Document Detail

Melkersson-Rosenthal syndrome in a patient with tubercular panuveitis.
Jump to Full Text
MedLine Citation:
PMID:  20029155     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We report a rare presentation of Melkersson-Rosenthal syndrome in a patient with tubercular panuveitis. A 45-year-old male being treated with antitubercular therapy for tubercular panuveitis presented with unilateral, non-pitting right upper eyelid edema. Excision biopsy showed granulomatous inflammation involving the lymphatics. Immunohistochemistry confirmed the presence of histiocytes around the lymphatics.
Authors:
Kalpana Babu; Prashanta V Gundannavar; Vidya Satish; Venkatesh C Prabhakaran
Related Documents :
12907215 - Evaluation of tumour markers in patients with burning mouth syndrome.
10971485 - Schnitzler's syndrome: no evidence for autoimmune basis in two patients.
8456855 - Craniofacial, limb, and abdominal anomalies in a distinct syndrome: relation to the spe...
10415465 - Optic disc elevation in down syndrome.
3167415 - Psychiatric presentation of third ventricular colloid cyst. a case report.
637755 - Tolosa-hunt syndrome. arteriographic evidence of improvement in carotid narrowing.
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Indian journal of ophthalmology     Volume:  58     ISSN:  1998-3689     ISO Abbreviation:  Indian J Ophthalmol     Publication Date:    2010 Jan-Feb
Date Detail:
Created Date:  2009-12-23     Completed Date:  2010-03-18     Revised Date:  2010-09-28    
Medline Journal Info:
Nlm Unique ID:  0405376     Medline TA:  Indian J Ophthalmol     Country:  India    
Other Details:
Languages:  eng     Pagination:  78-80     Citation Subset:  IM    
Affiliation:
Vittala International Institute of Ophthalmology & Prabha Eye clinic and Research center, Bangalore, India.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Antitubercular Agents / therapeutic use
Biopsy
Diagnosis, Differential
Humans
Male
Melkersson-Rosenthal Syndrome / diagnosis,  etiology*
Middle Aged
Panuveitis / complications*,  diagnosis,  drug therapy
Tomography, X-Ray Computed
Tuberculosis, Ocular / complications*,  diagnosis,  drug therapy
Chemical
Reg. No./Substance:
0/Antitubercular Agents
Comments/Corrections

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Full Text
Journal Information
Journal ID (nlm-ta): Indian J Ophthalmol
Journal ID (publisher-id): IJO
ISSN: 0301-4738
ISSN: 1998-3689
Publisher: Medknow Publications, India
Article Information
? Indian Journal of Ophthalmology
open-access:
Received Day: 03 Month: 11 Year: 2008
Accepted Day: 06 Month: 1 Year: 2009
Print publication date: Season: Jan?Feb Year: 2010
Volume: 58 Issue: 1
First Page: 78 Last Page: 80
Publisher Id: IJO-58-78
PubMed Id: 20029155
DOI: 10.4103/0301-4738.58481

Melkersson-Rosenthal syndrome in a patient with tubercular panuveitis
Kalpana BabuAF0001
Prashanta V GundannavarAF0001
Vidya SatishAF0001
Venkatesh C PrabhakaranAF0001
Vittala International Institute of Ophthalmology & Prabha Eye clinic and Research center, Bangalore, India
Correspondence: Correspondence to: Dr. Kalpana Babu, Prabha Eye clinic & Research centre, 504, 40th cross, Jayanagar 8th block, Bangalore - 560 070, India. E-mail: kb_7375@yahoo.co.in

Melkersson-Rosenthal syndrome (MRS) is characterized by recurrent, relapsing orofacial edema, facial palsy and a fissured tongue.[1, 2] It is a rare, non caseating granulomatous disease of unknown cause. It was described by Melkersson as a syndrome of recurrent facial paralysis and edema. Rosenthal added the third feature, furrowing of the tongue. The furrowed tongue is the least common finding. The complete triad is very rare and monosymptomatic or sequential involvement is more common.[1, 2] Histopathology showing the presence of perilymphatic granuloma, lymphangiectasia and granulomatous lymphangitis is diagnostic of the above syndrome.[3] Occasional reports of isolated painless, non-pitting and unilateral eyelid edema due to MRS has been described.[1, 2, 4] We report a case of isolated eyelid edema due to MRS, confirmed histopathologically, in a patient with tubercular panuveitis.


