Toxic Epidermal Necrolysis-Like Cutaneous Lupus in Pediatric Patients: A Case Series and Review

Abstract

Bullous eruptions in patients with underlying systemic lupus erythematosus (LE) can mimic toxic-epidermal necrolysis (TEN), a rapidly progressive mucocutaneous reaction usually associated with medication use. Differentiating between classic drug-induced TEN and TEN-like cutaneous LE is important but difficult. We report a series of 3 patients with pediatric systemic LE who were admitted with severe worsening of skin disease resembling TEN. However, the initial photo-distribution of the eruption, subacute progression, limited mucosal involvement, mild systemic symptoms, supportive biopsy and laboratory results, and lack of culprit drugs was more suggestive of a TEN-like cutaneous LE. These patients recovered with various systemic immunosuppressive medications including methylprednisolone, intravenous immunoglobulin, and plasmapheresis. Our cases are rare and demonstrate key clinical and histologic features of TEN-like cutaneous LE in young patients and the importance of differentiating this entity from drug-induced TEN.

<p>Vesiculobullous diseases in relation to lupus erythematosus</p>

“…In all subclasses of LE-specific vesiculobullous disease, immunopathology of lesional skin from direct immunofluorescence (DIF) may show coarse, granular, continuous deposition of multiple immunoglobulins and/or complement, eg, IgG, IgM, IgA (rare), and C3 along the basement-membrane zone. The reported sensitivity of DIF examination is 58%–93%; therefore, negative DIF does not rule out this condition 14,22,30,31. These findings have also been shown in other connective tissue diseases, eg, dermatomyositis, mixed connective-tissue diseases, and systemic sclerosis 30.…”

Epidermal necrolysis as the presenting manifestation of pediatric lupus

“…Few of them were also treated with plasmapheresis and immunosuppressants like cyclophosphamide, mycophenolate mofetil, and azathioprine (Table 1). 17,21 Among 13 children with TEN‐like lupus, 10 (76%) recovered, and the mortality rate was 23% (3/13), higher than the general mortality of childhood lupus (~10%) 8,22‐24 . A high mortality in children with TEN‐like lupus might be due to late diagnosis, delay in treatment, and associated severe systemic manifestations and infections (Table 1).…”

“…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.…”

“…Few of them were also treated with plasmapheresis and immunosuppressants like cyclophosphamide, mycophenolate mofetil, and azathioprine (Table 1). 17,21 Among 13 children with TEN-like lupus, 10…”

Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus in a patient with progressive systemic sclerosis

“…The differentiation between TEN and TEN-like CLE can be distinguished by a history of drugs used, clinical presentation, serology, histopathology and DIF studies. [2][3][4][5][6][7] Most patients with TEN-like CLE have no history of medications and have a clinical background of SLE. Cutaneous lesions are usually a gradual progression of the blistering eruptions that predominate on the photo distribution with minimal or limited mucosal involvement.…”