Laure Frumholtz1, Sara Laurent-Roussel2, Olivier Aumaître3, François Maurier4, Guillaume Le Guenno5, Agnes Carlotti2, Alexiane Dallot2, Jean Louis Kemeny6, Laurent Antunes7, Nicolas Froment8, Sylvie Fraitag9, Jonathan London1, Alice Berezne1, Benoît Terris2, Claire Le Jeunne10, Luc Mouthon10, Selim Aractingi11, Loïc Guillevin10, Nicolas Dupin11, Benjamin Terrier12. 1. Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France. 2. Department of Pathology, Cochin Hospital, AP-HP, Paris, France. 3. Department of Internal Medicine, Hôpital G Montpied, Centre, Hospitalier Universitaire, Clermont-Ferrand, France. 4. Department of Internal Medicine, Hôpitaux Privés, Metz, France. 5. Department of Internal Medicine, Hôpital Estaing, Centre, Hospitaliser Universitaire, Clermont Ferrand, France. 6. Department of Pathology, Centre, Hospitaliser Universitaire, Clermont Ferrand, France. 7. Department of Pathology, Thionville, France. 8. Department of Pathology, Centre Hospitalier Régional Metz, France. 9. Department of Pathology, Centre Hospitalier Universitaire, Hôpital Necker, AP-HP, Paris, France. 10. Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité, Paris, France. 11. Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité, Paris, France; Department of Dermatology, Cochin Hospital, AP-HP, Paris, France. 12. Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité, Paris, France. Electronic address: benjamin.terrier@aphp.fr.
Abstract
OBJECTIVES: Cutaneous manifestations (CM) in ANCA-associated vasculitides (AAV) are frequent, but data on clinical significance and clinical-pathological correlations are lacking. METHODS: We conducted a multicenter, retrospective study including 1553 AAV patients. Clinical, biological and pathological features have been analyzed, and tissue samples from 46 biopsies were reviewed in a blind manner. RESULTS: CM were more frequent in EGPA (53.0%) and MPA (51.9%) than in GPA (36.7%). Lesions more frequently associated with GPA were oral ulcers (4.6% vs. 2.5% in EGPA and 0.3% in MPA), while pyoderma gangrenosum and palpebral xanthoma were specific to GPA. Lesions associated with MPA were segmentary edema (19.5% vs. 12.7% in EGPA and 4.3% in GPA) and livedo (12.4% vs. 0.5% and 2.6%, respectively), whereas those associated with EGPA were urticarial lesions (11.5% vs. 1.9% in GPA and 3.5% in MPA) and nodules (12,2% vs. 8.9% in GPA and 4.7% in MPA). In GPA, CM patients had more frequent vasculitis than granulomatous phenotype, and poorer relapse-free and overall survival. Pathological analysis showed vasculitis and/or granulomatous infiltrates in 87.5% of GPA, in 61.1% of EGPA and in all MPA. Vasculitis was more frequently observed in purpura and nodules, while granulomas were differently located and organized within vessels or interstitium according to the type of lesions. CONCLUSION: Each AAV seemed to be associated with a peculiar pattern of cutaneous lesions. CM are associated with poorer prognosis in GPA. Clinical-pathological correlations showed no specific feature of each AAV, whereas granulomatous infiltrates differ according to the type of lesions.
OBJECTIVES: Cutaneous manifestations (CM) in ANCA-associated vasculitides (AAV) are frequent, but data on clinical significance and clinical-pathological correlations are lacking. METHODS: We conducted a multicenter, retrospective study including 1553 AAV patients. Clinical, biological and pathological features have been analyzed, and tissue samples from 46 biopsies were reviewed in a blind manner. RESULTS:CM were more frequent in EGPA (53.0%) and MPA (51.9%) than in GPA (36.7%). Lesions more frequently associated with GPA were oral ulcers (4.6% vs. 2.5% in EGPA and 0.3% in MPA), while pyoderma gangrenosum and palpebral xanthoma were specific to GPA. Lesions associated with MPA were segmentary edema (19.5% vs. 12.7% in EGPA and 4.3% in GPA) and livedo (12.4% vs. 0.5% and 2.6%, respectively), whereas those associated with EGPA were urticarial lesions (11.5% vs. 1.9% in GPA and 3.5% in MPA) and nodules (12,2% vs. 8.9% in GPA and 4.7% in MPA). In GPA, CMpatients had more frequent vasculitis than granulomatous phenotype, and poorer relapse-free and overall survival. Pathological analysis showed vasculitis and/or granulomatous infiltrates in 87.5% of GPA, in 61.1% of EGPA and in all MPA. Vasculitis was more frequently observed in purpura and nodules, while granulomas were differently located and organized within vessels or interstitium according to the type of lesions. CONCLUSION: Each AAV seemed to be associated with a peculiar pattern of cutaneous lesions. CM are associated with poorer prognosis in GPA. Clinical-pathological correlations showed no specific feature of each AAV, whereas granulomatous infiltrates differ according to the type of lesions.
Authors: L Frumholtz; J-D Bouaziz; M Battistella; J Hadjadj; R Chocron; D Bengoufa; H Le Buanec; L Barnabei; S Meynier; O Schwartz; L Grzelak; N Smith; B Charbit; D Duffy; N Yatim; A Calugareanu; A Philippe; C L Guerin; B Joly; V Siguret; L Jaume; H Bachelez; M Bagot; F Rieux-Laucat; S Maylin; J Legoff; C Delaugerre; N Gendron; D M Smadja; C Cassius Journal: Br J Dermatol Date: 2021-10-05 Impact factor: 11.113