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 Table of Contents  
Year : 2021  |  Volume : 8  |  Issue : 6  |  Page : 291-294

Stevens–Johnson syndrome complicated by pneumomediastinum and cerebral venous sinus thrombosis in a 10-year-old girl: A case Report

1 Department of Pediatrics, Ramkrishna CARE Hospital, Raipur, Chhattisgarh, India
2 Department of Rheumatology, Ramkrishna CARE Hospital, Raipur, Chhattisgarh, India
3 Division of Cornea, Shri Aurobindo Nethralaya Hospital, Raipur, Chhattisgarh, India
4 Department of Dermatology, Ramkrishna CARE Hospital, Raipur, Chhattisgarh, India
5 Department of Radiodiagnosis, Ramkrishna CARE Hospital, Raipur, Chhattisgarh, India

Date of Submission17-Jul-2021
Date of Decision06-Sep-2021
Date of Acceptance26-Sep-2021
Date of Web Publication19-Nov-2021

Correspondence Address:
Dr. Pawan Jain
Department of Pediatrics, Ramakrishna CARE Hospital, Aurobindo Enclave, Pachpedi Naka, Dhamtari Road, NH – 43, Raipur - 492 001, Chhattisgarh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpcc.jpcc_57_21

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Stevens–Johnson syndrome (SJS) is a life-threatening condition with severe mucocutaneous reaction in response to an offending medication. We report a case of SJS complicated with pneumomediastinum and cerebral sinus venous thrombosis in a girl. These two complications occurring simultaneously in a pediatric patient with SJS are hitherto unreported. Laboratory workup pointed underlying connective tissue disease in her. She received supportive care along with steroids, antimicrobials, and anticoagulant under supervision of a multidisciplinary team. She recovered well at discharge and is on follow-up. Clinical vigilance identifies rare but important complications of SJS early, mitigating severe morbidity and mortality.

Keywords: Cerebral venous sinus thrombosis, pneumomediastinum, Stevens–Johnson syndrome

How to cite this article:
Jain P, Singh R, Jain S, Saxena A, Verma U, Bhojasiya VK. Stevens–Johnson syndrome complicated by pneumomediastinum and cerebral venous sinus thrombosis in a 10-year-old girl: A case Report. J Pediatr Crit Care 2021;8:291-4

How to cite this URL:
Jain P, Singh R, Jain S, Saxena A, Verma U, Bhojasiya VK. Stevens–Johnson syndrome complicated by pneumomediastinum and cerebral venous sinus thrombosis in a 10-year-old girl: A case Report. J Pediatr Crit Care [serial online] 2021 [cited 2022 Jan 22];8:291-4. Available from: http://www.jpcc.org.in/text.asp?2021/8/6/291/330726

  Introduction Top

Stevens–Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) spectrum of manifestations are rare, potentially life-threatening, severe mucocutaneous adverse reactions characterized by epidermal detachment from the underlying tissue, erosion of mucosae, and severe constitutional symptoms.[1],[2] Detachment in SJS involves <10% body surface area (BSA) along with two or more mucosal surfaces. Known complications of SJS are pneumonitis, myocarditis, hepatitis, enterocolitis, polyarthritis, renal urological, and ocular (corneal ulceration and panophthalmitis).[2] We report two uncommon complications – pneumomediastinum and cerebral venous sinus thrombosis (CVST) in a 10-year-old girl affected by SJS. These two complications occurring simultaneously in a pediatric patient affected by SJS are hitherto unreported.

  Case Report Top

A 10 year old girl presented with complaints of fever, cough, facial puffiness for 10 days with generalized rashes and redness of the mouth and lips for 7 days. Her illness started with fever and cough, and for that, she received tablet ciprofloxacin and cetirizine from a general practitioner. Two days following consumption of medication, she developed lesions on the skin, mouth, and eyes and was shown at a multispecialty hospital. She was hospitalized and treated on lines of SJS. During hospital stay, she developed hemodynamic instability, requiring oxygen and inotropic support for suspected SJS-associated myocarditis. Although she responded to initial treatment, her general condition subsequently failed to improve with further deterioration in skin and mouth lesions. She experienced extreme photophobia, foreign body sensation, and blurring of vision in both eyes. She was referred to our center after 1 week of stay at a referring hospital. The patient or her relatives did not have a history of similar complaints.

