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Melkersson-Rosenthal syndrome

Melkersson-Rosenthal syndrome is a rare neurological disorder characterized by recurring facial paralysis, swelling of the face and lips (usually the upper lip), and the development of folds and furrows in the tongue. Onset is in childhood or early adolescence. After recurrent attacks (ranging from days to years in between), swelling may persist and increase, eventually becoming permanent. The lip may become hard, cracked, and fissured with a reddish-brown discoloration. The cause of Melkersson-Rosenthal syndrome is unknown, but there may be a genetic predisposition. It can be symptomatic of Crohn's disease or sarcoidosis. more...

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Treatment

Treatment is symptomatic and may include medication therapies with nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids to reduce swelling, as well as antibiotics and immunosuppressants. Surgery may be recommended to relieve pressure on the facial nerves and to reduce swollen tissue, but its effectiveness has not been established. Massage and electrical stimulation may also be prescribed.

Prognosis

Melkersson-Rosenthal syndrome may recur intermittently after its first appearance. It can become a chronic disorder. Follow-up care should exclude the development of Crohn's disease or sarcoidosis.

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Clofazimine as elective treatment for granulomatous cheilitis
From Journal of Drugs in Dermatology, 5/1/05 by Lourdes Rodriguez Fdez.-Freire

Abstract

Cheilitis granulomatosa (CG) is a rare, idiopathic inflammatory disorder that usually affects young adults and clinically is characterized by diffuse, non-tender, soft to firm swelling of one or both lips. A variant of granulomatous cheilitis is Melkersson-Rosenthal syndrome when associated with facial paralysis and furrowed tongue. Several treatments have been used with variable success. We report 3 cases of GC treated with oral clofazimine 100 to 200 mg daily for 3 to 6 months obtaining regression of lesions in all treated cases. Hyperpigmentation and elevation of liver enzymes were observed as side effects.

**********

Introduction

Granulomatous cheilitis was described in 1945 by Miescher. (1) Melkersson, in 1928, reported a patient with oedema of the lip and facial paralysis, suggesting a relationship between both symptoms. Rosenthal, in 1931, added the sign of creased tongue and since 1949 the triad has been called Melkersson-Rosenthal syndrome. (2)

Miescher's granulomatous cheilitis is an uncommon disease, which manifests in young adults, without predominance of sex. Clinically, it is characterized by a unilateral swelling of the lips, most frequently the upper, which becomes permanent if it persists. Histologically, non-necrotizing granulomas are seen, as well as oedema, lymphangiectasia, and perivascular lymphocytic infiltration.

The of this granulomatous inflammation is unknown, but it has been associated with hereditary, infectious, (3) allergic, and immunological (4) factors. Thus, this entity may be associated with another granulomatous disease, (5-7) in particular Crohn's disease, which can appear from months to years after the mouth lesion.

Several treatments have been used with variable degrees of success including prednisone, minocycline, (8) anti-malarials, (9) oral tetracycline, (10) metronidazole, (11,12) cheiloplasty, and intra-lesional corticoid injections. (13) We point out the excellent result of oral clofazimine in this condition.

Case Report 1

A 34-year-old male presented with a 1-year history of constant oedema of the upper lip. Physical examination showed an asymptomatic indurated swelling (Figure 1). The patient did not show facial paralysis or affected tongue. Histological examination revealed chronic inflammation with small non-necrotizing granulomas scattered throughout the submucosal connective tissue. PAS stains showed no evidence of fungal organisms or acid-fast bacilli (Figures 2 and 3). We carried out treatment with clofazimine at 300 mg/day, with an evident decrease of edema and induration of the lip after 5 months of treatment (Figure 4).

Case Report 2

A 24-year-old female presented with an 18-month history of swelling of the lower lip. Physical examination showed oedema with fissures and xerosis indurated on palpation. Laboratory and X ray examinations were performed in order to exclude infectious disease and digestive disorders. Histological examination confirmed GC. Oral treatment with clofazimine 100 mg/12 hours was started observing poor regression of lesions. Then we increased it to 300 mg/day for 6 months, obtaining a notable improvement of the lesions. Facial hyperpigmentation was seen as a side effect.

[FIGURE 1 OMITTED]

[FIGURE 2 OMITTED]

Case Report 3

A 73-year-old male presented with a permanent oedema of the upper lip after dental extraction, which did not respond to antibiotic treatment. He was treated with clofazimine 100 mg/12 hours for 2 months obtaining visible improvement of lesion. After 2 months of treatment an elevation of transaminases enzymes (GOT 150, GPT 185) was observed and we withdrew it.

Discussion

Clofazimine is an oral phenazine dye used for many years to treat leprosy. Clofazimine has demonstrated antibacterial, antiinflammatory, and immunomodulatory properties. (14) It appears to be specifically useful in conditions characterized by granulomas, including GC. Other indications are pyoderma gangrenosum, discoid systemic lupus erythematous, and pustular psoriasis. (15) Until now, there were few reports about oral clofazimine in GC.

