Stevens–Johnson syndrome in a child with epilepsy with a favourable outcome

INTRODUCTION

The incidence of Stevens–Johnson syndrome (SSD) and severe epidermal necrolysis (TEN) ranges from 0.4 to 5.3 per million, while mortality in these diseases due to the development of septic complications is high, ranging from 16.7% to 44%.^[1-4] The syndrome is usually induced by taking carbamazepines, antibiotics (sulfonamides and vancomycin), allopurinol and other medications and usually occurs 1–4 weeks after their use.^[5,6] Toxic skin lesions can also be caused and/or provoked by viral (herpes, cytomegalovirus and Epstein–Barr) and bacterial infections (rickettsia and mycoplasma). Reactivation of latent infection, especially Herpesviridae, probably plays a key role in the activation of T lymphocytes and eosinophils, in response to medication and exposure to infectious agents themselves, which leads to severe damage to the skin and organs.^[7] Idiopathic SSD is diagnosed in 25% of cases. Genome-wide profiling has shown that the genetic markers for predicting SSD/TEN are the HLA-B*1502 and HLA HLA-A*3101 alleles, which are consistent with immune pathogenesis.^[8]

The disease debuts with flu-like manifestations (fever, weakness, cough, sore throat and arthralgia), rarely vomiting and diarrhoea. The lesion of the skin and mucous membranes develops 4–6 days after infection or taking medications, with localisation on the extremities, face, genitals and mucous membranes. There are oedematous, painfully itchy, clearly delimited, flattened pink-red papules of rounded shape, up to 2–5 cm in diameter, often with a bubble in the centre filled with serous or haemorrhagic contents. After opening large blisters on the skin and mucous membranes, continuous bleeding, painful foci form, lips and gums become swollen, painful, with haemorrhagic crusts. When the eyes are affected, blepharoconjunctivitis and iridocyclitis are observed, bronchiolitis, colitis and proctitis rarely develop. The addition of infection and the development of a septic process, electrolyte disturbances, loss of protein, with massive skin damage, is the cause of fatal outcomes in SSD.

At present, there is no ‘gold standard’ in the treatment of SSD/TEN. The standard scheme includes the use of high doses of systemic glucocorticoids, with the addition of infectious complications of intravenous immunoglobulin, antibacterial drugs. The literature provides data on the successful use of cyclosporine.^[9,10]

CASE REPORT

The girl M., 8 years old, was admitted to the intensive care unit of the hospital in serious condition, complaining of a widespread discharge rash on the body, itchy skin, refusal to eat and drink and swelling of the face.

The baby was born from a third normal pregnancy, weighing 3,860 grams and measuring 54 centimeters. From 3 weeks of life, she received mixtures in her diet, and at an early age, there were manifestations of atopic dermatitis. There was an acute allergic reaction to interferon preparations in the form of a rash. At the age of 7.5 years, the child was diagnosed with epilepsy, with the appointment of valproic acid at a dose of 500–1,000 mg/day. Due to the instability of epilepsy (seizures persisted), lamotrigine was additionally prescribed at 25– 100 mg/day. At the time of the child’s admission, the duration of taking the drugs was 6 and 1 month, respectively. Two weeks before admission, the child suffered acute tonsillitis with a high fever, taking the antibiotic Amoxycillinum + Acidum clavulanicum for 5 days and a non-steroidal anti-inflammatory drug. The infectious syndrome had a wave-like course, with a repeated increase in temperature to 39–40° and the addition of cough syndrome. Infiltration was detected on a chest X-ray, immunoglobulin M antibodies to Mycoplasma pneumoniae were detected, and therefore, the girl was prescribed a new course of antibiotics for the treatment of pneumonia (Amikacin, Josamycinum and Ceftriaxonum) for 10 days. 3 days after the cancellation of the last drugs, an itchy rash appeared, the phenomena of stomatitis, vulvitis and cheilitis, regarded as allergic manifestations, which were partially stopped by the use of antihistamines and glucocorticoid drugs that the child received during the week. However, 2 days after the withdrawal of steroids, there was an increase in skin syndrome, the spread of rashes throughout the body, the appearance of blisters, necrotic changes in the oral cavity, on the lips, vulva, around the anus, conjunctivitis, and therefore, the child was admitted to the intensive care unit in a serious condition with a diagnosis of allergic urticaria.

DISCUSSION

Local status upon admission: face, shoulders and abdomen are covered with a draining papular rash with wavy edges, pronounced itching of the skin. In the area of the shoulders and chest, blisters up to 2–3 cm in diameter are visible, containing a cloudy liquid [Figures 1 and 2].

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Figure 1:

Changes in the skin of the shoulder in a patient with SSD.

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Figure 2:

papules, blisters, and epidermal detachment in a patient with SSD.

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The skin around the eyes is swollen, the sclera are injected, with areas of haemorrhage. The lips are wet with multiple ulcerative changes, the tongue is swollen with ulceration and aphthae on the oral mucosa [Figure 3].

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Figure 3:

Changes in the mucous membranes of the lips and skin of the face.

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There are pains when swallowing. There are cracks, hyperaemia and weeping around the nail plates [Figure 4]. Papular elements are rare on the thighs and shins. Ulcerativenecrotic lesions of the mucous membranes of the genitals (vulvitis). There are no signs of respiratory failure, and blood pressure is 90/70 mmHg. Urinated profusely, urine is light.

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Figure 4:

Single papular elements and erosions on the fingers.

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Blood tests show inflammatory changes in the form of leukocytosis with neutrophilia. Smear imprints on the cells of the pelvis are negative, and the cellular composition of the contents of the bladders is represented by lymphocytes. Antibodies to Epstein–Barr virus and cytomegalovirus has not been detected. Taking into account, the medical history (the presence of an infectious disease at the onset of the disease, the child taking a large number of medications), the acute onset of the disease, the characteristic clinical picture, the diagnosis was made: SSD with lesions of the skin, eyes, respiratory and digestive tract. Concomitant diagnosis: Focal epilepsy.

The girl received an infusion, preventive antibacterial therapy, systemic steroids at a high dose of 7 mg/kg/day, famotidine intravenously with the transition to omeprazolum orally as the condition stabilised, zinc lotions, ointment with bismuth subgallate, solcoseryl and dexamethasonum eye drops applied topically to the skin.

Antiepileptic drugs have been discontinued for the duration of SSD treatment. Against the background of therapy, the condition has a positive dynamic: the elements of the rash have epithelised, there is no itching, pigmented areas on the skin remain and the integrity of the mucous membranes of the mouth and eyes has been restored. The child was discharged after 22 days of treatment in satisfactory condition.

CONCLUSION

The presented clinical case demonstrates the severe course of SSD, probably caused by an infectious process and the use of an unreasonably large number of drugs in connection with polypragmasia. The treatment of pneumonia in the child did not comply with clinical recommendations, using protected penicillins. During the month, the girl received four antibacterial, two antiepileptic drugs, as well as nonsteroidal anti-inflammatory drugs. In children with an allergic background, taking potentially dangerous drugs is a risk factor for severe allergic reactions. It requires knowledge of doctors of any profile about this disease and alertness in connection with the possibility of its occurrence in any patient and high mortality.