Darier sign in a teenager with primary cutaneous marginal zone lymphoma

Pediatr Dermatol. 2021 Mar;38(2):494-496. doi: 10.1111/pde.14488. Epub 2020 Dec 18.

Abstract

Primary cutaneous marginal zone lymphoma (PCMZL) is a low-grade B-cell lymphoma that arises in the skin. An adolescent male presented with dermal nodules on the arms, legs, and back with a positive Darier sign, ultimately diagnosed as PCMZL. The nodules demonstrated a partial response to doxycycline in the setting of prior Lyme disease followed by a complete response to rituximab.

Keywords: malignant neoplasms; systemic therapy; urticaria.

MeSH terms

  • Adolescent
  • Humans
  • Lymphoma, B-Cell, Marginal Zone* / diagnosis
  • Lymphoma, B-Cell, Marginal Zone* / drug therapy
  • Male
  • Neoplasms, Connective Tissue*
  • Rituximab / therapeutic use
  • Skin
  • Skin Neoplasms* / diagnosis
  • Skin Neoplasms* / drug therapy

Substances

  • Rituximab