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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 6  |  Issue : 6  |  Page : 42-44

Fuchs syndrome: due to coinfection of mycoplasma and herpes simplex virus


1 Associate Professor, Department of Pediatrics and Intensive Care, INHS Asvini, Mumbai, Maharashtra, India
2 Resident, Department of Pediatrics, INHS Asvini, Mumbai, Maharashtra, India
3 Professor and Head, Department of Pediatrics, INHS Asvini, Mumbai, Maharashtra, India
4 Professor, Department of Pediatrics, INHS Asvini, Mumbai, Maharashtra, India

Date of Submission19-Aug-2019
Date of Acceptance13-Nov-2019
Date of Web Publication20-Dec-2019

Correspondence Address:
Bal Mukund
Associate Professor, Department of Pediatrics and Intensive Care, INHS Asvini, Mumbai 400005, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.21304/2019.0606.00546

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  Abstract 


Erythema multiforme minor is also termed as Fuchs Syndrome.We report a case of Fuchs syndrome due to co-infection with Mycoplasma Pneumoniae and Herpes simplex virus which has rarely been reported in literature.

Keywords: Erythema Multiforme, Mycoplasma Pneumoniae, Fuchs Syndrome


How to cite this article:
Mukund B, Augustya P, Adhikari K M, Yadav A K. Fuchs syndrome: due to coinfection of mycoplasma and herpes simplex virus. J Pediatr Crit Care 2019;6:42-4

How to cite this URL:
Mukund B, Augustya P, Adhikari K M, Yadav A K. Fuchs syndrome: due to coinfection of mycoplasma and herpes simplex virus. J Pediatr Crit Care [serial online] 2019 [cited 2022 Dec 6];6:42-4. Available from: http://www.jpcc.org.in/text.asp?2019/6/6/42/279477


  Introduction Top


Erythema multiforme (EM) is an acute immunological dermatological disease which presents with bullous symptoms. The etiology can be diverse. Causative factors may include Sulpha drugs, Ampicillin, Amoxicillin, Penicillin and Non-steroidal anti-inflammatory drugs (NSAIDs) and Herpes infection.[1],[2],[3] Mycoplasma have been implicated as trigger organism especially in children, whereas vaccination is the commonest trigger in infants.[4] It has been classified as EM minor involving only mucosa and EM major involving more severe mucocutaneous involvements.[5] When oral tissues are involved, airway management can be of critical importance. EM minor is also termed as Fuchs Syndrome.[6],[7] The name Fuchs syndrome has also been used in Ophthalmology for a chronic inflammatory disease of the eye usually affecting young adults with unilateral anterior uveitis. However in present description it pertains to its dermatological description and also called atypical Steven Johnson Syndrome. These lesions are predominant in males.[8] EM patients should be managed in PICU as likelihood for complications are high. In hospital management is required to provide fluid and electrolyte homeostasis, diagnostic testing and multispecialty consultation with Ophthalmologist, Dermatologist and Intensivists to manage optimally. We report a case of Fuchs syndrome due to co-infection with Mycoplasma Pneumoniae and Herpes simplex virus which has rarely been reported in literature.


