Scleroderma with Acro-Osteolysis and Papular Mucinosis Resembling Multicentric Reticulohistiocytosis

Authors

• João Dantas Hospital Universitário Professor Edgard Santos, Salvador, Bahia, Brazil
• Isabela S de Oliveira Universidade Federal da Bahia, Faculdade de Farmácia, Salvador, Bahia, Brazil
• Emanuela P Fonseca BIS, Instituto Bahiano de Imunoterapia, Salvador, Bahia, Brazil; FTC, Faculdade de Tecnologia e Ciências, Salvador, Bahia, Brazil
• Mittermayer Barreto Santiago Hospital Universitário Professor Edgard Santos, Salvador, Bahia, Brazil; Escola Bahiana de Medicina e Saúde Pública, Brotas, Salvador, Bahia, Brazil; Serviços Especializados em Reumatologia da Bahia, Salvador, Bahia, Brazi

mbsantiago2014@gmail.com

Keywords

Abstract

Objectives: We describe a case of systemic sclerosis (SS) with acro-osteolysis associated with cutaneous mucinosis, usually characterized by mucin deposition in the skin. The main differential diagnosis was multicentric reticulohistiocytosis due to the presentation of papulonodular skin lesions.
Materials and methods: A physical examination, imaging studies and laboratory tests were performed.
Results: Distal bone resorption was evident on plain radiographs, and skin biopsy confirmed mucinosis. The SS diagnosis was based on the clinical features, high levels of antinucleolar antibodies and typical nailfold capillaroscopy findings.
Conclusion: To the best of our knowledge, this is the first description of cutaneous mucinosis accompanying SS with acro-osteolysis.