Vasculitis and High Disease Activity in Penile Lichen Sclerosus and Planus Are Associated with Concomitant Systemic Autoimmune Disease
Y Razmara, K Pummer, S Mannweiler, S Regauer. Medical University, Graz, Austria
Background: Lichen sclerosus (LS) and lichen planus (LP) are autoimmune disorders without disease-specific antinuclear antibodies (ANA), although LS and LP patients commonly suffer from systemic autoimmune diseases with well defined organ specific ANA. In men, anogenital LS and LP involve mainly the foreskin. Circumcision is often the definitive treatment. Work-up of LS and LP patients in our institution includes routinely an immunological screening for systemic ANA (parietal cells, thyroidal peroxidase, thyreoglobulin, ds-DNA, RnP-70 and Ro) as recommended in „guidelines for the management of LS“ (S. Neill, Br J Dermatol, 2002,147:640-9).
Design: 42 men with a histological diagnosis of LS (31) and LP (11) in their circumcision specimen had a serological screen for ANA. Increased titres to parietal cells were followed by gastroscopy, gastric biopsy and blood tests to confirm autoimmune gastritis (AIG) / pernicious anemia, and increased titres to thyroglobulin with sonographic confirmation of Hashimoto's thyroiditis. Patients were also evaluated for the presence of psoriasis, vitiligo and alopecia areata.
Results: 23/42 men with penile LP/LS (55%; 8 LP; 11 LS) had a concomitant autoimmune disease. 11 / 42 (29%) had an autoimmune disease with organ specific ANA: 7/42 men (17%) had an AIG / pernicious anemia (4/11 LP; 3 /31 LS). Hashimoto's thyroiditis was diagnosed in 2 men each. 1 LS-patient suffered from Crohn's disease. All these circumcision specimens showed high disease activity; 5/8 pts with AIG and the patient with Crohn's disease showed additionally a prominent lymphocytic vasculitis involving large muscular blood vessels. T-cell mediated autoimmune diseases included psoriasis in 7/42 men (16%; 2/11 LP; 5/31 LS) and vitiligo in 4 LS patients. None of these patients had vasculitis in the circumcision specimens. 1 LS-patient had multiple autoimmune diseases (AIG, psoriasis, thyroiditis); all other men had only one other autoimmune disease in addition to the LP and LS.
Conclusions: Men with penile LS and LP have a high prevalence of systemic autoimmune diseases. T-cell mediated autoimmune diseases were more common in LS patients, while AIG was more common in LP patients. High disease activity and a lymphocytic vasculitis in foreskin specimens affected by LP and LS should raise the suspicion for additional systemic autoimmune diseases. Pathologists should include these parameters into their reports together with a suggestion to evaluate the patient for concomitant AID.
Category: Genitourinary (including renal tumors)
Monday, March 22, 2010 1:00 PM
Poster Session II # 93, Monday Afternoon