Stevens-Johnson Syndrome, Mucositis, or Something Else?

Abstract

ILLUSTRATIVE CASE EIA is a recurring section of Hospital Pediatrics where expert pediatric hospitalists give their interpretation of the recent evidence in reference to common clinical questions encountered in their daily practice.Stevens-Johnson Syndrome, Mucositis, or Something Else?Stevens-Johnson syndrome (SJS) has been described in the literature as a combination of erythematous blistering skin lesions covering <10% of body surface area and ≥1 mucous membrane erosion.1 SJS is usually triggered by a medication or infection. Infectious causes are more common in children, most notably herpes simplex virus (HSV) and Mycoplasma pneumoniae. Mucous membrane erosions without signifi cant skin involvement have been classifi ed as "atypical SJS" and mucositis.2 Case: An 18-year-old high school senior presented to his physician with a 2-week history of sore throat and cough, 1-week history of fever, and 1-day history of swollen lips and mucosal ulcerations in his mouth accompanied by pain with swallowing. He was an otherwise healthy adolescent male, with the exception of a PICU admission for anaphylaxis without obvious trigger 8 years ago and attention-defi cit/hyperactivity disorder for which he is taking methylphenidate. The treating physician ordered a chest radiograph that demonstrated streaky infi ltrates in the right lower lobe and sent serology samples for Mycoplasma, immunoglobulin G (IgG) antibodies for HSV type 1 and 2, and HIV testing. The patient was started empirically on azithromycin and acyclovir, with lidocaine viscous for his mouth sores. The following day he developed conjunctival erythema, urethral ulceration, and continued to have poor oral intake; he was therefore admitted to the inpatient pediatric ward for intravenous hydration, pain control, and further observation. Question: Does this patient have SJS?Discussion: There is disagreement in the literature on the exact defi nition of SJS. As fi rst described by Stevens and Johnson in 1922, a patient must have a generalized skin eruption in conjunction with mucosal involvement to meet the defi nition.3 This patient never had a generalized skin eruption and therefore does not meet the classic criteria. He did, however, develop 1 targetoid lesion with a central blister on his glans penis, as well as meatal ulceration (Fig 1). In addition, the patient had bilateral nonexudative conjunctival erythema and oropharyngeal ulcerations seen externally on his lips (Fig 2).We characterized this illness as mucositis secondary to atypical pneumonia, not meeting the classic diagnosis of SJS due to lack of skin lesions. According to Schalock and Dinulos,4 patients with disease limited to the mucous membranes are not at risk for widespread skin involvement and often recover faster than children with skin involvement, making diagnosis important for prognosis. AUTHORS

Mucositis Secondary to Chlamydia pneumoniae Infection: Expanding the Mycoplasma pneumoniae–Induced Rash and Mucositis Concept

“…There is no clear evidence to establish the best therapeutic approach to these cases; most affected individuals received antibiotics or immunosuppressive agents . Based on other reports and our experience, we propose that systemic steroids might help control the hypersensitivity reaction and thereby accelerate healing of mucocutaneous involvement . In our opinion, antibiotic therapy should be reserved for microbiologically confirmed cases and for individuals with signs of pneumonia.…”

“…A search in the PubMed and MEDLINE databases for an association between mucositis, EM, or SJS and C. pneumoniae produced 20 reported cases, which are summarized in Table . Drawing on similarities with MIRM, we have included a chlamydia ‐induced rash and mucositis (CIRM) column, where we classify these cases in terms of extensive spread (severe) or cases in which no skin lesions were present (without rash).…”

Chlamydia pneumonia – induced mucositis

“…Steroids have been demonstrated to be beneficial in some patients in reducing the severity of mucositis. 3 The role of intravenous immunoglobulin (IVIG) is still under debate, and it carries the risk of transfusion reactions and anaphylaxis. In a review by Meyer et al, 4 among 32 confirmed cases of MPAM, 31% showed improvement with corticosteroids and IVIG was used in 9% of cases.…”

Mucosal exfoliation as a selective reaction to etoricoxib

“…Sometimes, the mucous membrane erosions are the only manifestation of a drug reaction, without significant skin involvement. This has been classified as “atypical SJS” or mucositis . In these clinical entities, the duration from the drug intake to the first onset of a symptom may range between few hours and 45 days, and the recovery time may be very long because re‐epithelization of the skin or mucosa could be achieved within several weeks .…”

“…This has been classified as "atypical SJS" or mucositis. 23 In these clinical entities, the duration from the drug intake to the first onset of a symptom may range between few hours and 45 days, and the recovery time may be very long because re-epithelization of the skin or mucosa could be achieved within several weeks. 22,24 This can happen even after a single dose of drug, as reported, for example, for ibuprofen 25 and etoricoxib 19 and as in our case.…”