Steroid-responsive atypical marginal zone hyperplasia of the lip in a child

Michael Sedrak, Akila Muthukumar, M. Tarek Elghetany, You Wen Qian

Research output: Contribution to journalArticlepeer-review


Atypical marginal zone hyperplasia (AMZH) is a recently described disease entity seen mainly in children. AMZH most commonly affects tonsils and appendices. Cutaneous AMZH is rare. The authors report here a recurrent AMZH in the lip of a 9-year-old child who presented originally with a lip swelling for approximately 3 months. The lip lesion recurred after each incomplete excision for 4 times. Pathologically, the lesion demonstrated marginal zone B-cell hyperplasia with kappa monoclonality by flow cytometry and immunohistochemistry studies. Lymphoepithelial lesions were noted with involvement of minor salivary glands. Polymerase chain reaction for immunoglobulin heavy-chain gene rearrangement has been repeatedly negative. Polymerase chain reaction for Borrelia species DNA was negative on both paraffin-embedded tissue and plasma. Serum antibodies IgG and IgM for Helicobacter Pylori were positive. A diagnosis of AMZH was made. Two courses of anti H. Pylori therapy did not improve the lip lesion, which completely regressed after a course of prednisone therapy. With differential diagnosis of cutaneous marginal zone lymphoma, the case illustrated diagnostic challenges, especially with recurrent lesions. This is the first case of recurrent cutaneous AMZH that has uncharacteristic kappa light-chain restriction. AMZH should be considered in children with mucocutaneous lesions with features of marginal zone lymphoma.

Original languageEnglish (US)
Pages (from-to)e45-e48
JournalAmerican Journal of Dermatopathology
Issue number4
StatePublished - Apr 7 2015


  • atypical marginal zone hyperplasia
  • lip lesion
  • lymphoma
  • marginal zone lymphoma
  • skin

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Dermatology


Dive into the research topics of 'Steroid-responsive atypical marginal zone hyperplasia of the lip in a child'. Together they form a unique fingerprint.

Cite this