What is granulomatous cheilitis?
Granulomatous cheilitis refers to an uncommon condition in which there is lumpy swelling of the lips. It is also known as cheilitis granulomatosa and is part of the spectrum of orofacial granulomatosis. There are many different causes, such as allergy, Crohn disease, sarcoidosis and orofacial granulomatosis. Rare causes are infections, cancers and genetic disorders.
Miescher-Melkersson-Rosenthal syndrome refers to recurrent chronic (long lasting) swelling and enlargement of one or both lips. Facial palsy (muscle weakness) and fissuring of the tongue may also occur. There is no known cause for this syndrome but genetics may have a role to play as members of a single family have been affected.
The condition is rare and it may occur in people of any race, sex and age, although onset is usually in early adulthood.
What are the signs and symptoms?
In Miescher cheilitis, the changes are confined to the lip. The first symptom is a sudden swelling of the upper lip. In most cases this first episode goes away completely within hours or days. Swelling of the lower lip and one or both cheeks may follow in Melkersson-Rosenthal syndrome. Less commonly, the forehead, eyelids, or one side of the scalp may be involved. The swelling may feel soft, firm or nodular when touched.
Recurrent attacks may occur within days or even years after the first episode. At each episode the swelling may become larger, more persistent and eventually become permanent. At this time the lips may crack, bleed and heal leaving a reddish-brown colour with scaling. This can be painful. Eventually the lip takes on the consistency of hard rubber.
Other symptoms that may occur include:
- Fever, headache and visual disturbances
- Mild enlargement of regional lymph nodes in 50% of cases
- Fissured or plicated (pleat-like effect) tongue in 20-40% of cases
- Facial palsy (paralysis); intermittent, then possibly permanent and can be unilateral or bilateral, and partial or complete. It occurs in about 30% of cases.
How is the diagnosis made?
The diagnosis is often suspected clinically. Skin biopsy of the affected tissue shows characteristic histopathology, in which there are granulomas, i.e. a mixed inflammatory cell infiltration, in the dermis (the deeper layer of the skin).
What treatment is available?
If it is related to an allergy, responsible dietary components or causative substances should be avoided long term. If there is underlying disease, systemic treatment for this may also reduce the swelling of the lips.
The following measures have been reported to reduce the severity of Miescher cheilitis or Melkersson-Rosenthal granulomatous cheilitis in at least some cases.
- Topical corticosteroids
- Long term anti-inflammatory antibiotics e.g. a six to twelve month course of tetracycline, erythromycin or penicillin
- Intralesional corticosteroids injected into the lips to reduce swelling. Injections need to be repeated every few months.
- Non-steroidal antiinflammatory agents
- Mast cell stabilisers eg ketotifen
- Surgical reduction