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Year : 2020  |  Volume : 21  |  Issue : 2  |  Page : 142-144

Mycoplasma pneumonia-associated Fuchs syndrome: Challenging case of a young boy

Department of Skin and VD, Rajindra Hospital and Government Medical College, Patiala, Punjab, India

Date of Submission07-Dec-2019
Date of Decision04-Jan-2020
Date of Acceptance10-Feb-2020
Date of Web Publication01-Apr-2020

Correspondence Address:
Aarushi Kaushik
Room 70, PG Girls Hostel, Government Medical College, Patiala - 147 001, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_122_19

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Stevens–Johnson syndrome without skin involvement has been termed as Fuchs syndrome. In children, infections are the leading causes of SJS, with the most common being Mycoplasma pneumoniae infection. This is a case of a schoolboy who presented with erosions on the lips which were preceded by vesicles. There was an involvement of all four mucosae and an active respiratory tract infection. A detailed clinical examination is needed to rule out the common differential diagnosis. A suspicion of Mycoplasma in such cases can avoid delay in diagnosis and adverse sequelae. The identification of causative agent can be done by PCR or ELISA technique. A prompt treatment to treat the cause and halt the disease process can lead to rapid improvement.

Keywords: Fuchs syndrome, Mycoplasma pneumoniae, Stevens–Johnson syndrome without skin lesion

How to cite this article:
Chopra D, Kaushik A, Kaur L. Mycoplasma pneumonia-associated Fuchs syndrome: Challenging case of a young boy. Indian J Paediatr Dermatol 2020;21:142-4

How to cite this URL:
Chopra D, Kaushik A, Kaur L. Mycoplasma pneumonia-associated Fuchs syndrome: Challenging case of a young boy. Indian J Paediatr Dermatol [serial online] 2020 [cited 2020 Dec 4];21:142-4. Available from: https://www.ijpd.in/text.asp?2020/21/2/142/281727

  Introduction Top

Fuchs syndrome is a rare entity reported when Stevens–Johnson syndrome (SJS) occurs without any skin lesion.[1] It is more common in children and occurs in a background of Mycoplasma pneumoniae-associated respiratory tract infection. However, adult cases have also been reported.

  case Report Top

We present a case of a 12-year-old male who presented in skin emergency with difficulty in swallowing and hypersalivation, secondary to erosions on the lips.

There was a history of cough and fever for the last 7 days, which was not responding to usual antibiotics and antipyretics. There was no history of any drug intake. On examination, the patient was toxic, pulse 115/min, fever 102°F, respiratory rate 30/min. A single left cervical lymph node less than 1 cm was palpable. There was bilateral conjunctival erythema with white discharge. The lips were swollen with confluent erosions. There were profuse salivation and drooling [Figure 1]. The child had difficulty in opening the mouth and swallowing. The oral mucosa showed erosions on the ventral aspect of the tongue and buccal mucosa. The examination of glans penis also showed red erosion with oozing. There was a single 0.5-cm tense bulla on his nose. There was no desquamation of palms and soles. His complete blood count was normal, erythrocyte sedimentation rate was 18 mm/h, and C-reactive protein was positive. The chest X-ray was normal. Tzanck smear did not show any acantholytic cells or multinucleate giant cells. The patient was started on oral syrup acyclovir 200 mg/5 ml, 5 ml every 8 h, but no improvement was seen even after 48 h. Furthermore, ELISA for HSV 1 and 2 was negative. By the 9th day of illness, the patient developed hemorrhagic crusts on the lips and nasal mucosa, and his cough and difficulty in swallowing worsened. The possibility of M. pneumoniae infection leading to SJS without skin involvement was kept. His serum IgM for M. pneumoniae came out to be positive. With the clinical features and positive IgM ELISA for M. pneumoniae, this was diagnosed as a case of Fuchs syndrome secondary to Mycoplasma infection. He was started on azithromycin 500mg/day and cyclosporine 3mg/kg body weight per day. The lesions cleared after 10 days [Figure 2].
Figure 1: Clinical image showing crusting at the nostrils, a single fluid-filled bulla on the nose, hemorrhagic and yellow serocrust on the lips, and profuse salivation

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Figure 2: The patient improved after 10 days of treatment with cyclosporine and azithromycin

