neoplastic nature of the infiltrate at the present time where there is still a limited understanding of the etiology of LS, despite the first description of LS more than a century ago. 2 Of particular concern to us is the statement by Drs. Cerroni and Kerl that we are focusing on a single case and that we are losing the clinicopathologic overview. Quite to the contrary, we have collected data of more than 200 patients with LS. Although LS is a benign dermatosis, a fact no one denies, we have strong indications that patients with LS have an underlying systemic immune dysregulation. Recent publications describe autoimmune phenomena, such as autoantibodies to basement membrane material. 6–8 Our own preliminary serologic data of 50 patients with LS reveal low titer autoantibodies in more than half of the patients. These autoimmune phenomena are most likely not etiologically relevant but merely represent a secondary phenomenon due to extensive tissue damage. However, in approximately 65% of these patients, we were able to detect serologically complement deficiencies and T-cell mediated cellular immune deficiencies, such as lymphopenias, massive increases, or reductions of both suppressor and helper T-cells resulting in dramatic changes of the typical CD8:CD4 ratio (personal, not yet published data). These results suggest that the local abnormal lymphoid (non-neoplastic) infiltrate in LS with occasional monoclonality may be an indication of an associated systemic cellular T-cell immune dysregulation. In light of these observations, we propose that patients with LS and /or the demonstration of an abnormal T-cell infiltrate in LS should be tested serologically for an underlying systemic immune dysregulation, since a defective T-cell regulation and response may put these patients at a higher risk for other (possibly neoplastic) diseases.
Sigrid Regauer, MD
Christine Beham-Schmid, MD
Medical University of Graz, Institute of Pathology, Graz, Austria
REFERENCES
1. Citarella L, Massone C, Kerl H, et al. Lichen sclerosus with histopathologic feature simulating early mycosis fungoides.
Am J Dermatopathol. 2003;25:463–465.
2. Hallopeau H. Lichen plan scléreux.
Ann Derm Syph. 1889;10:447–449.
3. Harrington CI, Dunsmore IR. An investigation into the incidence of auto-immune disorders in patients with lichen sclerosus and atrophicus.
Br J Dermatol. 1981;104:563–566.
4. Meyrick Thomas RH, Ridley CM, McGibbon DH, et al. Lichen sclerosus et atrophicus and autoimmunity - a study of 350 women.
Br J Dermatol. 1988;118:41–46.
5. Powell JJ, Wojnarowska F. Lichen sclerosus.
Lancet. 1999;353:1777–1783.
6. Goolamali SK, Barnes EW, Irvine WJ, et al. Organ specific antibodies in patients with lichen sclerosus.
Br Med J. 1974;4:78–79.
7. Howard A, Dean D, Cooper S, et al. Circulating basement membrane zone antibodies are found in lichen sclerosus of the vulva.
Australas J Dermatol. 2004;45:12–15.
8. Oyama N, Chan I, Neill SM, et al. Autoanti-bodies to extracellular matrix protein 1 in lichen sclerosus.
Lancet. 2003;362:118–123.
