Síndroma de Stevens-Johnson e necrólise tóxica epidérmica: um estudo retrospectivo de 15 anos.

Abstract

Stevens-Johnson Syndrome (SJS), Overlap Syndrome (OS) and Toxic Epidermal Necrosis (TEN) are rare medical emergencies associated with a high morbidity and mortality. The literature on the characteristics of these diseases in Portugal is scarce. A retrospective study was conducted based on the clinical notes of the 20 patients admitted to São José Burn Unit in the previous 15 years with the diagnosis of SJS, OS or TEN. Most patients had TEN (65%), followed by OS (25%) and SJS (10%). Average age was 57,1 ± 19,0 years. The mean duration of stay in the Burn Unit was 12,6 ± 7,8 days. Mortality was 50%, being significantly higher than the 16,4% overall mortality in the general Burn Unit population in the same period (p < 0,01). The mean surface area involved was 43,9 ± 28,6 %. Allopurinol was the causal agent most frequently implicated (35%) followed by UV light exposure (15%). Fourteen patients (70%) were treated with steroids in the first days, whereas six patients were treated conservatively (30%). Mortality was inferior in the patients treated with steroids (42,8 vs. 66,7%), even though this difference did not reach statistical significance. Infection rates were not significantly different between the two groups. SCORTEN score proved to be a good predictor of mortality. Further studies are needed to reduce mortality in these diseases.

Toxic Epidermal Necrolysis: The Experience of Coimbra’s Burn Unit

“…20 Increased alveolocapillary permeability with secondary pulmonary edema, polypnea due to pain and aspiration of debris from oropharyngeal and tracheobronchial mucosa detachment can lead to atelectasias, bronchial pneumonia and, in more severe cases, to ARDS (adult respiratory distress syndrome). In agreement with these data and with other studies, [12][13][14] infectious complications, like pneumonia and sepsis, were frequent in our group of patients. Pneumonia was a major cause of morbid- ity during hospitalization and MRSA appeared as the principal microorganism cultured, probably related to the high incidence of this pathogen in skin flora.…”

“…9 In our sample we found a good correlation between predicted and actual mortality which supports the accuracy of SCORTEN in predicting SJS/TEN mortality, as already established in other studies. 12,14,16 The mortality rate in our group of patients was high (47.6%), but still consistent with literature. 3,12,36 Several features present in our sample may contribute to this high mortality rate namely higher proportion of patients with TEN, high mean TBSA involvement and mean age, high percentage of comorbidities and late transfer of the patient to our unit as well as late withdrawal of the culprit drug.…”

“…[13][14][15] TEN can affect people of all ages and all ethnicities, but is recognized to be more frequent at extreme ages. In our group of patients the mean age (55.6 years) was equivalent or slightly higher to other studies, [12][13][14]16 but is important to state that, in opposite to other centers, admissions in our burn unit are restricted to patients older than 12 years old. We have observed a preponderance of cases in the female gender, which is also in agreement with literature 4,17 and recent studies.…”

“…In Portugal, data concerning treatment of SJS and TEN patients in Burn Units are very scarce and the present retrospective study corresponds to the largest series of patients admitted in a Burn Center due to these exfoliating diseases in our country. [10][11][12] Since SJS/TEN are rare conditions, our sample size is limited but similar to other international series published in literature. [13][14][15] TEN can affect people of all ages and all ethnicities, but is recognized to be more frequent at extreme ages.…”

Photodistributed Stevens–Johnson syndrome and toxic epidermal necrolysis: a systematic review and proposal for a new diagnostic classification

“…These cases did not meet inclusion criteria because the studies did not report whether the patient received ultraviolet-B light therapy after starting the causal drug. Three additional cases of SJS/TEN, which reported patients who received methoxsalen with concomitant UVR for the treatment of vitiligo or psoriasis [ 133 ], were excluded for similar reasons.…”