  Case Report Top


Six year male child, case of West Syndrome on multiple anti-convulsants came with history of low grade fever, cough and tachypnoea of 2 days duration. There was history of decreased feeding and excessive somnolence and lethargy of 1 day duration. The child was on sodium valproate, topiramate, clonazepam and lamotrigine for West Syndrome and under Pediatric Neurology follow up. Parents denied any history of taking NSAIDs or accidental ingestion of any drug. On triage, child was in hypotensive shock with respiratory distress hence managed with fluid resuscitation, Oxygen by non-rebreathing mask (NRM) and Inj Ceftriaxone and Metronidazole to cover for aspiration pneumonia. On further detailed examination child had erythematous erosive lesions in buccal mucosa [Figure 1] and developed similar lesion on second day around anus [Figure 2]. Child continued to have fever, encephalopathy and mucosal lesions. Hence a serology for Mycoplasma and Herpes and a spinal fluid (CSF) analysis were sent. Considering most likely etiological agents for Fuchs Syndrome, serology for Mycoplasma and Herpes Simplex virus were sent. Empirically Azithromycin was added in view of suspected Mycoplasma induced Fuchs Syndrome and Lamotrigine was withhold. Investigation for tropical infections and H1N1 were negative. His blood for electrolytes, blood sugar and initial blood / urine culture were sterile. CSF was normal and meningitis/ encephalitis panel by PCR array for viruses and bacterial infection (total 14 organisms) were negative. Child continued to be febrile on 5th day, hence Inj Imipenem was started for hospital acquired infections and Inj Acyclovir was added after Anti HSV IgM positive report. Child had developed BSI which was covered adequately by imipenem and became afebrile after 13 days. His serum for Herpes simplex virus- IgM was 1.223 (Positive > 1.1) by Enzyme Immune Assay and anti-mycoplasma IgM titer was 1:382. Ophthalmic examination was normal that was validated by the Ophthalmologist,mucosal lesions healed gradually and child was discharged on D21 of admission on anti-convulscents and supplements with further follow up. There was no recurrence of similar symptoms up to 3 months follow-up.
Figure 1: Oral mucosal lesion

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Figure 2: Anal mucosal lesion

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  Discussion Top


Erythema multiforme (EM) is an acute dermatological disease characterized by bullous eruptions in skin and mucosal involvements and are thought to have auto-immune origin. Among infection, Mycoplasma and herpes simplex virus infections have been linked to EM. The EM have been traditionally classified as EM major and EM minor. EM major is described as severe mucocutaneous forms (outbreaks in ≥2 different mucosae and skin together) whereas EM minor has been described as benign skin irritation and generally one mucosal involvement.[8],[9] They are more common in males and peaks between 20 and 30 years of age. The lesions are most frequently seen in oral mucosa, however occasionally involves other orifices like genitals, ocular and anal mucosae. A particular form of EM without causing skin involvements was described in literature in Germany as Fuchs Syndrome. They generally have oral mucosal lesion characterized by erythematous, erosive and ulcerative lesions which have sometime yellowish coating. In contrast to EM major, prognosis in this variant EM is very good.

In our case, child had no skin involvement, hence a diagnosis of Fuchs Syndrome was considered. Diagnosis in mainly based on history and clinical examination. Occasionally skin biopsy may be required in atypical skin lesions to differentiate EM from other conditions like Urticaria Multiforme and other autoimmune blistering diseases.[4] Leukemia or drug induced neutropenic syndrome can present with mucosal lesion but initial workup for these were normal and hence were excluded. Finally our case having no skin lesions fulfilled the criteria for EM minor or Fuchs Syndrome.

The pathophysiological mechanisms of Fuchs syndrome have been enigmatic. A hyper-response of cytotoxic T-lymphocyte is postulated causing apoptosis of keratinocytes. In adult most cases are triggered by drugs, but in children the triggering factor is usually infections like Mycoplasma and Herpes Simplex virus.[10] Rarely do they have recurrent post herpetic EM outbreaks necessitating either acyclovir or Valacyclovir for long term.[3],[11] Severe oral lesions are painful and may lead to malnutrition, fluid or electrolyte imbalances and ocular lesion if there are any, can cause symblepheritis, keratoconjunctivitis sicca, ectropion or even corneal opacities.

