Perniosis is an inflammatory disease process commonly occurring in patients living in cold, damp geographical regions. It typically consists of erythematous papules involving the fingers, toes, nose, or ears. In general, most cases resolve within a few days and are not related to SLE. However, some lesions persist and demonstrate blister or ulcer formation. French investigators have determined that one third of the patients with chilblain lupus having this disease process for greater than 1 month have evidence of LE (Viguier et al. 2001). Another one third are patients with atypical chilblains characterized by persistent perniosis lesions who possess one to three of the preliminary criteria of the American College of Rheumatology (ACR) for the diagnosis of SLE. Follow-up of the latter patients indicated that 2 of 10 (20%) developed SLE.
Those patients with chilblain LE demonstrated that 50% of the time the chilblain lesions persisted during spring and summer. In addition, chronic discoid lupus lesions were detected in approximately 60% of these patients, and 50% had arthralgias. Raynaud's phenomenon was detected in approximately one third. A comparison of the histopathologic features of chilblain LE and idiopathic chilblains is given in Table 7.4 (Cribier et al. 2001,Viguier et al. 2001).
Two subsequent studies have reported an association of chilblain lupus with the presence of anti-Ro/SSA antibodies (Aoki et al. 1996, Franceschini et al. 1999). These preliminary studies plus our own observations indicate that perniosis associated with LE is an unusual cutaneous finding, occurring in patients with SLE living in geographical areas with cold, damp winters (e.g., Great Britain and other European countries). For example, during a recent winter in Baltimore, Maryland, characterized by dampness and nonfreezing temperatures, the author personally saw two anti-Ro/SSA antibody-positive patients develop chilblain lupus. One had Sjogren's/LE overlap syndrome and the other had anti-Ro/SSA antibody-positive SLE.
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