eMedicine Specialties > Dermatology > Diseases of the Oral Mucosa
Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome): Treatment & Medication
Updated: Oct 5, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Simple compression for several hours daily may produce significant improvement. Compression devices may reduce lip edema. Orofacial granulomatosis may improve with implementation of a cinnamon- and benzoate-free diet. Intralesional corticosteroids may be helpful in some patients.15 Success with other treatments has been reported anecdotally. None of the agents listed below has been systematically evaluated.
Immunomodulatory agents
- Nonsteroidal anti-inflammatory agents
- Mast cell stabilizers16
- Clofazimine17
- Methotrexate18
- Tacrolimus
- Infliximab19
- Dapsone
- Triamcinolone and dapsone injections20
Antimicrobials
- Tetracycline (used for anti-inflammatory activity)
- Minocycline21
- Roxithromycin22
- Antibiotic treatment of dental abscess - Resulted in remission in anecdotal cases
Surgical Care
- Surgery and radiation have been used.
- Surgery alone is relatively unsuccessful.
- Reduction cheiloplasty with intralesional triamcinolone and systemic tetracycline offer the best results.23 Give corticosteroid injections periodically after surgery to avoid an exaggerated recurrence.
Consultations
Consult a gastroenterologist, an immunologist, and an oral medicine specialist.
Medication
Clofazimine or metronidazole may produce resolution in granulomatous cheilitis. Intralesional corticosteroid (triamcinolone) injections may reduce swelling. Systemic corticosteroids are rarely indicated and not all cases respond.
Azathioprine, dapsone, sulfapyridine, or sulfasalazine may be helpful.
Long-term penicillin, tetracycline, erythromycin, and ketotifen are other management approaches that are occasionally helpful.
No randomized controlled trials have yet been recorded with possible therapies such as tacrolimus, thalidomide, or infliximab.
Antibiotics
Therapy must be comprehensive and should cover all likely pathogens in the clinical setting.
Clofazimine (Lamprene 50- or 100-mg cap)
Inhibits mycobacterial growth, binds preferentially to mycobacterial DNA. Has antimicrobial properties, but mechanism of action is unknown.
Adult
100 mg PO bid for 10 d, then twice weekly for 4 mo
Pediatric
1 mg/kg/d PO qd
Dapsone may inhibit anti-inflammatory activity
Documented hypersensitivity; breastfeeding; hepatic disease; renal disease
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Severe abdominal symptoms may require exploratory laparotomies; caution in patients with GI problems (eg, abdominal pain, diarrhea); skin discoloration due to drug may result in depression or suicide; apply oil to skin for dryness and ichthyosis; stains soft contact lenses
Dapsone (Avlosulfon)
Bactericidal and bacteriostatic against mycobacteria. Mechanism of action is similar to that of sulfonamides where competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth.
Adult
50-300 mg PO qd (average dose, 100 mg qd)
Pediatric
Not established
May inhibit anti-inflammatory effects of clofazimine; hematologic reactions may increase with folic acid antagonists, eg, pyrimethamine (monitor for agranulocytosis during second and third mo of therapy); probenecid increases toxicity; trimethoprim with dapsone may increase toxicity of both drugs; because of increased renal clearance, levels may significantly decrease when administered concurrently with rifampin
Absolute: Documented hypersensitivity
Relative: G-6-PD deficiency (especially in African Americans, Middle Easterners, and Asians), significant cardiopulmonary disease, significant hematologic disease, sulfa allergy (cautious use in patients with sulfa allergy may be attempted; cross-reactivity is relatively rare and mild)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Perform weekly blood counts (first mo), then perform WBC counts monthly (6 mo), and then semiannually; discontinue if significant reduction in platelets, leukocytes, or hematopoiesis occurs; caution in methemoglobin reductase deficiency, G-6-PD deficiency, or hemoglobin M because of high risk for hemolysis and Heinz body formation; caution in patients exposed to other agents or conditions (eg, infection, diabetic ketosis) capable of producing hemolysis; peripheral neuropathy can occur (rare); phototoxicity may occur when exposed to UV light
Anti-inflammatories
These agents decrease inflammatory responses and systemically interfere with events leading to inflammation.
Sulfapyridine (Dagenan)
Competitive antagonist of PABA. Mechanism of action in linear IgA dermatosis is unknown.