Management of EM is supportive and severe or recurrent cases require corticosteroids or antimicrobial drugs.[4] There are few randomized trials for treatments of EM and they lack broad based consensus opinion. Treatment with steroids in EM is controversial as they may lead to infectious complications and delayed healing.[1] However many cases with oral mucosal involvements have shown good response to steroid as they even hastens resolution of pain and shown improved oral intake. Even in many patients with EM minor due to mycoplasma, treatment is not initiated in absence of pulmonary symptoms by many clinicians.[8] EM minor is generally managed with supportive treatment only.[4] Usually macrolide antibiotics or Doxycycline is given for Mycoplasma infection by some. In recalcitrant cases and EM major, intravenous immunoglobulins have also been recommended and tried.[10] Other supportive therapy includes hydration, anti-septic or analgesic gargles. In cases of eye involvement combined topical preparations of antibacterial substances and/ or corticosteroids are recommended.

The case illustrates the classical variety of EM minor with only mucosal involvement. In a recent review of all published literature done in 2019 of pediatric EM representing 580 cases, 104 (17.9%) had herpes simplex infection, 91 (15.7%) had Mycoplasma infection, 140 (24.1%) drug related and 89 (15.3%) had other triggers such as vaccination. Long term sequelae are rare (1.3%).[4] Co-infection of both HSV and MP is unique feature in this case. Possibility of EM Minor should always be considered while approaching any child with mucosal erosive lesions.

Source of Funding: Nil

Conflicts of Interest: Nil



 
  References Top

1.
Al-Johani KA, Fedele S, Porter SR. Erythema multiforme and related disorders. Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics. 2007;103(5):642-54.  Back to cited text no. 1
    
2.
Canavan TN, Mathes EF, Frieden I, Shinkai K. Mycoplasma pneumoniae-induced rash and mucositis as a syndrome distinct from Stevens-Johnson syndrome and erythema multiforme: a systematic review. Journal of the American Academy of Dermatology. 2015;72(2):239-45.  Back to cited text no. 2
    
3.
Staikuniene J, Staneviciute J. Long-term valacyclovir treatment and immune modulation for Herpes-associated erythema multiforme. Central-European journal of immunology. 2015;40(3):387-90.  Back to cited text no. 3
    
4.
Zoghaib S, Kechichian E, Souaid K, Soutou B, Helou J, Tomb R. Triggers, clinical manifestations, and management of pediatric erythema multiforme: A systematic review. Journal of the American Academy of Dermatology. 2019.  Back to cited text no. 4
    
5.
Huff JC, Weston WL, Tonnesen MG. Erythema multiforme: a critical review of characteristics, diagnostic criteria, and causes. Journal of the American Academy of Dermatology. 1983;8(6):763-75.  Back to cited text no. 5
    
6.
Li K, Haber RM. Stevens-Johnson syndrome without skin lesions (Fuchs syndrome): a literature review of adult cases with Mycoplasma cause. Archives of dermatology. 2012;148(8):963-4.  Back to cited text no. 6
    
7.
Havliza K, Jakob A, Rompel R. Erythema multiforme majus (Fuchs syndrome) associated with Mycoplasma pneumoniae infection in two patients. Journal der Deutschen Dermatologischen Gesellschaft - Journal of the German Society of Dermatology : JDDG. 2009;7(5):445-8.  Back to cited text no. 7
    
8.
Gossart R, Malthiery E, Aguilar F, Torres JH, Fauroux MA. Fuchs Syndrome: Medical Treatment of 1 Case and Literature Review. Case reports in dermatology. 2017;9(1):114-20.  Back to cited text no. 8
    
9.
Ingen-Housz-Oro S, Ortonne N, Chosidow O. The diagnosis is in the rings. BMJ. 2017;359:j3817.  Back to cited text no. 9
    
10.
Meyer Sauteur PM, Gansser-Kalin U, Lautenschlager S, Goetschel P. Fuchs syndrome associated with Mycoplasma pneumoniae (Stevens-Johnson syndrome without skin lesions). Pediatric dermatology. 2011;28(4):474-6.  Back to cited text no. 10
    
11.
Tatnall FM, Schofield JK, Leigh IM. A double-blind, placebo-controlled trial of continuous acyclovir therapy in recurrent erythema multiforme. The British journal of dermatology. 1995;132(2):267-70.  Back to cited text no. 11
    


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