Cheilitis granulomatosa Miescher is a rare condition of unknown cause characterized by intermittent lip swelling that gradually persists and causes cosmetic deformity. We report the case of a young woman with cheilitis granulomatosa as a monosymptomatic manifestation of Melkersson-Rosenthal syndrome successfully treated by the antileprosy agent clofazimine, and propose clofazimine as an alternative treatment in cases refractory to corticosteroids. The differential diagnosis and current methods of treatment are summarized, and the literature is reviewed and discussed.
MiescherG. Über essentielle granulomatöse Makorcheilie (Cheilitis granulomatosa). Dermatologica1945;91:57–85.
2.
FisherAA. Chronic lip edema with particular reference to the Melkersson-Rosenthal syndrome (MRS). Cutis1990;45:144–6.
3.
HornsteinOR. Melkersson-Rosenthal syndrome — A challenge for dermatologists to participate in the field of oral medicine. J Dermatol1997;24:281–96.
4.
WorsaaeNPindborgJJ. Granulomatous gingival manifestations of Melkersson-Rosenthal syndrome. Oral Surg Oral Med Oral Pathol1980;49:131–8.
5.
MelkerssonE. Ett fall av recidiverande facial spares i samband med angioneurotiskt ödem. Hygiea (Stockh)1928;90:737–41.
6.
RosenthalC. Klinisch-erbbiologischer Beitrag zur Konstitutionspathologie. Gemeinsames Auftreten von (rezidivierender familiärer) Fazialislähmung, angioneurotischem Gesicht- södem und Lingua plicata in Arthritis-Familien. Z Ges Neurol Psych1931;131:475–501.
7.
TauschISonnichsenN. Erfahrungen mit der Clofazimin-Therapie des Melkersson-Rosenthal-Syndroms. Hautarzt1992;43:194–8.
8.
LygidakisCTsakanikasCIliasAVassilopoulosD. Melkersson-Rosenthal's syndrome in four generations. Clin Genet1979;15:189–92.
EllitsgaardNAnderssonAPWorsaaeNMedgyesiS. Long-term results after surgical reduction cheiloplasty in patients with Melkersson-Rosenthal syndrome and cheilitis granulomatosa. Ann Plast Surg1993;31:413–20.
11.
PodmorePBurrowsD. Clofazimine — An effective treatment for Melkersson-Rosenthal syndrome or Miescher's cheilitis. Clin Exp Dermatol1986;11:173–8.
GarreltsJC. Clofazimine: A review of its use in leprosy and Mycobacterium avium complex infection. DICP1991;25:525–31.
14.
HoldinessMR. Clinical pharmacokinetics of clofazimine. A review. Clin Pharmacokinet1989;16:74–85.
15.
MackeyJRBarnesJ. Clofazimine in the treatment of discoid lupus erythematosus. Br J Dermatol1974;91:93–6.
16.
MichaelssonGMolinLOhmannS. Clofazimine. A new agent for the treatment of pyoderma gangrenosum. Arch Dermatol1976;112:344–9.
17.
KaplanBTrauHSoferEFeinsteinASchewach-MilletM. Treatment of pyoderma gangrenosum with clofazimine. Int J Dermatol1992;31:591–3.
18.
ArbiserJLMoschellaSL. Clofazimine: A review of its medical uses and mechanisms of action. J Am Acad Dermatol1995;32:241–7.
19.
KanoYShioharaTYagitaANagashimaM. Granulomatous cheilitis and Crohn's disease. Br J Dermatol1990;123:409–12.
20.
ClearyKRBatsakisJG. Orofacial granulomatosis and Crohn's disease. Ann Otol Rhinol Laryngol1996;105:166–7.
21.
RogersRSIII. Melkersson-Rosenthal syndrome and orofacial granulomatosis. Dermatol Clin1996;14:371–9.
22.
MirallesJBarnadasMAde MoragasJM. Cheilitis granulomatosa treated with metronidazole. Dermatology1995;191:252–3.
23.
SteinSLManciniAJ. Melkersson-Rosenthal syndrome in childhood: Successful management with combination steroid and minocycline therapy. J Am Acad Dermatol1999;41:746–8.
24.
SussmanGLYangWHSteinbergS. Melkersson-Rosenthal syndrome: Clinical, pathologic, and therapeutic considerations. Ann Allergy1992;69:187–94.
25.
MahlerVBHornsteinOPBoatengBIKiesewetterFF. Granulomatous glossitis as an unusual manifestation of Melkersson-Rosenthal syndrome. Cutis1995;55:244–6, 248.
26.
RidderGJStrohhäckerHLöhleEGolzAFradisM. Laryngeal sarcoidosis: Treatment with the antileprosy drug clofazimine. Ann Otol Rhinol Laryngol2000;109: 1146–9.
SarracentJFinlayCM. The action of clofazimine on the level of lysosomal enzymes of cultured macrophages. Clin Exp Immunol1982;48:261–7.
29.
AndersonRZeisBMAndersonIF. Clofazimine-mediated enhancement of reactive oxidant production by human phagocytes as a possible therapeutic mechanism. Dermatologica1988;176:234–42.
30.
JoplingWH. Complications of treatment with clofazimine (Lamprene: B663). Lepr Rev1976;47:1–3.
31.
DesikanKVRamanujamKRamuGBalakrishnanS. Autopsy findings in a case of lepromatous leprosy treated with clofazimine. Lepr Rev1975;46:181–9.