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Other chronic CLE

  • DLE
  • Panniculitis
  • Profundus
  • Tumidus
  • Chillblains

  • Fixed annular or urticarial erythematous edematous plaques
  • No epidermal changes, face > trunk
  • Similar morphology to Jessner’s lymphocytic infiltrate
  • Low prevalence of SLE, no serology abnormalities, low Ig deposition in skin
Histology
  • Prominent dermal mucin
  • Lymphocytic infiltrate in upper and lower dermis ± subcutis + periadnexal ± dermal edema
  • No epidermal changes
Differential diagnosis
  • Lymphocytic infiltrate of Jessner
  • Polymorphous light eruption
  • Pseudolymphoma

  • Risk of SLE 5-10%
  • Indurated and / or depressed plaques on face, upper arms, upper trunk, breasts, buttocks and thighs
  • Lupus profundus if overlap with DLE (seen in 1/3)
Histology
  • ± Vacuolar alteration of basal layer, ± apoptotic keratinocytes, ± epidermal atrophy,
  • Lymphocyte-rich lobular panniculitis
  • ± Dermal mucin
  • Major DDX is panniculitis-like CTCL

  • Risk of SLE ~20%
  • Diagnosed if presence of Pernio lesions (red, dusky purple papules, and plaques on extremities) + clinical or lab features of CLE or SLE
  • Aggravated / induced by cold especially humid cold
  • Can develop features of DLE
  • Familial chilblain: heterozygous mutation TREX1 or SAMHD1
  • ADAR1, IFIH1 and RHASEH2A/B/C mutation → Aicardi-Goutières syndrome with chilblain lesions