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Giornale Italiano di Dermatologia e Venereologia 2004 October;139(5):415-27


language: English, Italian

Cutaneous manifestations of Sjögren syndrome

Fabbri P.

Divison of Dermatology II Department of Dermatological Sciences University of Florence, Florence, Italy


The Sjögren syndrome (SS) is a chronic inflammatory disease of the exocrine glands, especially of the salivary and lacrimal glands, clinically characterized by xerostomia, xerophthalmia and frequently arthritis. SS is classified in primary and secondary when associated with other connective tissue diseases. Different sets of diagnostic criteria employing combination of various parameters have been proposed by different groups. The most used are the Fox’s classification and the Diagnostic Criteria for Sjögren Syndrome of the European Study Group. Various cutaneous manifestations have been described in primary SS: some appear very often, others are less frequent or are considered casual associations. In studies performed in large number of patients, cutaneous xerosis, diffuse to large part of the cutaneous surface, is the most frequent manifestation (23-67%). In a recent study of the Italian Group of Immunopathology, xerosis was documented in 43% of cases. Some researches of our group demonstrated that xerosis occurring in primary SS is not related to a reduction neither quantitative nor functional of the sebaceous and sweat glands or to the periadnexial lymphocytic infiltration, but it is related to biological alterations and functional characteristics of the corneum layer. Eyelid dermatitis (ED) was observed in 21-41% of primary SS patients. ED is symmetrical and often interests only the superior eyelid. Even if many authors have demonstrated contact sensibilization to numerous haptens, ED must be considered an inflammatory reaction to rubbing. Angular cheilitis significantly associated to xerostomia was observed in 37% of primary SS patients studied by our group, while annular erythema (multiple elevated lesions of small dimensions with an incomplete central resolution) in 6.5%. Finally, one of the most common (30-36%) manifestations of primary SS is the appearance of a cutaneous vasculitis interesting the postcapillary venules and clinically represented by palpable purpura or by urticarial vasculitis. Hystopathologically the cutaneous vasculitis showed the typical findings of the leukocytoclastic vasculitis (55%) of a lymphocytic vasculitis (40%) or of a mixed condition (with an infiltrate composed equally by neutrophilic granulocytes and lymphocytes). In the patients with leukocytoclastic vasculitis with low levels of complement activity there was a significant incidence of neurological complications and often of pulmonary and renal involvement following the appearance of cutaneous lesions. In conclusion, many cutaneous manifestations can be observed in primary SS, but none can be considered as pathognomic.

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