Dear Label_Name,

Greetings! I hope you are well.

I am writing on behalf of an international publishing house, Science and Education Publishing, USA (http://www.sciepub.com).

Science and Education Publishing (SciEP) is a multidisciplinary open access publisher of Journals covering the fields of Science, Technology and Medicine. All SciEP publications are subject to high-quality peer review, editorial and production processes and are freely available online.

Welcome to the April, 2013 edition of SciEP newsletter, providing monthly summaries of articles on science & education related topics of interest to scientist and professionals. The mission of our journals is to provide an open access forum for publishing new findings on issues pertinent to scientific and academic field. We also invite you to submit original research papers, review articles, short reports, or case studies are considered for publication in our Multidisciplinary Journals.

American Journal of Public Health Research
DOI: 10.12691/ajphr-1-1-3

The Implementation of a Tele-Homecare System with Service Oriented Architecture and HL7 Message Transmission Standard
Ching-Sung Wang

American Journal of Medical and Biological Research
DOI: 10.12691/ajmbr-1-1-2

The Influence of Ethanol on Pyruvate Kinases Activity in Vivo, in Vitro, in Silico
Lelevich Sergey Vladimirovich, Khrustalev Vladislav Victorovich, Barkovsky Eugene Victorovich, Shedogubova Tatyana Aleksandrovna

American Journal of Medical Sciences and Medicine
DOI: 10.12691/ajmsm-1-1-1

Clinical and Histological Features in Wegener Granulomatosis
Li Cavoli Gioacchino, Passantino Rita, Bono Luisa, Tortorici Calogera, Ferrantelli Angelo, Giammarresi Carlo, Rotolo Ugo

American Journal of Infectious Diseases and Microbiology
DOI: 10.12691/ajidm-1-1-2

The Curse of Lymphatic Filariasis: Would the Continual Use of Diethylcarbamazine Eliminate this Scourge in Papua New Guinea?
Anne Lanham, Lillian Mwanri

We have released issues of some journals on February 2013.
American Journal of Public Health Research
American Journal of Medical Sciences and Medicine
American Journal of Medical and Biological Research
American Journal of Pharmacological Sciences
American Journal of Clinical Medicine Research
American Journal of Nursing Research
American Journal of Cancer Prevention
American Journal of Medicine Studies
American Journal of Infectious Diseases and Microbiology
American Journal of Cardiovascular Disease Research
American Journal of Biomedical Research
American Journal of Mechanical Engineering
American Journal of Microbiological Research
Journal of Preventive Medicine
American Journal of Food and Nutrition
American Journal of Hypertension Research
American Journal of Sports Science and Medicine
Journal of Cancer Research and Treatment
American Journal of Zoological Research
Journal of Biomedical Engineering and Technology
American Journal of Microbiological Research
Biomedical Science and Engineering

We are pleased to announce that submissions are now being accepted for peer review for all the journals across all our subject areas in SciEP.

Please submit your manuscript via our online submission system:
http://www.sciepub.com/author/signin

To unsubscribe from our mailing list, please click on the following link: unsubscribe

I am looking forward to hearing from you.

Kind regards,

David Rain
Email:join@sciepub.com
Science and Education Publishing (http://www.sciepub.com)

Melkersson-Rosenthal Syndrome Associated to Behçet Disease

Salem Bouomrani*, Abir Masmoudi, Sihem Riahi, Mohamed Amin Bouaziz, Nour Wali, Warda Mahdhaoui

Department of Internal medicine, Military Hospital of Gabes, Gabes 6000, Tunisia

Abstract: Melkersson-Rosenthal syndrome (MRS) is an extremely rare disorder whose complete and typical form associates the triad: recurrent nonpitting orofacial edema, facial paralysis, and lingua plicata (fissured dorsal tongue). The exact pathogenesis of this disease is not yet fully understood. Its association with several other autoimmune disorders and the granulomatous nature of this syndrome suggest a dysimmune origin. The association with systemic vasculitis remains unusual with only two cases previously reported. We report the original case of MRS associated with Behçet disease in a 36-year-old Tunisian woman with favorable outcome under colchicine. Our observation is, to our knowledge, the first to report this association.

