Lupus erythematosus profundus

Last Updated: 2023-10-12

Author(s): Anzengruber F., Navarini A.

ICD11: EB51.Y

  • Kaposi, 1875
  • Irgang, 1940

Lupus panniculitis, Kaposi-Irgang syndrome, lupus erythematosus-panniculitis, LEP.

Special form of chronic cutaneous discoid lupus erythematosus.

  • Peak of frequency of first manifestation: 20th-60th year
  • Women:men = 2-4:1

Rarely periorbital oedema as an initial symptom. Mostly symmetrically arranged, sharply demarcated, erythematous, subcutaneous nodules or plaques with very subtle to no scaling. Ulceration and calcification may occur. Scarless healing, sometimes with lipatrophy.

  • S. cutaneous lupus erythematosus
  • Note:
    • ANAs (in 75% ↑)
    • Anti-dsDNA antibodies (rare)

Face, gluteal, upper arms and thighs.

Lobular panniculitis, hyaline necrosis of adipose tissue are characteristic, lymphoid cell infiltrates with formation of germinal centres, mucin deposition, adipose tissue necrosis, "interfacedermatitis", epitheloid cell nodules, oedematous upper corium. Orthohyperkeratosis and follicular keratosis.

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