Successful treatment of Melkersson‐Rosenthal Syndrome with lymecycline

ARTICLE

Auteur(s) : Barbara PIGOZZI, Anna Belloni FORTINA, Andrea PESERICO

Clinica Dermatologica di Padova via Cesare Battisti, 206 35128 Padova Italy

Article accepted 02/03/2004

Melkersson-Rosenthal syndrome, a granulomatous disease, of which the etiology is unknown [1-3], is clinically characterized by the triad: recurrent orofacial oedema, lingua plicata, recurrent mono- or bilateral peripheral facial nerve palsy.
Many therapeutic regimens have been tried, including corticosteroids, antihistamines, antibiotics, clofazimine, hydroxychloroquine, salazosulphapyridine and dapsone, often with disappointing results. When the oedema becomes persistent, the final therapeutic option is surgical reduction.
We report the case of a 31-year-old woman affected by Melkersson-Rosenthal syndrome successfully treated with lymecycline, after disappointing results with steroids alone or combined with antihistamines, sulphasalazine and clofazimine.

Case report

A 31-year-old woman came to our clinic in November 2001 with persistent swelling of the lips which had lasted for some weeks. The visit revealed a labial oedema of parenchymatous consistence and a fissured tongue. The patient denied any symptoms besides the functional and psychological distress caused by the oedema. These symptoms first occurred 15 years previously with recurrent episodes of swelling of the upper lip and subsequently of the lower lip and were treated with a cycle of local steroid injection with complete remission each time. Ten years ago she had a relapse with 2-3 episodes/year and then 4-5 episodes/year of oedema of the lips and part of the face, in spite of treatment with oral and intralesional steroids, antihistamines and subsequently with clofazimine. From December 2000, in spite of treatment with salazosulphapyridine and steroids, she experienced a further worsening and in the last weeks a persistent oedema. She also recalled an episode of facial palsy about 10 years ago and a second episode in September 2001 which were treated with steroids. Laboratory findings including complement factors, IgG/A/M, total and specific IgE dosage, anti-nuclear and extractable nuclear antigen antibody titer, angiotensin-converting enzyme levels, prick and patch tests against the most common haptens, chest and abdomen X-ray were normal. On the basis of laboratory and clinical data, the diagnosis of Melkersson-Rosenthal syndrome was made and a therapy with lymecycline 300mg/die was started, once informed consent was obtained and a pregnancy was excluded. The patient noted a complete regression of the oedema after one week of treatment and she did not experience relapse or facial nerve palsy during the following months. After 3 months the dosage of lymecycline was reduced to 150mg/die and after another 3 months to 150mg/every other day. The patient is now in persistent complete remission.

Discussion

The treatment of Melkersson-Rosenthal syndrome still remains largely symptomatic and often disappointing. The use of tetracyclines is based on the anti-inflammatory effect of the drugs, which have elective affinity for inflamed tissues and act on polymorphonuclear neutrophils by inhibiting chemotaxis [4, 5] and interfering with phagocytosis and the oxidative process [6]. Other authors have already reported success and failures with the use of tetracyclines [7], in particular minocycline [8]. This is the first report on the use of lymecycline in a granulomatous disease. Lymecycline, generated from the fusion of a tetracycline molecule with a L-lysin molecule [9], was chosen instead of minocycline or another tetracycline, for its positive safety profile and for the practicality of this therapy [10]. In our case, lymecycline used at a medium-low dosage induced a complete remission of the cutaneous symptomatology after one week of treatment. After 2 years of therapy with lymecycline the patient is in complete remission and the follow-up hematologic and biochemical assays are all within normal limits.
Lymecycline may present an efficacious option for the treatment of Melkersson-Rosenthal syndrome while avoiding the side effects of corticosteroids and/or other more aggressive therapeutic approaches. n

References

1. Greene RM, Rogers III RS. Melkersson-Rosenthal syndrome: a review of 36 patients. J Am Acad Dermatol 1989; 21: 1236-70.

2. Rogers III RS. Granulomatous cheilitis, Melkersson-Rosenthal syndrome and orofacial granulomatosis. Arch Dermatol 2000; 136: 1557-8.

3. Meisel-Stosiek M, Horenstein OP, Stosiek M. Family study on Melkersson-Rosenthal syndrome. Acta Derm Venereol (Stockh) 1990; 70: 221-6.

4. Gemmell CG. Antibiotics and neutrophil function – potential immunomodulating activities. J Antimicrob Chemotherapy 1993; 31, suppl. B: 23-33.

5. Esterly NB, Koransky JS, Furey NL, Trevisan M. Neutrophil chemotaxis in patients with acne receiving oral tetracycline therapy. Arch Dermatol 1984; 120: 1308-13.

6. of the oxiSorsa T, Ramamurthy NS, Vernillo AT, Zhang X, Konttinen YT, Rifkin BR, Golub LM. Functional sites of chemically modified tetracyclines: inhibition dative activation of human neutrophil and chicken osteoclast pro-matrix metalloproteinases. J Rheumatol 1998; 25: 975-82.

7. Henderson CD, Tschen JA. Granulomatous cheilitis: case report and literature review. Cutis 1988; 41: 35-7.

8. Veller Fornasa C, Catalano P, Peserico A. Minocycline in granulomatous cheilitis: experience with 6 cases. Dermatology 1992; 185: 220.

9. Schreiner A, Digranes A. Pharmacokinetics of lymecycline and doxycycline in serum and suction blister fluid. Chemotherapy 1985; 31: 847-8.

10. Cunliffe WJ et al. A comparison of the efficacy and safety of lymecycline and minocycline in patients with moderately severe acne vulgaris. Eur J Dermatol 1998; 8: 161-6.