Miescher syndrome receives poor attention and is often misdiagnosed

Introduction:

Miescher syndrome, cheilitis granulomatosa (CG) is an inflammatory disorder of unknown aetiology with possble genetic predispositi. It appears in second decade of life and regresses with age. Although it may resolve spontainously, after several reccurences, it may become chronic, resulting granulomas. It is characterised by swellig of lips and sof oropharyngeal tissues and may be misdiagnosed as allergic or hereditary angiooedema.

Material and Methods:

In this study we have examined seven patients, all previously treated for reccuring allergic angiooedema non responsive to steroids or antihistamines or as chronic forms of macrochelia. Only after appropriate diagnostics to exclude other etiologies of allergic or granulomatous disease and patohistological analysis correct diagnosis was established.

Results:

Patients did not agree on surgical procedure. Treatment with intralesional administration of triamcinolone was conducted with varying results.

Conclusion:

CG responds well to steroid treatment and need for surgery is minimal and should be reserved for most recalcitrant cases.

Publikationsverlauf

Publikationsdatum:
18. April 2018 (online)