Idiopathic Lymphoplasmacellular Mucositis of the Vulva in a Patient with Partial Interferon-γ Receptor 1 Deficiency

Kuan Yu Chen, Tseng Tong Kuo, Ya Ching Chang, Rosaline Chung Yee Hui, Ya Hui Chuang*

*Corresponding author for this work

Research output: Contribution to journalJournal Article peer-review

Abstract

We report a case of idiopathic lymphoplasmacellular mucositis (ILPM) of the vulva in a 48-year-old woman with partial interferon-γ receptor 1 (IFN-γR1) deficiency. The lesion had an unusual ulcerovegetative presentation. Remarkable response was observed with oral and topical steroids in the first 3 weeks. However, the lesion recurred after tapering oral steroids and continuous low-dose oral steroids were required to suppress recurrence. To the best of our knowledge, this is the first case report of ILPM in a patient with partial IFN-γR1 deficiency. ILPM should be included in the differential diagnosis of persistent vulvar ulcerovegetative lesions.

Original languageEnglish
Pages (from-to)50-52
Number of pages3
JournalDermatologica Sinica
Volume37
Issue number1
DOIs
StatePublished - 01 2019
Externally publishedYes

Bibliographical note

Publisher Copyright:
© 2019 Dermatologica Sinica | Published by Wolters Kluwer - Medknow.

Keywords

  • Idiopathic lymphoplasmacellular mucositis
  • interferon-receptor 1 deficiency
  • vulva

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