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  Discussion Top

In pediatric age group, infections are the leading causes of SJS and M. pneumoniae appears to be the most common agent. Mycoplasma-associated pneumonia presents with acute onset of fever with cough; the radiographic findings may or may not be present. Few days after onset of illness, there was abrupt onset of mucositis predominantly of the oral mucosa.[2] However, when progressing to SJS, it presents with more severe mucosal involvement and less significant skin manifestations. The most commonly involved mucosa is oral mucosa. Lip lesions can present as swelling, vesicles, bleeding, and crusting. Conjunctival lesions are present in two-third of the cases while genital mucosa is involved in three-fourth of the cases.[3] In addition to this, our patient also had nasal crusting, involvement of the pharyngeal mucosa, and profuse salivation. It should be noted that, out of fear of pain, these children avoid swallowing. In such a situation, profuse salivation puts them at risk of aspiration. Skin manifestations, when present, are mild. These include vesiculobullous or maculopapular rash; however, target lesions are found only in half of the cases.[3] Diagnosis should be established with serum IgM ELISA or throat swab for real-time PCR.[4]

Rarely, the patient can present with isolated mucosal involvement without skin lesions, which is described as Fuchs syndrome. It was first described in Germany as “herpes oris conjunctivae.”[1]Mycoplasma-associated Fuchs syndrome has been reported in children and very rarely in adults.[2],[4],[5] This entity has been variably described as atypical SJS[5] or incomplete SJS[6] or erythema multiforme (EM) major without skin lesion.[5] An antibody-mediated action against a specific skin antigen could be a plausible pathogenetic basis for an isolated mucosal involvement, similar to the molecular mimicry concept of Mycoplasma-related Barre´ syndrome.[7]

Respiratory tract infection by M. pneumoniae can present with simple mucositis which responds well to antibiotics alone. However, when the disease is rapidly progressing and not responding to antibiotics, like in our case, we suggest that such cases should be labeled as Fuchs syndrome rather than just mucositis. Prompt treatment with antibiotics such as macrolides (azithromycin or clarithromycin), tetracycline, or fluoroquinolones should be started. An aggressive approach with systemic steroid or cyclosporine or IVIg can halt the disease process and prevent inadvertent sequelae.

  Conclusion Top

In a child with respiratory symptoms, fever, and severe mucositis typical of SJS, the possibility of Fuchs syndrome should be kept. This can easily be confused with primary episodes of herpes, and thus, an ELISA or PCR to isolate M. pneumoniae can help clinch the diagnosis.

  Declaration of Patient Consent Top

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Fuchs E. Herpes iris conjunctivae. Klin Monatsbl Augenheilkd 1876;14:333-51.  Back to cited text no. 1
Schalock PC, Dinulos JG. Mycoplasma pneumoniae-induced Stevens-Johnson syndrome without skin lesions: Fact or fiction? J Am Acad Dermatol 2005;52:312-5.  Back to cited text no. 2
Tay YK, Huff JC, Weston WL. Mycoplasma pneumoniae infection is associated with Stevens-Johnson syndrome, not erythema multiforme (von Hebra). J Am Acad Dermatol 1996;35:757-60.  Back to cited text no. 3
Ravin KA, Rappaport LD, Zuckerbraun NS, Wadowsky RM, Wald ER, Michaels MM. Mycoplasma pneumoniae and atypical Stevens-Johnson syndrome: A case series. Pediatrics 2007;119:e1002-5.  Back to cited text no. 4
Mangal S, Narang T, Saikia UN, Kumaran MS. Fuchs syndrome or erythema multiforme major, uncommon or underdiagnosed? Indian J Dermatol Venereol Leprol 2015;81:403-5.  Back to cited text no. 5
[PUBMED]  [Full text]  
Ramasamy A, Patel C, Conlon C. Incomplete Stevens-Johnson syndrome secondary to atypical pneumonia. BMJ Case Rep 2011;2011:8-10.  Back to cited text no. 6
Kusunoki S, Shiina M, Kanazawa I. Anti-Gal-C antibodies in GBS subsequent to mycoplasma infection: Evidence of molecular mimicry. Neurology 2001;57:736-8.  Back to cited text no. 7


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