Keywords: Melkersson-Rosenthal syndrome, Behçet disease, systemic vasulitis, granulomatosis, lingua plicata

Received February 19, 2021; Revised March 22, 2021; Accepted March 30, 2021

Citation: Salem Bouomrani, Abir Masmoudi, Sihem Riahi, Mohamed Amin Bouaziz, Nour Wali, Warda Mahdhaoui, Melkersson-Rosenthal Syndrome Associated to Behçet Disease. American Journal of Medical Case Reports, 9 (6). 342-344. January 2021. doi:10.12691/ajmcr-9-6-10

1. Introduction

Described for the first time in 1928 by Ernst Gustav Melkersson [1], and defined as an authentic nosological entity in 1932 by Curt Rosenthal [2], Melkersson-Rosenthal syndrome (MRS) is an extremely rare neuromucocutaneous disorder with multifactorial pathogenesis involving inflammation, immune deficiency, infections, stress, food intolerance, and genetic predisposition [3,4]. Its incidence is estimated at 0.08%, and its complete and typical clinical presentation associates recurrent nonpitting orofacial edema, facial paralysis, and fissured dorsal tongue (lingua plicata) [3,4,5]. This triad is noted only in 8-25% of patients with MRS; other forms are called oligosymptomatic or monosymptomatic and represent a real diagnostic challenge [5].

The association of this syndrome with other systemic and/or autoimmune diseases has been reported [6]. That with systemic vasculitis remains exceptional and unusual with only two cases found in the world literature [7,8].

We report the original case of MRS associated with Behçet disease; an association which, to our knowledge, has not been reported before.

2. Case Presentation

A 36-year-old woman with a past personal history of primary hypothyroidism, allergic rhinitis, asthma, and three episodes of reversible peripheral facial paralysis managed by systemic corticosteroids, was referred to our consultation for persistent lips edema.

Its history goes back four years, with the occurrence of cyclic episodes of edema of the lips, particularly the upper one, associated three times with peripheral facial palsy (left in 2016, right in 2017, and left in 2018). The biological assessment in the three episodes was without abnormalities as well as cerebral magnetic resonance imaging in 2017. She was treated with systemic corticosteroids with regression of the facial paralysis but the orofacial edemas continue to recur.

Figure 1. marked edema of the lips

Somatic examination noted an indolent and nonpitting edema of lips without any change in color (Figure 1), macroglossia, and fissured tongue with multiple dorsal transverse cracks (lingua plicata) (Figure 2). No facial asymmetry was noted and examination of the cranial pairs did not show any abnormalities.

Figure 2. macroglossia with fissured tongue (lingua plicata)

Biopsy of accessory salivary glands objectified noncaseating granulomas with chronic sialadenitis, moderate fibrosis, and vasculitis.

The basic laboratory tests were within normal limits: total blood count, erythrocyte sedimentation rate, C-reactive protein, fast glycaemia, transaminases, calcemia, creatinine, uric acid, muscle enzymes, ionogram, lipid parameters, and electrophoresis of serum proteins. Thyroid-stimulating hormone was at 2.60 µUI/ml and C1-inhibitor assay was normal.

Subsequent investigations made it possible to eliminate other granulomatosis, especially sarcoidosis and tuberculosis: specialized ENT examination with sinus radiography, chest X-ray, thoraco-abdomino-pelvic CT scan, quantiferon, and angiotensin converting enzyme activity measurement.

Based on these findings (recurrent facial paralysis, recurrent orofacial edema, lingua plicata, and noncaseating granulomas at lip biopsy) the diagnosis of MRS was retained.

The images of vasculitis on the lip biopsy were unusual and prompted us to look for possible associated systemic vasculitis.

The resumption of the questioning revealed the notion of recurrent mouth ulcers since childhood and three episodes of genital ulcers in the last year.

Closer somatic examination noted two aphthous ulcerations on the inside of the right cheek, hypochromic scars on the labia majora, dermographism, and multiple necrotic pseudofolliculitis on the back.

The Pathergy test was positive and HLA typing revealed a B51 haplotype. The ophthalmologic examination was without abnormalities as well as brain MRI and lumbar puncture. Thus, the diagnosis of Behçet's disease without neurological involvement was retained.

Anti-nuclear, anti- double-stranded DNA, anti-soluble antigen, and anti-neutrophil cytoplasmic antibodies (ANCA) were negative eliminating ANCA-associated vasculitis or vasculitis secondary to connective tissue diseases.

The patient was treated with colchicine with a rapidly favorable outcome: total regression of lip edema (Figure 3), clear regression of macroglossia and lingual fissures (Figure 4), and absence of recurrence.

Figure 3. Total regression of lip edema after colchicine treatment

Figure 4. Clear regression of macroglossia and lingual fissures after colchicine treatment

3. Discussion

Patients diagnosed with MRS may have other comorbidities, particularly of dysimmune origin. The most frequently reported associations are: autoimmune thyroiditis, systemic lupus erythematous, psoriasis, sarcoidosis, inflammatory bowel diseases, and multiple sclerosis [6,8,9,10,11,12].

