ARTICLE
ejd.2011.1517
Auteur(s) : Zhengxiao Li lizhengxiao1979@163.com,
Jingyi Yuan, Ping Liu, Shengxiang Xiao
Department of Dermatology and Venereology, The Second Affiliated
Hospital, Xi’an Jiaotong University, 157 Xi Wu Road, Xi’an 710004,
Shaanxi Province, PR. China
Melkersson-Rosenthal syndrome (MRS) is a rare neuromucocutaneous
disorder of unknown etiology characterized by the triad of
recurrent or persistent orofacial swelling, facial nerve palsy and
fissured tongue. Infections, autoimmunity, neurotropic factors,
atopy and hypersensitivity to food additives have been hypothesized
for this syndrome [1]. We report an orofacial granulomatosis in
complete MRS which was dramatically improved by systemic
azathioprine and corticosteroids, as well as compression
therapy.
A 45-year-old man presented with a 4-year history of
progressively persistent oedema of upper lip. The swelling
initially started on the left upper lip and gradually extended to
the right upper lip, right cheeks, right eyelids and periorbital
region. He was initially treated with oral antihistamines, topical
corticosteroids and metronidazole, without improvement. He had had
a trauma on the right forehead 15 years previously and had no
history of any medications.
The physical examination revealed a soft, non-pitting oedema of
the upper lip, right cheek and right eyelid, involving the
periorbital region (figure 1A)
and a fissured tongue. A 6 cm scar under the right supraorbital
ridge was noted. The rest of his physical examination was
unremarkable.
Electromyography studies showed a bilateral, partial facial
nerve palsy. Chest and gastrointestinal tract X-ray, cranial
computed tomographic scanning and magnetic resonance imaging were
normal. Routine laboratory tests and creatine phosphokinase,
lactate dehydrogenase, aldolase, angiotensin-converting enzyme,
rheumatoid factor, serum calcium, immunoglobulin, C-reactive
protein and complement (C3, C4) levels were within normal ranges.
The only positive findings included antinuclear antibody (1:40) and
anti-Jo1 (1:40). Histopathological findings in biopsy specimens of
the swollen lip showed edema of the corium with dilated lymphatic
and blood vessels and non-specific inflammatory infiltrate.
The patient was started on oral minocycline (100 mg/d),
clofazimine (100 mg/d) and topical tacrolimus for 3 weeks. As no
changes in the orofacial swelling resulted, oral azathioprine (100
mg/d) and methylprednisolone (16 mg/d), as well as compression
therapy was begun. Techniques to compress the swelling used a mask
(figure
1C) made with carbasus and cotton for approximately
30 minutes thrice daily. Orofacial swelling was dramatically
improved in 2 weeks by this association of treatments (figure
1B). Compression therapy was withdrawn, the
azathioprine and corticosteroid dose was gradually tapered over the
next four weeks. He has had no relapse after a 3 month
follow-up.
The treatment of orofacial granulomatosis in MRS remains a
challenge because of the unclear aetiopathogenesis. Various
therapeutic methods have been described [2-5]. Although complete
remission of facial swelling is possible in about half of patients
within 3 years of therapy, long-term systemic azathioprine and/or
corticosteroid usage is connected with a high risk of side-effects
[2], and so should not be the first line of treatment. Topical
triamcinolone or clobetasol in orobase may cause atrophy;
intralesional administration of triamcinolone remains favourable
with a rapid improvement, but the response is temporary [3].
Ratzinger et al. [4] observed complete or partial responses
in the majority of patients treated with clofazimine. Unfortunately
our case was unresponsive to clofazimine. To our knowledge, there
is only one English literature report of lip edema managed with
compression techniques. Van der Kooi et al. [5] reported two
patients with progressive swelling of the lips resistant to oral
antihistamines, topical tacrolimus and systemic or topical
corticosteroids, which responded successfully to compression
therapy.
Orofacial granulomatosis in our patient was not alleviated by
topical tacrolimus, or oral minocycline, clofazimine,
antihistamines and dramatically improved with systemic
azathioprine, corticosteroids and compression therapy. Although we
cannot determine the causal relationship between compression
therapy and rapid improvement of facial swelling, due to absence of
side effects of this physical treatment, it may be a useful
adjuvant treatment for refractory orofacial granulomatosis in MRS.
Further investigations are required.
Disclosure
Acknowledgments: We are grateful to Dr J. Wang for critical
comments and discussions. We thank Dr F. Ji for critic reading of
the manuscript. Financial support: none. Conflicts of interest:
none.
References
1. Zimmer WM, Rogers RS, Reeve CM, Sheridan P.J.
Orofacial manifestations of Melkersson-Rosenthal syndrome. A study
of 42 patients and review of 220 cases from the literature. Oral
Surg Oral Med Oral Pathol 1992 ; 74 : 610-619.
2. Al Johani KA, Moles DR, Hodgson TA, Porter SR, Fedele
S. Orofacial granulomatosis: clinical features and long-term
outcome of therapy. J Am Acad Dermatol 2010 ; 62 :
611-620.
3. Sciubba JJ, Said-Al-Naief N. Orofacial granulomatosis:
presentation, pathology and management of 13 cases. J Oral
Pathol Med 2003 ; 32 : 576-585.
4. Ratzinger G, Sepp N, Vogetseder W, Tilg H. Cheillitis
granulomatosa and Melkersson-Rosenthal syndrome: evaluation of
gastrointestinal involvement and therapeutic regimens in a series
of 14 patients. J Eur Acad Dermatol Venereol 2007 ; 21 :
1065-1070.
5. Van der Kooi K, Davis MD, McCloskey G. Chronic edema
of the lips – a rare but real problem: a report of 3 cases and
their response to therapy. J Am Acad Dermatol 2005 ; 53 :
875-877.
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