Background Primary Sjögren's syndrome (pSS) is a systemic disease marked by the dysfunction and destruction of exocrineglands due to lymphocytic infiltration. pSS patients may develop a large number of systemic manifestations. Mucocutaneousmanifestations are one of the most typical extraglandular features of pSS. A wide spectrum cutaneous lesions, includingleucocytoclastic vasculitis, mononuclear vasculitis, urticarial vasculitis, alopecia, non-specific photosensitive cutaneous lesions,vitiligo, cutaneous amyloidosis, annular granuloma, erythema multiforme have been reported in pSS patients.
Objectives The aim of this work is to study the demographic, clinical, serologic features and outcomes of Tunisian patients withpSS and mucocutaneaous involvement.
Methods It is a single-center retrospective study including all the cases of pSS diagnosed since 1998 until 2011 in InternalMedicine Department from Tunis in Tunisia. All the patients fulfilled the 2002 American-European Consensus Group (AECG)criteria for pSS
Results The study include 31 patients: 4 men and 27 women. The age average was 51 years. Dryness of the mouth and eyes wasobserved in all the cases. Among them, 10 patients presented mucocutaneous manifestations: purpura in 7 cases, erythema multiformein 2 cases associated with livedo in one of them and one case of severe skin dryness.In the cases of the purpura, the skin biopsy have revealed leucocytoclastic vasculitis. There was no other disease associated with pSS.One patient with leucocytoclastic vasculitis have developed sensory axonal neuropathy. In the patient with erythema multiforme andlivedo, the mucosa-associated lymphoid tissue (MALT) lymphoma of the parotids was diagnosed.A diffuse dry itchy skin especially in the lower limbs was observed in a patient. The skin biopsy showed the presence of a lymphochronic inflammatory infiltrate organized in nodules around the excretory canals of the sweat glands of the skin. A hypohidrosis hasbeen diagnosed.
Conclusions During the pSS, the most common clinical manifestation is mouth and eyes dryness. But extraglandularmanifestations are frequent. Skin involvement is one of the most typical systemic features of pSS often underestimated.
Disclosure of Interest None declared
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