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SAPHO syndrome with acne fulminans and severe polyosteitis involving axial skeleton

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis), a rare inflammatory disorder, is an association of distinct skin disorders with pustules with osteoarticular inflammation. Its etiology remains unclear, and various treatment regimens frequently fail to control the disease. An 18-...

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Detalles Bibliográficos
Autores principales: Divya, B Lakshmi, Rao, P Narasimha
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5038107/
https://www.ncbi.nlm.nih.gov/pubmed/27730042
http://dx.doi.org/10.4103/2229-5178.190495
Descripción
Sumario:SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis), a rare inflammatory disorder, is an association of distinct skin disorders with pustules with osteoarticular inflammation. Its etiology remains unclear, and various treatment regimens frequently fail to control the disease. An 18-year-old male patient presented to the outpatient department with severe nodulocystic acne on the face with pain at both the wrists and lower back associated with high-grade fever and chills. On physical examination, he had severe tenderness at both wrist joints and lower back, along with swelling of right wrist. Magnetic resonance imaging revealed osteitis of the distal end of the right radius. Technetium-99m-MDP Whole Body Bone Scan revealed increased metaphyseal uptake in distal radius on both sides and prominent uptake at the sacroiliac joints, vertebral end plate, left 7(th) costo-vertebral joint and bilateral sternoclavicular joints and manubrium sternum (resulting in “bull's head” sign, which is characteristic of SAPHO syndrome). He responded very well to a combination therapy of nonsteroid anti-inflammatory drugs, antibiotics, colchicine, and isotretinoin over a 12-week period.