Vaianu Leroy1, Pauline Henrot2, Thomas Barnetche3, Muriel Cario4, Anne-Sophie Darrigade1, Pauline Manicki3, Marie-Sylvie Doutre1, Estibaliz Lazaro5, Joel Constans6, Damien Barcat7, Jean-Philippe Vernhes8, Christophe Richez3, Alain Taieb9, Marie-Elise Truchetet10, Julien Seneschal11. 1. Department of Dermatology, National Reference Center for Rare Skin Disorders, Hôpital Saint-André, CHU de Bordeaux, Bordeaux, France. 2. Department of Rheumatology, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France; Inserm U1035, Biothérapie des Maladies Génétiques et Cancers, University of Bordeaux, Bordeaux, France. 3. Department of Rheumatology, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France. 4. Inserm U1035, Biothérapie des Maladies Génétiques et Cancers, University of Bordeaux, Bordeaux, France. 5. Department of Internal Medicine, Hôpital Haut-Levêque, CHU de Bordeaux, Bordeaux, France. 6. Department of Vascular Medicine, Hôpital Saint-André, CHU de Bordeaux, Bordeaux, France. 7. Department of Internal Medicine, Hôpital Robert Boulin, Libourne, France. 8. Department of Rheumatology, Hôpital Robert Boulin, Libourne. 9. Department of Dermatology, National Reference Center for Rare Skin Disorders, Hôpital Saint-André, CHU de Bordeaux, Bordeaux, France; Inserm U1035, Biothérapie des Maladies Génétiques et Cancers, University of Bordeaux, Bordeaux, France. 10. Department of Rheumatology, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France; Immunoconcept, CNRS UMR 5164, Bordeaux University, Bordeaux, France. 11. Department of Dermatology, National Reference Center for Rare Skin Disorders, Hôpital Saint-André, CHU de Bordeaux, Bordeaux, France; Inserm U1035, Biothérapie des Maladies Génétiques et Cancers, University of Bordeaux, Bordeaux, France. Electronic address: julien.seneschal@chu-bordeaux.fr.
Abstract
BACKGROUND: Skin pigmentation disorders in systemic sclerosis (SSc) have been sparsely described in the literature. Nevertheless, they could be a diagnostic and/or severity marker. OBJECTIVES: To assess the association between pigmentation disorders and systemic involvement in patients with SSc. METHODS: A total of 5 patterns of skin pigmentation disorders were defined: diffuse hyperpigmentation; hyperpigmentation of sun-exposed areas; hypopigmentation of the head, neck, and/or upper part of the chest; acral hypopigmentation; and diffuse hypopigmentation. RESULTS: A total of 239 patients were included; 88 patients (36.8%) had skin pigmentation disorders as follows: diffuse hyperpigmentation and hyperpigmentation of sun-exposed areas in 38.6% (n = 34) and 27.3% (n = 24) of patients, respectively; hypopigmentation of the face, neck, and/or chest in 10.2% of patients (n = 9); diffuse hypopigmentation in 12.5% (n = 11); and acral hypopigmentation in 17% (n = 15). Diffuse hyperpigmentation was associated with diffuse SSc (P = .001), increased modified Rodnan skin score (P = .001), and shorter duration of Raynaud phenomenon (P = .002) in univariate analysis but not in multivariate analysis. Moreover, diffuse hyperpigmentation was associated with digital ulcers (P = .005), as confirmed by multivariate analysis (odds ratio, 2.96; 95% confidence interval, 1.28-6.89). LIMITATIONS: This was a single-center retrospective study of a cohort of patients with SSc. CONCLUSION: Screening for skin pigmentation disorders could be useful in the management of patients with SSc to identify those with a high risk of development of digital ulcers, which is a symptom of vascular involvement in SSc.
BACKGROUND: Skin pigmentation disorders in systemic sclerosis (SSc) have been sparsely described in the literature. Nevertheless, they could be a diagnostic and/or severity marker. OBJECTIVES: To assess the association between pigmentation disorders and systemic involvement in patients with SSc. METHODS: A total of 5 patterns of skin pigmentation disorders were defined: diffuse hyperpigmentation; hyperpigmentation of sun-exposed areas; hypopigmentation of the head, neck, and/or upper part of the chest; acral hypopigmentation; and diffuse hypopigmentation. RESULTS: A total of 239 patients were included; 88 patients (36.8%) had skin pigmentation disorders as follows: diffuse hyperpigmentation and hyperpigmentation of sun-exposed areas in 38.6% (n = 34) and 27.3% (n = 24) of patients, respectively; hypopigmentation of the face, neck, and/or chest in 10.2% of patients (n = 9); diffuse hypopigmentation in 12.5% (n = 11); and acral hypopigmentation in 17% (n = 15). Diffuse hyperpigmentation was associated with diffuse SSc (P = .001), increased modified Rodnan skin score (P = .001), and shorter duration of Raynaud phenomenon (P = .002) in univariate analysis but not in multivariate analysis. Moreover, diffuse hyperpigmentation was associated with digital ulcers (P = .005), as confirmed by multivariate analysis (odds ratio, 2.96; 95% confidence interval, 1.28-6.89). LIMITATIONS: This was a single-center retrospective study of a cohort of patients with SSc. CONCLUSION: Screening for skin pigmentation disorders could be useful in the management of patients with SSc to identify those with a high risk of development of digital ulcers, which is a symptom of vascular involvement in SSc.