Adult
Initial dose is 500 mg PO bid; increase by 1 g q1-2wk until disease is controlled; control may require 1-4 g/d
Pediatric
35 mg/kg PO bid; not to exceed 100 mg/kg/d
Bioavailability of digoxin is reduced (interval of 2-3 h between administrations is recommended)
Documented hypersensitivity; slow acetylators may require smaller doses or more gradual initial dosage adjustment
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Idiosyncratic reactions, such as hypersensitivity pneumonitis, a lupuslike syndrome, pancreatitis, and toxic hepatitis, may occur; agranulocytosis rarely occurs; both immune hemolytic anemia and nonimmune hemolytic anemia develop (the latter is more common in patients with G-6-PD deficiency); folate deficiency may occur secondary to impaired absorption; nephrolithiasis may occur as with other sulfa drugs; toxic epidermal necrolysis has been reported with medications containing sulfa groups; check CBC count and liver function tests monthly for 5 mo then q6wk thereafter
Risk-benefit analysis should be considered if (1) allergy to sulfapyridine, other sulfonamides, furosemide, thiazide diuretics, sulfonylureas, or carbonic anhydrase inhibitors; (2) blood dyscrasias (sulfapyridine may cause agranulocytosis, aplastic anemia, or other blood dyscrasias); (3) G-6-PD deficiency (sulfapyridine may cause hemolytic anemia; (4) hepatic function impairment (sulfonamides are metabolized in liver and may cause hepatitis); (5) porphyria (sulfonamides may precipitate acute attack of porphyria); (6) renal function impairment (sulfapyridine excreted primarily through kidneys)
Sulfasalazine (Azulfidine)
Decreases inflammatory response and systemically inhibits prostaglandin synthesis.
Adult
500 mg PO qd
Pediatric
Not established
Decreases effects of iron, digoxin, and folic acid; conversely, increases effect of oral anticoagulants, oral hypoglycemic agents, and methotrexate
Documented hypersensitivity; sulfa drugs or any component; those diagnosed with GI or GU obstruction
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in patients with renal or hepatic impairment, blood dyscrasias, or urinary obstruction
Corticosteroids
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.
Triamcinolone (Aristocort)
For inflammatory dermatosis responsive to steroids. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability.
Adult
2.5-40 mg (10 mg/mL or 40 mg/mL formulations; intralesional); repeat prn but not to exceed q4-6wk
Pediatric
2.5-15 mg (10 mg/mL or 40 mg/mL solutions; intralesional); repeat prn but not to exceed q4-6wk
None reported
Documented hypersensitivity; fungal, viral, and bacterial skin infections
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Do not use in decreased skin circulation; prolonged or frequent use may result in Cushing syndrome, reversible HPA-axis suppression, hyperglycemia, and glycosuria
Immunosuppressants
These agents inhibit key factors that mediate immune reactions, which, in turn, decrease inflammatory responses.
Azathioprine (Imuran)
Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.
Adult
1 mg/kg qd/bid (empiric) or by TPMT level; increase by 0.5 mg/kg q4wk until response, not to exceed 2.5 mg/kg/d
TPMT testing not entirely reliable; involves testing activity of TPMT activity in RBCs, which correlates with systemic TPMT activity; functional enzyme test has been shown to have variability between test sites, and kits may contain varying amounts of enzyme inhibitor; starting at low doses, monitoring for pancytopenia, then increasing the dose is an alternative; if clinical response is not good, patient may be a homozygote for high activity and may need increased dose; some references recommend checking before treatment in all patients
TPMT <5 U: No treatment with azathioprine
TPMT 5-13.7 U: Not to exceed 0.5 mg/kg
TPMT 13.7-19 U: Not to exceed 1.5 mg/kg
TPMT >19 U: Not to exceed 2.5 mg/kg
Pediatric
Safety and efficacy not established
Allopurinol increases risk of pancytopenia; captopril/ACE inhibitors may increase risk of anemia and leukopenia; warfarin dose may need to be increased; pancuronium dose may need to be increased for adequate paralysis; live virus vaccines and cotrimoxazole increase risk of hematologic toxicity; rifampicin may cause transplants to possibly be rejected; clozapine may increase risk of agranulocytosis
Absolute: Documented hypersensitivity, pregnancy or attempting pregnancy, clinically significant active infection
Relative: Concurrent use of allopurinol; prior treatment with alkylating agents (eg, cyclophosphamide, chlorambucil, melphalan, others [high risk of neoplasia])
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Increased risk of neoplasia; caution in liver disease and renal impairment; hematologic toxicities may occur; rarely, patients may develop fever without associated infections; measure thiopurine methyltransferase level prior to treatment; periodically monitor CBC count and liver function
More on Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome) |
| Overview: Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome) |
| Differential Diagnoses & Workup: Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome) |
 Treatment & Medication: Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome) |
| Follow-up: Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome) |
| Multimedia: Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome) |
| References |
| Further Reading |
| « Previous Page | Next Page » |
References
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Further Reading
National Institute of Neurological Disorders and Stroke -NINDS Melkersson-Rosenthal Syndrome Information Page
Keywords
granulomatous cheilitis, Miescher-Melkersson-Rosenthal syndrome, orofacial granulomatosis, Miescher cheilitis, Melkersson-Rosenthal syndrome, Rossolimo-Melkersson-Rosenthal syndrome, swelling of the lip, Crohn disease, Crohn's disease
