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 journalArticle

Abstract

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
Volume37
Issue number4
DOIs
StatePublished - Apr 7 2015

Fingerprint

Lip
Hyperplasia
Steroids
Helicobacter pylori
Skin
Lymphoma
Immunoglobulin Heavy Chain Genes
Minor Salivary Glands
Borrelia
Polymerase Chain Reaction
Gene Rearrangement
Palatine Tonsil
Appendix
Prednisone
Paraffin
Immunoglobulin M
Flow Cytometry
Differential Diagnosis
B-Lymphocytes
Immunoglobulin G

Keywords

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

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Dermatology

Cite this

Steroid-responsive atypical marginal zone hyperplasia of the lip in a child. / Sedrak, Michael; Muthukumar, Akila; Elghetany, M. Tarek; Qian, You-Wen.

In: American Journal of Dermatopathology, Vol. 37, No. 4, 07.04.2015, p. e45-e48.

Research output: Contribution to journalArticle

@article{b69fe9c873794d988c2881bae277d97c,
title = "Steroid-responsive atypical marginal zone hyperplasia of the lip in a child",
abstract = "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.",
keywords = "atypical marginal zone hyperplasia, lip lesion, lymphoma, marginal zone lymphoma, skin",
author = "Michael Sedrak and Akila Muthukumar and Elghetany, {M. Tarek} and You-Wen Qian",
year = "2015",
month = "4",
day = "7",
doi = "10.1097/DAD.0000000000000089",
language = "English (US)",
volume = "37",
pages = "e45--e48",
journal = "American Journal of Dermatopathology",
issn = "0193-1091",
publisher = "Lippincott Williams and Wilkins",
number = "4",

}

TY - JOUR

T1 - Steroid-responsive atypical marginal zone hyperplasia of the lip in a child

AU - Sedrak, Michael

AU - Muthukumar, Akila

AU - Elghetany, M. Tarek

AU - Qian, You-Wen

PY - 2015/4/7

Y1 - 2015/4/7

N2 - 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.

AB - 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.

KW - atypical marginal zone hyperplasia

KW - lip lesion

KW - lymphoma

KW - marginal zone lymphoma

KW - skin

UR - http://www.scopus.com/inward/record.url?scp=84926287480&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84926287480&partnerID=8YFLogxK

U2 - 10.1097/DAD.0000000000000089

DO - 10.1097/DAD.0000000000000089

M3 - Article

C2 - 25794373

AN - SCOPUS:84926287480

VL - 37

SP - e45-e48

JO - American Journal of Dermatopathology

JF - American Journal of Dermatopathology

SN - 0193-1091

IS - 4

ER -