Diagnostic delay in sternocostoclavicular hyperostosis: impact on various aspects of quality of life.
OBJECTIVE
Sternocostoclavicular hyperostosis (SCCH) is a rare, debilitating, chronic inflammatory disorder of the anterior chest wall due to a chronic sterile osteomyelitis of unknown origin, often associated with characteristic skin lesions of palms and soles: pustulosis palmoplantaris. SCCH goes often unrecognized for years before the diagnosis is established and treatment instituted. The objective of this study was to trace the diagnostic paths of patients with SCCH and to investigate the consequences associated with diagnostic delay.


METHODS
Data were collected through structured interviews of 52 patients with a clinically, scintigraphically, and radiologically established diagnosis of SCCH.


RESULTS
The majority of patients presented with swelling and/or pain in the sternocostoclavicular region and/or limited movement of the shoulder girdle. Pustulosis palmoplantaris was present in approximately 30% of patients. The disease went unrecognized for a median of 3.5 years. Patients were often seen by at least 3 members of the medical profession before the diagnosis was suspected and eventually established. Lack of recognition of the clinical manifestations of the disease and delay in diagnosis were associated with important physical, psychological, and socioeconomic consequences affecting quality of life.


CONCLUSIONS
SCCH remains an ill-recognized disease despite its characteristic clinical features. A low level of awareness of the disorder leads to a delay in diagnosis, which has a significant impact on various aspects of quality of life. Awareness should be raised for this disorder, enabling timely diagnosis and initiation of treatment to prevent the irreversible physical and psychological sequelae associated with the protracted untreated state.
van der Kloot
Willem A
WA
Leiden University, Leiden, The Netherlands. w.a.van.der.kloot@umail.leidenuniv.nl
Chotkan
Sadhna A
SA
Kaptein
Ad A
AA
Hamdy
Neveen A T
NA
D000328:Adult; D000368:Aged; D057210:Delayed Diagnosis / Q000191:economics; D005260:Female; D006801:Humans; D015218:Hyperostosis, Sternocostoclavicular / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology* / Q000523:psychology / Q000628:therapy; D007407:Interviews as Topic; D008297:Male; D008511:Medicine; D008875:Middle Aged; D010146:Pain / Q000503:physiopathology; D026741:Physical Therapy Modalities; D010821:Physicians, Family; D011565:Psoriasis / Q000150:complications; D011788:Quality of Life; D011877:Radionuclide Imaging; D012959:Socioeconomic Factors; D014057:Tomography, X-Ray Computed; D055815:Young Adult