Case Report

A 45-year-old male was being treated for tubercular panuveitis in both eyes since one year with four-drug regimen of antitubercular therapy (ATT) which included isoniazid, rifampicin, pyrazinamide, ethambutol for two months followed by isoniazid, rifampicin and pyrazinamide for ten months. ATT was given for a possible tubercular etiology on the basis of a strongly positive Mantoux test (25-mm induration), a calcified nodule on chest X-ray and demonstration of Mycobacterium tuberculosis (MTb) genome by polymerase chain reaction (PCR) on the vitreous sample. He had no recurrences and there was complete resolution of ocular inflammation following treatment with ATT. During his follow-up visits, he gave a history of intermittent right upper eyelid swelling since the last four months, which was more prominent during the day. Past history revealed a history of recurrent left-sided facial edema five years previously for which no investigations were done at that point of time. He did not have any facial nerve palsy or furrowed tongue at presentation. On ocular examination, a firm, non-tender and non-pitting edema involving the entire right upper eyelid was seen. [Fig. 1] His best corrected visual acuity was 20/20 in both eyes. Slit-lamp examination was unremarkable. Fundus examination showed areas of healed retinal vasculitis and multifocal choroiditis. Thyroid function tests, renal function tests and computed tomography of the cranium and orbits were normal. A right upper eyelid biopsy was done. Histopathology showed many empty, ectatic, thin-walled vessels consistent with lymphatics [Fig. 2]. An inflammatory infiltrate consisting of lymphocytes and histiocytes was seen surrounding these vessels. One area of section showed intralymphatic histiocytic infiltration [Fig. 3]. Immunohistochemistry was positive for CD68 marker confirming the presence of histiocytes [Fig. 4]. Quantitative PCR (qPCR) on the paraffin-embedded skin biopsy specimen was negative for MTb. The excision of a part of eyelid skin improved the patient's appearance and the patient preferred to be under observation [Fig. 5].


Discussion

In our case, the complete clinical triad of MRS was not present. Although eyelid edema due to MRS is an unusual entity, the clinical findings may be difficult to differentiate from blepharochalasis or thyroid-associated ophthalmopathy. There is an isolated report of a possible association with tuberculosis.[3] However, in our case, the paraffin-embedded skin biopsy specimen did not demonstrate the MTb antigen by qPCR. The association in our case with tubercular panuveitis may be casual. Treatment is challenging and includes intralesional usage of corticosteroids and or oral treatment with dapsone, clofazimine, tetracycline, clindamycin and methotrexate without any reproducible improvement. A combination of surgical debulking and intralesional steroid therapy has been advocated to improve cosmesis. Because of its rarity, this syndrome is usually ignored and misdiagnosed. MRS may be considered in all cases of isolated eyelid edema and a diagnostic incision biopsy may be performed to establish a diagnosis.


The authors would like to acknowledge Dr.Narsing A Rao, MD, Doheny Eye Institute, Los Angeles for his help in doing the qPCR on the paraffin embedded skin biopsy.


References
1. Shapiro M,Peters S,Spinelli HM. Melkersson-Rosenthal syndrome in the periocular area: a review of the literature and case reportAnn Plast SurgYear: 2003506448 Review. 12783021
2. Cockerham KP,Hidayat AA,Cockerham GC,Depper MH,Sorensen S,Cytryn AS,et al. Melkersson-Rosenthal syndrome: new clinicopathologic findings in 4 casesArch OphthalmolYear: 20001182273210676788
3. Apaydin R,Bahadir S,Kaklikkaya N,Bilen N,Bayramg?rler D. Possible role of Mycobacterium tuberculosis complex in Melkersson-Rosenthal syndrome demonstrated with Gen Probe amplified Mycobacterium tuberculosis direct testAustralas J DermatolYear: 20044594915068454
4. Akarsu C,Atasoy P,Erdo?an S,Ko?ak M. Bilateral upper eyelid edema in Melkersson-Rosenthal syndromeOphthal Plast Reconstr SurgYear: 2005212435

Figures

[Figure ID: F0001]
Figure 1 

Photograph of the face showing right upper eyelid edema



[Figure ID: F0002]
Figure 2 

Photomicrograph showing the thin-walled lymphatic vessels with lymphocytes infiltration (Hematoxylin and Eosin, ?20)



[Figure ID: F0003]
Figure 3 

Photomicrograph showing intralymphatic histiocytic infiltration (Hematoxylin and Eosin, ?20)



[Figure ID: F0004]
Figure 4 

Photomicrograph showing positivity to CD68 marker confirming the presence of histiocytes (Immunohistochemistry, ?20)



[Figure ID: F0005]
Figure 5 

Postoperative photograph of the face showing improved cosmesis



Article Categories:
  • Brief Communications

Keywords: Isolated eyelid edema, Melkersson-Rosenthal syndrome, tubercular panuveitis.

Previous Document:  Intravitreal anti-vascular endothelial growth factor agents as an adjunct in the management of Coats...
Next Document:  Resolution of macular edema in Coats' disease with intravitreal bevacizumab.