A 40-year-old woman, Acehnese civil servant, presented to the Dr Zaionel Abidin Hospital in Banda Aceh Indonesia with shortness of breath. One week before the admission, the patient's skin was peeling; red blotches and rashes emerged all over the body. The patient also reported that their eyes were itchy. The patient was coughing up phlegm for three days before admission. There was no fever and no documented allergy history.
The patient had the same complaints in 2011 and 2013 and was diagnosed with allergic contact dermatitis and Steven Johnson Syndrome (SJS). However, the patient was unable to recall the drug(s) that was associated with it. The patient had type 2 diabetes mellitus, and tuberculosis and was routinely treated with insulin Novorapid 12-12-12, Levemir 0-0-0-12 and tuberculosis drugs. The patient's condition worsened since the patient took tuberculosis drugs for one month.
On physical examination, eyelid edema, bulbar conjunctiva, and tarsal superior and inferior hyperemia were observed; the mouth was excoriated and fissured. The genital area was within normal limits. On pulmonary examination, there was a decreased tactile fremitus, decreased breath sound and ronchi over the left lung suggesting pleural effusion. Resonant percussion and auscultation were vesicular in the right lung field. On the dermatological status, macules, erythematous plaques, multiple brownish crusts, and nummular-size plaques were observed on regio of facialis, superior and inferior thoracic, and superior and inferior extremities with epidermolysis > 90% of body surface area, and Nikolsky sign (+) ( Figure 1). The SCORTEN score was used to calculate the prognostic score and yielded a score of 2 with a possible mortality rate of 12.1%.
The blood test showed hemoglobin 13.6 g/dL, platelets 302.000/mm 3, leukocytes increased by 16.000/mm 3. Kidney and liver function, as well as blood sugar levels within normal limits. Blood gas analysis showed pH 7.497 mmHg, pCO 2 29.00 mmHg, pO 2 61 mmHg, bicarbonate 22.7 mmHg, total CO 2 23.6 mmHg, BE 0.3 mmol/L, and oxygen saturation 93.5%.
Histopathological examination showed mild acanthosis, vacuolar changes, and scattered apoptotic keratinocytes in the epidermis, consistent with TEN. The dermis has a perivascular infiltrate consisting of lymphocytes, eosinophils, and neutrophils. There were no subcorneal spongiform and/or intraepidermal pustules.
Based on the physical examination and histopathology test result, the patient was diagnosed to have toxic epidermal necrolysis. The tuberculosis drug was discontinued because it was suspected the drug was the cause. Furthermore, the patient was taking oral prednisone and loratadine. After consultation to Department of Dermato-Venereology, the therapies were replaced by methylprednisolone injection 62.5 mg, oral mebhydrolin napadisylate 50 mg and combination of salicylic acid 3% and desoximethasone 0.25%, and vaseline album. Because there was no improvement observed, the patient was planned to undergo plasmapheresis with 5% albumin using the prismaflex machine. On the fourth day of plasmapheresis, the patient was given desoximethasone ointment as additional therapy. Methylprednisolone started to be tapped into 31.25 mg per day, fluconazole drip therapy200 mg/day and sun protection factor (SPF) 50 PA+ sunscreen was added on the fifth day.
After a week of therapy, the skin lesions improved, and the itchy skin complaints decreased. Purplish red macules with hypopigmented macules in the middle begin to diminish. Methylprednisolone injection therapy was replaced by oral treatment ( Figure 2). Re-epithelialization was observed after three days, and the patient was discharged on day eight.