winter 2018 AMHE Newsletter december 24 Haitian Medical Association Abroad Association Medicale Haïtienne à l'Étranger Newsletter # 245 AMHE NEWSLETTER Editor in Chief: Maxime J-M Coles, MD Editorial Board: Rony Jean Mary, MD Reynald Altema, MD Technical Adviser: Jacques Arpin Chers lecteurs et lectrices de l’infolettre de la AMHE, In this number 2 - Words of the Editor, Maxime Coles,MD - Poème de Marlène Apollon - SAPHO Syndrome, Maxime Coles,MD - Poème de Dr Jean Serge Dorismond - Henry R Ford MD, MHA recently became Dean and Chief… - Dr. Brutus Maurice Dieudonne - La chronique de Rony Jean-Mary,M.D. - Sickle cell Clinic in Leogane - La chronique de Reynald Altéma,M.D. - Upcoming Events - AHDH’s Mission Report - Published on the AMHE Facebook page last two weeks - Outcomes of minority patients with very severe hypertension - And more... SAPHO Syndrome Maxime Coles MD SAPHO Syndrome (Acquired Hyperostosis 1987 by Chamot to describe this spectrum of Syndrome, Pustulosis, Hyperostosis and inflammatory bone disorder, often but not Osteomyelitis, Synovitis acne pustulosis always associated with dermatologic lesions. hyperostosis osteitis) is described as any Symptoms will vary from person to person. combination of Synovitis (inflammation of the People diagnosed with the disease may have joints), with acne, Pustulosis (thick yellow blisters different symptoms based on the human containing pus) often in the palms and soles, phenotype ontology (HPO). The information Hyperostosis (increase in bone substance) and collected is updated regularly with bone pain, Osteitis (inflammation of the bone). The cause of arthralgia, chest pain, cranio-fascial Sapho syndrome is unknown, but not fully osteosclerosis, Enthesitis (SC Joints). understood and the treatment is focused on 60-90% of patients suffering from this condition managing symptoms. will present with anterior chest wall This is a variety of inflammatory bone disorder hyperostosis, sclerosis or bone hypertrophy associated with skin changes coupled to various involving especially the sternoclavicular joint. clinical, radiologic and pathologic conditions. In We all know well the predilection of the SC 1972, orthopedists considered this entity as a joint to tuberculosis and syphilis in our country Chronic Recurrent Multifocal Osteomyelitis. Later of Haiti. The spine is commonly affected in 32- in 1978 It was noted a closed association with 50% of cases in which most of the lesions are in blisters in the palms and soles named palmoplantar the thoracic spine demonstrating pustulosis, existed. Since, numerous skin conditions spondylodiscitis, osteosclerosis, paravertebral with osteoarticular disorders were reported bringing ossifications and sacroilliac joint involvement. a variation in the name of the pathology as 30% of patients will have long bone sternoclavicular Hyperostosis, pustulotic arthro- involvement at the metaphyseal-diaphyseal osteitis and acne associated spondyloarthropathy. junctions, around the knee joint: distal femur The term SAPHO Syndrome (Synovitis, Acne, and proximal tibia. pustulosis, Hyperostosis osteitis) was coined in These lesions tend to mimic osteomyelitis 3 without the acute or chronic signs of abcess formation, suggesting of enthesis. 30% of patients with the involucrum and further sequestrum formation. 10% of syndrome can rule out osteomyelitis or discitis. the patients suffering of the condition will also have SAPHO Syndrome cannot be diagnosed flat bone involvement like the ilium or the mandibula. hystopathologically. Biopsy can only rule out If the disease is seen in children, the metaphysis of the the possibility of infection. As we have already long bone is generally involved in the process, stated, the pathogenesis is not completely followed by the spine and the clavicle. The aseptic understood but appears to have genetic, skeletal inflammatory manifestation often mimic immunologic and infectious components. Some osteomyelitis, lymphomas, Ewing sarcoma and even studies have demonstrated a relation to can give the appearance of metastasis to bone. Ankylosing Spondylitis with the Human SAPHO Syndrome occurs also in other Lymphocyte Antigen (HLA-B27). Infectious spondyloarthropathies like psoriatic arthritis, origins have been postulated because of isolation idiopathic ankylosing spondyloarthropathy, of Propionibacterium acnes in some skin lesions. spondylopathy associated with inflammatory bowel There is no specific treatment to this SAPHO syndrome. Psoriatic arthritis with axial skeletal syndrome. It can be chronic or heal suddenly. involvement and pustular arthritis can be like SAPHO Joint pain may be managed with anti- but radiologic signs of osteitis with hyperostosis are inflammatory drugs (NSAIDS) and not seen in psoriatic