The combination of MRS with systemic vasculitis remains exceptional and unusual; indeed the review of the literature finds only two cases: one sporadic case of vascular purpura reported by Braunsteiner and Sailer in 1964 [7], and one case of unspecified vasculitis in 2013 in the large series of Elias MK et al. of 72 patients with biopsy-confirmed MRS [8].

The association with Behçet's disease, which is a systemic vasculitis particularly frequent in the Mediterranean basin and countries along the ancient silk road [13,14], has not been previously reported.

These two diseases present several common pathogenic mechanisms, particularly genetic predisposition, autoinflammatory character, and immune dysfunction [3,4,13,14]. Likewise, several specific symptoms of Behçet's disease have been reported in patients with MRS: aphthous ulcers [2], uveitis [15], arthritis [16], and erythema nodosum [2].

These different findings suggest that the association between MRS and Behçet's disease is far from being a mere coincidence. MRS can thus, as in our observation, be an early manifestation of Behçet's disease.

4. Conclusion

Our observation is to our knowledge the first reporting the association of Melkersson - Rosenthal Syndrome and Behçet's disease. Behçet's disease should therefore be mentioned in patients with MRS, particularly in countries where this vasculitis is frequently responded and if the patient has other systemic symptoms. In our case, the good evolution under colchicine and the absence of recurrences after treatment reinforce this hypothesis.

References

[1]   | Melkersson E. Ett fall av recidiverande facialispares i samband med angioneurotiskt ödem. Hygiea. 1928; 90: 737-41.
[2]   | Rosenthal C. Klinisch-erbbiologischer beitrag zur konstitutionspathologie Gemeinsames auftreten von (rezidivierender familiärer) facialislähmung, angio-neurotischem gesichtsödem und lingua plicata in arthritismus-familien. Z Neurol Psychiatr. 1931; 131: 475-501.
[3]   | Bakshi SS. Melkersson-Rosenthal Syndrome. J Allergy Clin Immunol Pract. 2017; 5(2): 471-472.
[4]   | Wehl G, Rauchenzauner M. A Systematic Review of the Literature of the Three Related Disease Entities Cheilitis Granulomatosa, Orofacial Granulomatosis and Melkersson - Rosenthal Syndrome. Curr Pediatr Rev. 2018; 14(3):196-203.
[5]   | Basman A, Gumusok M, Degerli S, Kaya M, Toraman Alkurt M. Melkersson-rosenthal syndrome: a case report. J Istanb Univ Fac Dent. 2017; 51(1): 42-45.
[6]   | Bouomrani S, Lamloum I, Mesfar R. Melkersson-Rosenthal syndrome associated to Hashimoto’s thyroiditis. Clin Sci Res Rep. 2020; 3: 1-2.
[7]   | Braunsteiner H, Sailer S. Vascular purpura as prodromal symptom of the Melkersson-Rosenthal syndrome. Wien Z Inn Med. 1964; 45: 509-12.
[8]   | Elias MK, Mateen FJ, Weiler CR. The Melkersson-Rosenthal syndrome: a retrospective study of biopsied cases. J Neurol. 2013; 260(1): 138-43.
[9]   | Halevy S, Shalom G, Trattner A, Bodner L. Melkersson-Rosenthal syndrome: a possible association with psoriasis. J Am Acad Dermatol. 2012; 67(4): 795-6.
[10]   | Haaramo A, Kolho KL, Pitkäranta A, Kanerva M. A 30-year follow-up study of patients with Melkersson-Rosenthal syndrome shows an association to inflammatory bowel disease. Ann Med. 2019; 51(2): 149-155.
[11]   | Cabrera-Gómez JA, Echazabal-Santana N, Real-González Y, Romero García K, Junior Sobrinho M, Gil Ocaña MA, et al. Hereditary Melkersson-Rosenthal syndrome and multiple sclerosis. Mult Scler. 2005; 11(3): 364-6.
[12]   | Binkley GW. Melkersson-Rosenthal syndrome with associated features of chronic discoid lupus erythematosus and sarcoidosis. Arch Dermatol. 1964; 90: 111.
[13]   | Bulur I, Onder M. Behcet disease: New aspects. Clin Dermatol. 2017; 35(5): 421-434.
[14]   | Williams DS. Behcet's Disease. J Insur Med. 2019; 48(1): 103-105.
[15]   | Ates O, Yoruk O. Unilateral anterior uveitis in Melkersson-Rosenthal syndrome: a case report. J Int Med Res. 2006; 34(4): 428-32.
[16]   | Liu R, Yu S. Melkersson-Rosenthal syndrome: a review of seven patients. J Clin Neurosci. 2013; 20(7): 993-5.