Sjögren's syndrome: a retrospective review of the cutaneous features of 93 patients by the Italian Group of Immunodermatology

OBJECTIVE: Various cutaneous manifestations have been described (xerosis, eyelid dermatitis, angular cheilitis, cutaneous vasculitis and annular erythema) in Sjögren's syndrome (SS), but so far only three studies on large numbers of SS patients have been carried out. The frequency of cutaneous manifestations and their association with specific clinical or immunological parameters have never been studied. The aim of the Italian Group of Immunodermatology was to evaluate these associations in a large number of SS patients. METHODS: A retrospective review was performed on 93 patients with SS followed over a ten-year period (1990-2000) at 6 Italian dermatological centers. They were subdivided into two groups, 62 with primary SS and 31 with secondary SS, and the frequency of cutaneous manifestations and specific antibodies was determined and compared between them. RESULTS: We found significantly higher levels of xerosis (p = 0.009) (56.4% versus 25.8%) and angular cheilitis (p = 0.017) (38.7% versus 16.1%) in primary SS patients than in those with secondary SS. A significant association of xerosis with anti-SSA + SSB (p = 0.033) antibodies was also demonstrated. Eyelid dermatitis and pruritus were common but less specific cutaneous symptoms. Annular erythema was found more often in primary (6.45%) than in secondary (3.2%) SS and was associated with SSA + SSB antibodies in 75% of the cases. Cutaneous vasculitis was present in 30.6% of primary SS (manifesting as palpable purpura in 84%) and in 29.3% of secondary SS cases. CONCLUSION: Xerosis is the most frequent and characteristic cutaneous manifestation of primary SS. It is not linked to decreased sebaceous or sweat gland secretion, but more probably to a specific alteration of the protective function of the stratum corneum. Angular cheilitis is a common but less specific skin lesion in SS and is associated with xerosis and xerostomia.