On examination, generalized macular rashes with scattered areas of peeling, bilateral conjunctival injection, oral mucosal erythema, coated tongue, ulcer over the palate, hemorrhagic crusting, and edema of the lips were noted [Figure 1]. She had fever (100.2°F) with chills, heart rate 98/min, respiratory rate 26/min, blood pressure (BP) 97/65 mmHg on inotropic support (dopamine infusion at 5 mcg/kg/min), and oxygen saturation of 98% on oxygen support of 2 L/min via nasal prong. On chest examination, mildly increased work of breathing, crepts over precordial and sternal area on palpation, and fine basal crepts were noted bilaterally on auscultation. Central nervous system examination was normal with no focal neurological deficits. In the presence of typical lesions with BSA involvement <10%, a diagnosis of SJS was considered.
Figure 1: Erythematous macules over the face, eyelids, neck, and trunk with desquamation on the face and trunk, hemorrhagic crusting, and edema of the lips

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Appropriate fluid resuscitation and supportive treatment were given, and relevant laboratory investigations were sent. High-resolution computed tomography (HRCT) chest was done in the emergency department per institutional guidelines during COVID-19 pandemic. Then, the patient was shifted to pediatric intensive care unit (PICU). Intravenous (IV) antimicrobials cefepime (150 mg/kg/day), clindamycin (30 mg/kg/day), and fluconazole (6 mg/kg/min) were empirically started as sepsis is a leading cause of mortality in SJS. HRCT chest showed air leak with pneumomediastinum as a prominent finding [Figure 2]. Antigen test and RT-PCR for SARS-CoV-2 sent on arrival were negative.
Figure 2: High-resolution computed tomography chest showing pneumomediastinum, minimal pulmonary interstitial emphysema, and subcutaneous emphysema

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Two-dimensional echocardiography showed moderate cardiac dysfunction without pericardial effusion. Antinuclear antibody sent as part of workup was strongly positive (163.73 units). This result in the presence of palatal ulcer suggested a possible diagnosis of systemic lupus erythematosus (SLE), prompting rheumatologist to advise SLE antibodies profile. Anti-Ro and anti-La antibodies were strongly positive (149.66 and 104.5 units, respectively) though ds-DNA was negative in our patient. Anti-histone and Anti-Smith antibodies could not be sent due to financial constraints.

A multidisciplinary team comprising pediatric intensivist, ophthalmologist, dermatologist, rheumatologist, plastic surgeon, and ENT surgeon provided treatment. In PICU, supportive treatment with invasive BP monitoring was continued. IV dexamethasone (0.2 mg/kg/day) was commenced as per team recommendation. Topical mupirocin, steroid, and chlorhexidine gauze dressing were applied for cutaneous lesions. For oral lesions, triamcinolone 0.1% mixed with chlorhexidine gluconate was applied twice daily, and benzocaine 20%w/w gel to relieve pain and promote healing. Normal saline-soaked gauze was applied to avoid drying, irritation, and exposure keratitis. A course of moxifloxacin and prednisolone eye drops were given to avoid ocular complications

Oxygen and inotropic support were gradually weaned and stopped on day 3. Enteral feed was started via nasogastric tube on day 4. The patient complained of left-sided headache on day 8 of hospital stay. Restricted eye movements on the left side were observed though range of movement and strength were preserved in all extremities. Magnetic resonance (MR) imaging of the brain with MR venography (MRV) and MR angiography was performed. CVST involving the posterior half of the superior sagittal sinus, left transverse, sigmoid sinus, and part of the left internal jugular vein was detected on MRV images [Figure 3]a and [Figure 3]b. IV infusion of unfractionated heparin was commenced which was discontinued after 5 days switching over to oral anticoagulant warfarin with target INR of 2–3 under supervision of a neurologist.
Figure 3: (a) Mid saggital section of Magnetic resonance brain T1W image showing thrombosed superior sagittal sinus(green arrows). (b) Magnetic resonance venography coronal view showing normal right sigmoid sinus (green arrows), with no flow in the left side due to thrombus

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At the time of discharge, there was substantial healing of lesions allowing oral feeds. Discharge medications included oral hydroxychloroquine and prednisolone for incomplete SLE and 6-month plan of warfarin for CVST.

On 4 weeks follow up recurrence of keratitis was observed, necessitating resumption of systemic steroids in tapering doses and addition of azathioprine, as well as addition of 0.05% cyclosporine eye drops. There were no fresh mucocutaneous lesions seen. Target INR of 2–3 was maintained on warfarin therapy.

  Discussion Top

SJS is rare, with a reported incidence of 6.3 per 100,000 hospitalized children/year.[3] The primary trigger for SJS is medications, with infections as the next most common trigger. Medications commonly incriminated in development of SJS/TEN include anticonvulsants (carbamazepine, phenytoin, lamotrigine, etc.), nonsteroidal anti inflammatory drugs, sulfonamide antibiotics, allopurinol, and nevirapine. The mucocutaneous reaction in SJS may start with a prodrome of fever, pharyngitis followed by generalized erythematous macules, and characteristic painful blistering of the skin. Usually, two mucosal membranes, conjunctiva and oral mucosa, are often involved. Mucosal lesions precede skin lesions.[1],[2] SCORTEN is a validated tool computed 24 h after admission and again on day 3 for assessing severity and predicting mortality in adult SJS/TEN patients.[1] It has been adapted for the prognostication in pediatric SJS/TEN patients.[4] SCORTEN could not be applied in our patient due to delayed presentation. Severe mucocutaneous reactions and multiple early and late complications can occur in patients of SJS. Our report describes two potentially life-threatening complications – pneumomediastinum and CVST occurring simultaneously in a pediatric patient affected by SJS.

