title: Incidentally diagnosed melorheostosis of upper limb: case report.
abstract: BACKGROUND
  Melorheostosis is quite a rare bone disease with still unclear ethiology. Although multifocal affection is highly debilitating with unfavorable prognosis, there is no clear consensus about therapeutical approach. There is still insufficient evidence in the literature for almost a century after the first description. Affected bone has a typical appearance of melting wax. Diagnosis is usually incidental with pain as a leading symptom. Diagnosis itself is relatively easy, routine X-ray examination is sufficient. Even though it could be easily overlooked and mistaken with other diseases. Melorheostosis is incurable, the therapy is mostly focused on maintaining patient quality of life. Presented case is unique in terms of extent of the affection (index finger, metacarp shaft, carpal bones, forearm, humerus and whole scapula) in combination with osteopoikilotic islands in other 3 regions (vertebrae, manubrium sterni and left collar bone). Currently there is only one such a case published in the literature (Campbell), but without osteopoikilotic islands.
  
  
  METHODS
  Melorheostosis was diagnosed in 26-year old female after injury as an incidental finding. This was quite surprising as the patient already suffered by limited movement in the upper limb and pain before the injury. Detailed examination were performed to confirm the diagnosis, no family history was found. Pharmacotherapy with bisphosphonates, non-steroidal antirheumatics and vasodilatans/rheologic drugs seemed to be effective to maintain the relatively good quality of patient life and good performance in daily routine. Questionable is further development of patient performance status and sustainability of conservative treatment in the long term follow up.
  
  
  CONCLUSIONS
  Conservative treatment with bisphopshonates and COX-2 inhibitors in combination with naftidrofuryl can delay surgery solution.
authors: Department of Orthopaedic Surgery, Faculty Hospital Plzen, Alej Svobody 80, Plzen, 304 60, Czech Republic. vyskocil@fnplzen.cz.
authors: Vaclav
authors: V
authors: Vyskocil
authors: Department of Orthopaedic Surgery, Faculty Hospital Plzen, Alej Svobody 80, Plzen, 304 60, Czech Republic. koudelak@fnplzen.cz.
authors: Karel
authors: K
authors: Koudela
authors: Department of Orthopaedic Surgery, Faculty Hospital Plzen, Alej Svobody 80, Plzen, 304 60, Czech Republic. pavelka@fnplzen.cz.
authors: Tomas
authors: T
authors: Pavelka
authors: Department of Imaging Methods, Charles University Teaching Hospital Plzen and Medical Faculty Plzen, Alej Svobody 80, Plzen, 304 60, Czech Republic. ohlidalova@fnplzen.cz.
authors: Kristyna
authors: K
authors: Stajdlova
authors: Department of Clinical Pharmacology, Charles University Teaching Hospital E. Benese 13, Plzen, 30599, Czech Republic. suchyd@fnplzen.cz.
authors: David
authors: D
authors: Suchy
mesh_terms: D015502:Absorptiometry, Photon; D000328:Adult; D002574:Cervical Vertebrae / Q000000981:diagnostic imaging; D052246:Cyclooxygenase 2 Inhibitors / Q000627:therapeutic use; D004164:Diphosphonates / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D005542:Forearm / Q000000981:diagnostic imaging; D006225:Hand / Q000000981:diagnostic imaging; D006801:Humans; D008557:Melorheostosis / Q000175:diagnosis* / Q000188:drug therapy; D009257:Nafronyl / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D034941:Upper Extremity; D051598:Whole Body Imaging
chemical_list: D052246:Cyclooxygenase 2 Inhibitors; D004164:Diphosphonates; D009257:Nafronyl
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