Camille Villon1, Laure Orgeolet1, Anne-Marie Roguedas1, Laurent Misery1, Jacques-Eric Gottenberg2, Divi Cornec3, Sandrine Jousse-Joulin3, Raphaele Seror4, Jean-Marie Berthelot5, Philippe Dieude6, Jean-Jacques Dubost7, Anne-Laure Fauchais8, Vincent Goeb9, Eric Hachulla10, Pierre-Yves Hatron10, Claire Larroche11, Gilles Hayem12, Véronique Le Guern13, Aleth Perdriger14, Jacques Morel15, Olivier Vittecoq16, Xavier Mariette4, Valérie Devauchelle-Pensec3, Alain Saraux17. 1. Dermatologie, CHU de Brest, 29200 Brest, France. 2. Rhumatologie, CHU de Strasbourg, hôpitaux universitaires Strasbourg, 67000 Strasbourg, France. 3. Inserm 1227, LabEx IGO, rhumatologie, centre de référence maladies rares CERAINO, université de Bretagne Occidentale, CHU de Brest, 29200 Brest, France. 4. Rhumatologie, université Paris Sud XI, AP-HP, 94270 Le Kremlin-Bicêtre, France. 5. Rhumatologie, CHU Hôtel-Dieu, 44093 Nantes, France. 6. Rhumatologie, hôpital Bichat-Claude Bernard, 75018 Paris, France. 7. Rhumatologie, CHU Gabriel-Montpied, 63003 Clermont-Ferrand, France. 8. Médecine interne, CHU de Limoges Dupuytren, 87042 Limoges, France. 9. Rhumatologie, CHU Amiens-Picardie Site Nord, 80054 Amiens, France. 10. Service de médecine interne et immunologie clinique, centre de référence des maladies rares autoimmunes rares (CeRAINO), université de Lille, CHU, 59100 Lille, France. 11. Médecine interne H5, hôpital Avicenne, CHU, 93000 Bobigny, France. 12. Service de rhumatologie, CHU Ambroise Paré, 92100 Boulogne-Billancourt, France. 13. Médecine interne, hôpital Cochin, 75679 Paris, France. 14. Rhumatologie, hôpital Sud, 35200 Rennes, France. 15. Immuno-rhumatologie, CHU Lapeyronie, 34000 Montpellier, France. 16. Rhumatologie, CHU Rouen, 76000 Bois-Guillaume, France. 17. Inserm 1227, LabEx IGO, rhumatologie, centre de référence maladies rares CERAINO, université de Bretagne Occidentale, CHU de Brest, 29200 Brest, France. Electronic address: alain.saraux@chu-brest.fr.
Abstract
OBJECTIVE: To determine the prevalence and significance of dermatological disorders in primary Sjögren syndrome (pSS). METHODS: We used 2 pSS French cohorts (ASSESS, in which prevalence of skin disorders in 395 patients was evaluated; and diapSS, in which 76 on 139 pSS patients had an examination by a dermatologist) and baseline data of the TEARS randomized trial (110 patients with recent or active pSS treated with rituximab or placebo and evaluated for skin dryness using a visual analogue scale (VAS) out of 100). RESULTS: Skin manifestations included in the EULAR Sjögren syndrome disease activity index (ESSDAI) were rare in the ASSESS cohort (n=16/395, 4.1%, mainly purpuras; only 3 had high activity), but they were associated with activity in the other ESSDAI domains (peripheral neurological (P<0.001), muscular (P<0.01), haematological (P<0.05), biological (P<0.05), history of arthritis (P<0.01), splenomegaly (P<0.05) and higher gamma globulin levels (P<0.01)). In the diapSS cohort, compared to pSS patients not receiving a dermatological consultation, the pSS patients who had a dermatological consultation had significantly more dermatological involvement outside the ESSDAI score [38.2% (29/76) versus 15.9% (10/63); P<0.01]. The TEARS study showed a high prevalence of cutaneous dryness (VAS>50; 48.2%) and found that patients with dry skin had higher VAS pain (P<0.01) and drought (P<0.01) scores. CONCLUSION: ESSDAI skin activity is rare and associated with hypergammaglobulinemia and ESSDAI activity. Systematic dermatological examination is informative for non-specific lesions. The most common skin disorder is skin dryness, which is associated with a higher pain and overall subjective dryness.
OBJECTIVE: To determine the prevalence and significance of dermatological disorders in primary Sjögren syndrome (pSS). METHODS: We used 2 pSS French cohorts (ASSESS, in which prevalence of skin disorders in 395 patients was evaluated; and diapSS, in which 76 on 139 pSS patients had an examination by a dermatologist) and baseline data of the TEARS randomized trial (110 patients with recent or active pSS treated with rituximab or placebo and evaluated for skin dryness using a visual analogue scale (VAS) out of 100). RESULTS: Skin manifestations included in the EULAR Sjögren syndrome disease activity index (ESSDAI) were rare in the ASSESS cohort (n=16/395, 4.1%, mainly purpuras; only 3 had high activity), but they were associated with activity in the other ESSDAI domains (peripheral neurological (P<0.001), muscular (P<0.01), haematological (P<0.05), biological (P<0.05), history of arthritis (P<0.01), splenomegaly (P<0.05) and higher gamma globulin levels (P<0.01)). In the diapSS cohort, compared to pSS patients not receiving a dermatological consultation, the pSS patients who had a dermatological consultation had significantly more dermatological involvement outside the ESSDAI score [38.2% (29/76) versus 15.9% (10/63); P<0.01]. The TEARS study showed a high prevalence of cutaneous dryness (VAS>50; 48.2%) and found that patients with dry skin had higher VAS pain (P<0.01) and drought (P<0.01) scores. CONCLUSION: ESSDAI skin activity is rare and associated with hypergammaglobulinemia and ESSDAI activity. Systematic dermatological examination is informative for non-specific lesions. The most common skin disorder is skin dryness, which is associated with a higher pain and overall subjective dryness.