Melkersson-Rosenthal syndrome is the term used when there
is cheilitis (chronic swelling of the face), peripheral facial palsy, and lingua plicata (“scrotal”
tongue). Granulomatous cheilitis is a chronic swelling of the lip caused by granulomatous inflammation.
Miescher cheilitis is the term used when the granulomatous changes are confined to the lip.
Miescher cheilitis generally is regarded as a monosymptomatic form of the Melkersson-Rosenthal
syndrome, although the possibility remains that they are separate diseases.
Melkersson Syndrome; Cheilitis Granulomatosis; Orofacial
Granulomatosis Syndrome; Wiescher-Melkersson-Rosenthal Syndrome.
Unknown. Condition is rare. No racial or gender
predilection. Onset usually is in young adult life.
Autosomal dominant; gene on chromosome 9.
Probably of autoimmune origin related to HLA-DR
histocompatibility complex. Normal lip architecture eventually is altered by
the presence of lymphedema and noncaseating granulomas in the lamina
Episodic swelling of the face with relapsing peripheral
nerve palsy; uveitis is accompanied by granulomas elsewhere. The main
differential diagnosis is with sarcoidosis. However, the Kveim test is
Characterized by episodic swelling of the face
and orofacial granulomatosis. The eyelids, pharynx, oral mucosa, and larynx
may be affected by granuloma formation. The tongue is said to be “scrotal”
in appearance. Recurrent peripheral facial paralysis as a result of
involvement of the facial nerve.
History and examination should
assess the extent of airway involvement by granulomas.
Even in the presence of a normal upper
airway, the possibility of laryngeal and upper tracheal granulomas must be
considered. Upper airway and facial swelling may cause considerable problems
with face-mask fit and tracheal intubation. Postoperative airway obstruction
has been reported.
No specific considerations have been
Iceland Amyloidosis (Amyloiosis Type VI; Cerebral Amyloid
Angiopathy Syndrome; Icelandic Cerebroarterial Amyloidosis Syndrome):
Autosomal dominant disorder characterized by facial paralysis or swelling
around the mouth, which is similar to Melkersson-Rosenthal syndrome. Other
clinical features include premature stroke, intracranial hemorrhage, and
dementia with an onset during the third or fourth decade of life. Death
occurs before age 40 years and most often related to intracerebral
hemorrhage. There is evidence that the disorder is caused by mutation in
the gene encoding cystatin C in some patients. Cerebral amyloid angiopathy
has also been observed with high-density lipoprotein deficiency. The British
type of cerebral amyloid angiopathy is caused by mutations in the ITM2B
James DG: All that palsies is not Bell's. J R Soc Med
Jayamaha JEL: Respiratory obstruction in a patient with Melkersson-Rosenthal
syndrome. Anesth Analg