Stevens–Johnson syndrome in a juvenile systemic lupus erythematosus patient

Abstract

Stevens-Johnson syndrome (SJS) is a severe and rare immune-mediated cutaneous reaction usually induced by drugs or infections. Few case reports have demonstrated SJS associated with adult systemic lupus erythematosus (SLE), and rarely in juvenile SLE (JSLE) patients. However, to the best of our knowledge the prevalence of this life-threatening cutaneous disease in the pediatric lupus population has not been studied. Therefore, from January 1983 to December 2010, 5508 patients were followed-up at the Pediatric Rheumatology Unit of our University Hospital and 279 (5%) of them met the American College of Rheumatology (ACR) classification criteria for SLE. Only one (0.4%) of our JSLE patients had SJS and was described. This female patient was diagnosed with JSLE at 14 years old. After four years of follow-up, she was hospitalized due to congestive heart failure and renal insufficiency. During hospitalization, the patient developed sepsis with positive blood culture for Stenotrophomonas maltophilia and was treated with vancomycin and meropenem. One week later, she developed septic shock and chest x-ray showed acute widespread pulmonary infiltrate. Antimicrobials were changed to linezolid and trimethoprim-sulfamethoxazole. After four days, the blood culture isolated Staphylococcus aureus resistant to vancomycin, and she presented with erythematous cutaneous lesions involving her face, trunk, and limbs, with evolution in a few hours to diffuse hemorrhagic vesicles and blisters. Epidermal detachment was observed on 5% of the body surface area. Concomitantly, she had conjunctivitis, cheilitis, oral erosions, and hemorrhagic crust on the nasal mucosa. Vulva, vagina, and perianal erosions were also evidenced. The diagnosis of SJS was established and intravenous immunoglobulin was promptly administered. Three days later, she died of pulmonary hemorrhage. The autopsy findings demonstrated generalized infection and widespread subepidermal detachment with necrotic keratinocytes. In conclusion, SJS is a rare and severe vesiculobullous disease in a pediatric lupus population and is probably associated with infections and drug therapy.

Epidermal necrolysis as the presenting manifestation of pediatric lupus

“…All the reported cases had clinical and serological evidence of lupus and histopathological evidence of TEN‐like changes. Only 4 cases including our case had epidermal necrolysis as the presenting manifestation of lupus 18,19,21 . Nine out of 13 children (69%) had evidence of major organ involvement (CNS, renal, and cardiac).…”

“…Our index case, 2020, India Although the TEN-like variant of SLE is regarded as one of the cutaneous criteria in the Systemic Lupus International Collaborating Clinics (SLICC) classification system, 15 there are only a handful of reports on TEN-like lupus in the pediatric population (Table 1). [16][17][18][19][20][21] Including our case, a total of 13 patients of TEN-like lupus have been reported worldwide in the pediatric age group (female-to-male ratio of 12:1). Age at disease onset ranged from 2 to 18 years.…”

Concomitant Disruption of CD4 and CD8 Genes Facilitates the Development of Double Negative αβ TCR+ Peripheral T Cells That Respond Robustly to Staphylococcal Superantigen

“…Nonetheless, chronic activation of DNT cells in HLA-DQ8 transgenic mice with SSAg accelerated the onset of the systemic inflammatory disease in our DKO mice. Given that DNT cells are readily demonstrable in systemic inflammatory diseases such as lupus (20, 52), that there is a significant association between S. aureus carriage and lupus in humans (53-56) and that a lupus-like disease could be induced in DKO mice with SSAg, SSAg may play an important role in the immunopathogenesis of lupus (35). Further studies are warranted to gain an in-depth understanding of the role of DNT cells in autoimmune diseases.…”

Avaliação da influência da exposição à poluição atmosférica sobre o escore de atividade do lúpus eritematoso sistêmico (SLEDAI-2K) em crianças e adolescentes

“…Lúpus eritematoso sistêmico (LES) juvenil (LESJ) é uma doença inflamatória crônica de caráter autoimune e multifatorial, de grande complexidade e amplo espectro clínico, incluindo manifestações em diferentes órgãos e sistemas (Kone-Paut et al, 2007, Silva et al, 2009, Almeida et al, 2009, Deen et al, 2009, Campos et al, 2010, Silva et al 2010, Almeida et al, 2011, Cavalcante et al, 2011a, Cavalcante et al, 2011b, Spadoni et al, 2011, Araújo et al, 2012.…”