Please login or create an account. If you do not have access to this content, you will be shown a 30 second preview and licensing options.

  • Presentation

Skinternal Medicine: An Update on Cutaneous Manifestations of Systemic Disease

Description

In a presentation on cutaneous manifestations of systemic diseases, a detailed case of a 32-year-old woman with lupus and rheumatoid arthritis was discussed. She had experienced severe skin reactions after resuming medications like hydroxychloroquine, which had previously caused rashes unless she was on prednisone. Upon her hospital admission, she exhibited diffuse rash, fever, pleuritic chest pain, and significant laboratory abnormalities including elevated liver enzymes and low platelets. Following a workup, her skin condition showed full-thickness necrosis, indicative of toxic epidermal necrolysis (TEN), but further evaluations suggested it was an acute cutaneous lupus erythematosus (ACLE). The talk explored associations between hydroxychloroquine and severe cutaneous adverse reactions, particularly highlighting that certain autoimmune diseases like dermatomyositis might increase these risks. It also discussed diagnostic considerations, histopathological findings, and treatment responses, reinforcing that rapid recovery is often seen in TEN-like lupus with systemic corticosteroids. The importance of a thorough investigation when encountering similar cases was emphasized, alongside a reminder that lupus may manifest through unexpected skin reactions, serving as a vital clue for diagnosis.

View more

Conclusions

  • Hydroxychloroquine is strongly associated with severe cutaneous adverse reactions like acute generalized exanthematous pustulosis and Stevens-Johnson syndrome/toxic epidermal necrolysis.
  • Patients with dermatomyositis exhibited a higher risk of cutaneous adverse reactions to hydroxychloroquine compared to those with cutaneous lupus.
  • Starting hydroxychloroquine at a lower dose can reduce the risk of adverse cutaneous reactions in patients with lupus.
  • A high C4 level may predict reactions to hydroxychloroquine.
  • The patient's TEN-like acute cutaneous lupus erythematosus required aggressive treatment but showed rapid improvement with corticosteroids.
  • Histopathology and direct immunofluorescence (DIF) can help distinguish TEN-like acute cutaneous lupus erythematosus from drug-induced reactions and other conditions like Rowell syndrome.
  • TEN-like acute cutaneous lupus erythematosus is rare but can occur in patients with lupus and may be misclassified as toxic epidermal necrolysis due to similar presentations.
  • Akabane AL, Smith GP. Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus: report of a case and review of the literature. J Am Acad Dermatol. 2021;85(4):1046-1047.
  • Arch Dermatol. 2002;138(9):1231-1233.
  • J Am Acad Dermatol. 2019;81(3):859-860.
  • Lupus. 2004;13:941-950.
  • Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus: report of a case and review of the literature. Lupus. 2013;22:839-846.
  • Epidermal necrolysis as the presenting manifestation of pediatric lupus. Pediatric Dermatology. 2020;37:1119-1124.
  • Ziemer M, Kardaun SH, Liszt Y, Mockenhaupt M. Stevens-Johnson syndrome and toxic epidermal necrolysis in patients with lupus erythematosus: a descriptive study of 17 cases from a national registry and review of the literature.