Vol 39 # 2 June 2007 - Kma.org.kw

176SAPHO: an Unusual Cause of Pulmonary Nodules June 2007• Palmoplantar pustulosis with osteoarticularmanifestations• Axial or appendicular hyperostosis with orwithout a sterile pustular dermatosis• C h ronic re c u r rent multifocal osteomyelitisinvolving the axial or appendicular skeleton withor without a pustular dermatosisSAPHO has been described mainly in childrenbut also young to middle-aged adults. Most of thepublished cases have been reported from Japan orNorthern and Western Europe. Discrepancies in thereported prevalence may relate to under re c o g n i t i o nor under-reporting of the condition although it hasbeen suggested that ethnic immunogenetic variationmay account for some of the geographicalheterogeneity in prevalence [3] . The cause of SAPHOremains unknown. An infectious aetiology has beensuggested and Propiono-bacterium acnes has beenisolated from the lesions [4] . P. acnes is an anaerobic,Gram positive bacillus that has been implicated ins e v e re cases of acne but it is a common skinsaprophyte and its presence does not necessarilyimply causation.The course of SAPHO is usually relapsing andremitting. Bone lesions may persist for many yearsand hyperostosis remains stable on sequentialradiological examinations. Pulmonary involvementin SAPHO has been reported rarely [5-7] although itmay be more common than previously described,as it appears to remain asymptomatic and detectionrelies on fortuitous discovery of the associatedradiological abnormalities. The major implicationof making this diagnosis is to differentiate thepulmonary lesions from other potential causes andavoid the need for unnecessary investigations ortreatment. The presence of pulmonary lesions inSAPHO does not appear to alter the benign, albeitprotracted, nature of the condition. However, theinvolvement of the lungs in this condition is stillpoorly understood and there may be a case forpulmonary assessment of all patients newlydiagnosed with SAPHO.CONCLUSIONSAPHO is an important clinical entity that maybe confused with other less benign conditionsaffecting the skin, bones and lungs of children andyoung adults. The history and pattern of skindisease is often the key to the diagnosis and earlyrecognition of this syndrome can pre v e n tunnecessary and potentially hazardous investigationsand treatment.ACKNOWLEDGEMENTSThe authors wish to thank Dr M Hughes,Consultant Pediatrician, North Devon DistrictHospital, UK for referring this case.REFERENCES1. Chamot AM, Vion B, Gerster JC. Acute Pseudosepticarthritis and palmopustular psoriasis. Clin Rheumatol1986; 5:118-123.2. Benhamou CL, Chamot AM, Khan MF. Synovitis-acne -pustulosis hyperosteomyelitis Syndrome(SAPHO). A newsyndrome among the spondyloarthropathies? Clin ExperRheumatol 1988; 6:109-112.3. Khan MF, Chamot AM : SAPHO Syndrome. Rheum DisClin of N Amer 1992; 18:225-246.4. Gerster JC, Lagier R, Livio JJ. Propionibacterium acnes inspondylitis with palmoplantar pustulosis. Ann Rheum Dis1990; 49:337-339.5. Vaile JH, Langlands DR, Prichard MG. Sapho syndrome: anew pulmonary manifestation ? J Rheumatol 1995; 22:2190-2191.6. Ravelli A, Martin A. Sapho syndrome and pulmonarydisease. J Rheumatol 1996; 23:1482-1483.7. K a rem E, Manson D, Laxer RM, et al. Pulmonary associationin a case of chronic recurrent multifocal osteomyelitis.Pediatr Pulmonol 1989; 7:55-58.