arthritis. Generally psoriatic Bisphosphonates as a first line of treatment, but arthritis is asymmetric and remain associated with Vitamin A, Serotonin has been suggested for the only sacroiliitis and sparing of the facet joints while in treatment of acne as well as Corticosteroids ankylosing spondylitis the sacroilliac joints are (Psoralen) and Ultraviolet therapy with retinoids. typically involved until ankylosis is fully established. Physical therapy has been used as an adjunct to Reactive spondyloarthritis is believed to be a musculoskeletal symptoms but there are component of a systemic autoimmune response questionable evidences that such treatment is following an infection of the conjunctivae, or the beneficial. Surgical treatment of deformity to bowels or the urethral-genital system, two to four relieve pain and discomfort may have also a role weeks prior but involving larger joints in progressively destructive spondylitis requiring asymmetrically. Enteropathic spondyloarthritis is stabilization. generally associated to two major chronic We have seen patients undergoing serologic inflammatory diseases: Ulcerative Colitis and Chrohn testing with subsequent antibiotic therapy. Many disease which also have radiologic spondylitis may have had prior biopsies before a diagnosis sacroilliac joint involvement like Ankylosing Arthritis. is made. The skin manifestations like Acne We have already discussed that the most common site Fulminans, Pustular Psoriasis, Palmoplantar of skeletal involvement in SAPHO Syndrome is the Pustulosis usually occur simultaneously with the anterior chest followed by the spine with bone lesions. Typically, a patient will have 2 or osteosclerosis and hyperostosis. It becomes logical to 3 months of progressive mi-thoracic pain use imaging studies to differentiate them. exacerbated by exercises but no history of fever, Technetium 99m bone scanning demonstrates an no swollen joints, often no other signs of increase tracer uptake for the active and chronic infection. lesions. Pet scanning maybe used to differentiate acute Laboratories tests will reveal a normal white from chronic healed inflammatory lesions mandating a blood cell count as well as a normal C-reactive biopsy to rule out any possible metastatic lesions. protein level. CT Scan or MRI of the thoracic MRI imaging will demonstrate bone marrow edema in spine may show sclerosis or edema. Lymphoma any active lesion. Recently a curvilinear or can be suspected but Bone biopsies of the semicircular pattern was described in the vertebral thoracic spine remained in the normal limit body segments helping in differentiating SAPHO ruling out that diagnosis. Core biopsy do show Syndrome from Metastatic disease of the spine. chronic inflammatory process at the thoracic Another finding is an anterior vertebral corner erosion vertebrae level. Bacterial cultures are often 4 inconclusive or negative for fungi or bacteriae. Unfortunately, treatment remains ill-defined but Serologic test are also generally negative for Bisphosphonates and NSAIDs have been suggested Human Leucocyte Antigen (HLA) B27. as the first-choice drugs in the treatment of this Neoplasm and Infection can be, at once condition, controlling symptoms. Other have tried eliminated. Tumor Necrosis Factor alpha antagonist (TNF One may conclude that the SAPHO syndrome is inhibitors), Topical Corticosteroids, Colchicine, a diagnosis of exclusion. Often when MRI Ciclosporin, Sulfonamide, Calcitonin, Methotrexate, studies are often repeated, demonstrating no Leflunomide, Infliximab, Etanercept and have changes to the previous lesions reported or no shown some effects in reducing symptoms. Finally, resolution to the inflammatory process year after. Antibiotics to treat Propionibacterium infection have I tried to present the evidences that diagnosing also been used in the treatment of the SAPHO the SAPHO Syndrome can be challenging and syndrome, once biopsies or cultures have proven tricky when dealing with clinical, radiologic and this pathogen to be responsible of the infection. serologic studies but at least one of the Always keep in mind that a Differential diagnosis osteoarticular manifestations added to between Osteomyelitis or Arthritis, Langerhans cell dermatologic lesions like Acne or Palmoplantar histiocytosis and/or Bone Tumors such as Ewing pustules with or without chronic recurrent sarcoma, Osteoblastoma and Osteoid Osteoma need osteomyelitis or any other dermatosis are enough always to alert the clinician. to assure the diagnosis. In conclusion, SAPHO Syndrome is indeed, a Maxime Coles MD diagnosis of exclusion even if cutaneous manifestations are not present. It is important to differentiate it from other conditions. References: 1- Bjorksten B, Gustavson KH, Enksson B et al. (1978).