Patients with SJS may rarely present with air leak syndrome and respiratory failure. It is a very rare, reported complication of SJS.[5] SJS may lead to inflammation and sloughing of airway mucosa and bronchorrhea facilitating formation of mucus plugs having a ball valve effect and alveolar distension. Forceful coughing in our patient might have resulted in alveolar rupture with air leak syndrome having pneumomediastinum as a prominent finding. In our patient, air leak resolved spontaneously with supplemental oxygen administration and pain relief.

CVST per se has not been reported as a complication of SJS. Strongly positive laboratory along with palatal ulcer in a 10-year-old girl made incomplete SLE a strong possibility. Sjogren's syndrome (SS) was also considered differential. Our patient did not meet the preliminary case definition criteria for MIS-C.[6] Reports from Taiwan have mentioned SS as a sequela of SJS/TEN as well as a predisposing cause for CVST.[7],[8] Prednisolone along with hydroxychloroquine was started for likely incomplete SLE.

Withdrawal of culprit drug and intensive supportive care is a cornerstone of management in SJS. A multidisciplinary team including dietician was involved early in the care of the patient, addressing management and nutritional requirements of the child. IV immunoglobulin either as monotherapy or in combination is advised early in SJS,[1],[5] precluding its use in our patient. Immunosuppressants cyclosporine and tumor necrosis factor blockers (etanercept and infliximab) have shown benefit in smaller studies.[9]

To conclude, SJS is a rare, life-threatening disorder fraught with rare but life-threatening complications. Although the usual trigger is culprit medication, connective tissue disease should be actively searched in a young female patient. Early diagnosis and management of common as well as rare complications mitigates severe morbidity and mortality. Follow-up may identify sequelae in a patient of SJS and allows optimum management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's legal guardian has given the consent for images and other clinical information to be reported in the journal. The patient's legal guardian understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Gupta LK, Martin AM, Agarwal N, D'Souza P, Das S, Kumar R, et al. Guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis: An Indian perspective. Indian J Dermatol Venereol Leprol 2016;82:603-25.  Back to cited text no. 1
[PUBMED]  [Full text]  
Joyce JC. Stevens-Johnson syndrome. In. Kliegman RM, St Geme JW 3rd, editors. Nelson Textbook of Pediatrics. 21st ed. New Delhi, India: Elsevier; 2020. p. 3482-3.  Back to cited text no. 2
Antoon JW, Goldman JL, Lee B, Schwartz A. Incidence, outcomes, and resource use in children with Stevens-Johnson syndrome and toxic epidermal necrolysis. Pediatr Dermatol 2018;35:182-7.  Back to cited text no. 3
Sorrell J, Anthony L, Rademaker A, Belknap SM, Callahan S, West DP, et al. Score of toxic epidermal necrosis predicts the outcomes of pediatric epidermal necrolysis. Pediatr Dermatol 2017;34:433-7.  Back to cited text no. 4
Bruce-Hickman D, Jiang X, Thia JJ, Kansal A. Stevens-Johnson syndrome complicated by obstructive uropathy, pneumothorax, and pneumomediastinum: A case report and literature review. Burns Trauma 2019;7:14.  Back to cited text no. 5
World Health Organization. Multisystem Inflammatory Syndrome in Children and Adolescents with COVID-19; May 15, 2020. Available from: https://www.who.int/publications-detail/multisystem-inflammatory-syndrome-in-children-and-adolescents-with covid-19. [Last accessed on 2021 Sep 02].  Back to cited text no. 6
Yang CW, Cho YT, Chen KL, Chen YC, Song HL, Chu CY. Long-term sequelae of Stevens-Johnson syndrome/toxic epidermal necrolysis. Acta Derm Venereol 2016;96:525-9.  Back to cited text no. 7
Lang Y, Zhang W, Wu X, Deng F, Cui L. Sjögren's syndrome with cerebral venous sinus thrombosis: A case report and literature review. Ann Indian Acad Neurol 2020;23:110-2.  Back to cited text no. 8
  [Full text]  
Ramien M, Goldman JL. Pediatric SJS-TEN: Where are we now?. F1000Res. 2020;9:F1000 Faculty Rev-982. Published 2020 Aug 13. doi:10.12688/f1000research.20419.1.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3]


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