[FIGURE 3 OMITTED]

[FIGURE 4 OMITTED]

In 1986, Podmore et al (16) described 3 cases of Miescher's granulomatous cheilitis and 1 case of Melkersson-Rosenthal syndrome treated with oral clofazimine over a 4-month period. All cases showed histological improvement with clearance of granulomata, but persistence of oedema. Sussman (17) treated 10 patients with MRS with clofazimine (100 mg 4 times weekly for 3 to 11 months) observing a complete remission in 50% of patients. In our cases, oral clofazimine obtained excellent results in all cases.

Secondary effects are dose dependent, the most frequent being those produced at a dermatological level, especially skin pigmentation (75% to 100% of cases) acquiring a color that varied from red to brownish-black, and normally appeared a few weeks after the treatment; ichthyosis and xerosis (8% to 28%), gastrointestinal level, with nausea and vomiting (40% to 50%); hepatotoxicity (1%) and ophthalmological problems (1%) were other reported side effects.

In conclusion, due to the unknown aethiology, the treatment of granulomatous cheilitis is difficult. We have obtained good responses with oral clofazimine at high doses, but we recommend clinical and analytical control to avoid secondary effects.

References

1. Miescher G. Uber essentielle granulomatose Makrocheillie (cheilitis granulomatosa). Dermatologica. 1945;91:57-58

2. Armstrong DKB, Burrows D. Orofacial granulomatosis. Int J Dermatol. 1995;34:830-3.

3. Muellegger RR, Weger W, Zoechling N, et al. Granulomatous cheilitis and Borrelia burgdorferi: polymerase chain reaction and serologic studies in a retrospective case series of 12 patients. Arch Dermatol. 2001;136(12):1502-6.

4. Allen CM, Camisa C, Hamzeh S, et al. Cheilitis granulomatosa: report of six cases and review of the literature. J Am Acad Dermatol. 1990;23:444-50.

5. Worsaae N, Christensen KC. Melkersson-Rosenthal syndrome and Crohn's disease. British Journal of Oral Surgery, 1980;18:254-258.

6. Van de Scheur MR, van der Waal RI, Volker-Dieben HJ, Klinkenberg-Knol EC, Starink TM, van der Waal I. M. R. van de Scheur, et al. Orofacial granulomatosis in a patient with Crohn's disease. J Am Acad Dermatol. 2003;49:952-4.

7. Bogenrieder T, Rogler G, Vogt T, Landthaler M, Stolz W. Orofacial granulomatosis as the initial presentation of Crohn's disease in an adolescent. Dermatology. 2003;206(3):273-8.

8. Stein SL, Mancini AJ. Melkersson-Rosenthal syndrome in childhood: successful management with combination steroid and minocycline therapy. J Am Acad Dermatol. 1999;41:746-8.

9. Schumacher F, Schnyder UW. Cheilitis et pareitis granulomatosa: spontanverlauf. Hautarzt. 1991;42:516-517.

10. Fisher AA. Chronic lip edema with particular reference to the Melkersson-Rosenthal syndrome(MRS). Cutis. 1990;45:144-146.

11. Martinez Fernandez M, Rguez Prieto. Queilitis granulomatosa:respuesta al tratamiento con Metronidazol. Actas Dermo-Sifiliograficas. 2002;93:187-9.

12. Kano Y, Shiohara T, Yagita A. Treatment of recalcitrant cheilitis granulomatosa with metronidazole. J Am Acad Dermatol. 1992;27(4):629-30.

13. Camacho F, Garcia-Bravo B, Carrizosa A. Treatment of Miescher's cheilitis granulomatosa in Melkersson-Rosenthal syndrome. J Eur Acad Dermatol Venereol. 2001;15:546-549.

14. Sarracent J, Finlady C M. The action of clofazimine on the level of lysosomal enzymes of cultured macrophages. Clinical and Experimental Immunology. 1975;48:261-267.

15. Neuhofer J, Fritsch P. Cheilitis granulomatosa: therapy with clofazimine. Hautarzt. 1984, 35, 459-463.

16. Podmore P, Burrows D. Clofazimine-an effective treatment for Melkersson-Rosenthal syndrome or Miescher's cheilitis. Clin Exp Dermatol. 1986;11:173-178.

17. Sussman GL, Yang WH, Steinberg S. Melkersson-Rosenthal syndrome: clinical, pathological and therapeutic considerations. Annals of Allergy. 1992;69:187194.

Lourdes Rodriguez Fdez.-Freire MD, (a) Amalia Serrano Gotarredona MD, (a) Jose Bernabeu Wittel MD, (a) Agueda Pulpillo Ruiz MD PhD, (b) Rocio Cabrera MD, (c) Manuel Navarrete Ortega MD, (c) Julian Conejo-Mir MD PhD (d)

a. Resident of Dermatology

b. Consultant of Dermatology

c. Consultant of Dermatopathology

d. Head professor of Dermatology

Departments of Dermatology and Dermatopathology, Virgen del Rocio University Hospital, Seville, Spain

Address for Correspondence

Julian Conejo-Mir, MD

Rep. Argentina 22-B, 2 A

41011 Sevilla. Spain

email: julian.sanchez.sspa@juntadeandalucia.es

COPYRIGHT 2005 Journal of Drugs in Dermatology, Inc.
COPYRIGHT 2005 Gale Group

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