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

doi:10.4103/ds.ds_18_18

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 journal › Journal 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 language	English
Pages (from-to)	50-52
Number of pages	3
Journal	Dermatologica Sinica
Volume	37
Issue number	1
DOIs	https://doi.org/10.4103/ds.ds_18_18
State	Published - 01 2019
Externally published	Yes

Bibliographical note

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

Keywords

Idiopathic lymphoplasmacellular mucositis
interferon-receptor 1 deficiency
vulva

Access to Document

10.4103/ds.ds_18_18

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

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.",

keywords = "Idiopathic lymphoplasmacellular mucositis, interferon-receptor 1 deficiency, vulva",

author = "Chen, \{Kuan Yu\} and Kuo, \{Tseng Tong\} and Chang, \{Ya Ching\} and Hui, \{Rosaline Chung Yee\} and Chuang, \{Ya Hui\}",

note = "Publisher Copyright: {\textcopyright} 2019 Dermatologica Sinica | Published by Wolters Kluwer - Medknow.",

year = "2019",

month = jan,

doi = "10.4103/ds.ds\_18\_18",

language = "英语",

volume = "37",

pages = "50--52",

journal = "Dermatologica Sinica",

issn = "1027-8117",

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number = "1",

}

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T1 - Idiopathic Lymphoplasmacellular Mucositis of the Vulva in a Patient with Partial Interferon-γ Receptor 1 Deficiency

AU - Chen, Kuan Yu

AU - Kuo, Tseng Tong

AU - Chang, Ya Ching

AU - Hui, Rosaline Chung Yee

AU - Chuang, Ya Hui

PY - 2019/1

Y1 - 2019/1

N2 - 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.

AB - 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.

KW - Idiopathic lymphoplasmacellular mucositis

KW - interferon-receptor 1 deficiency

KW - vulva

UR - https://www.scopus.com/pages/publications/85152298321

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DO - 10.4103/ds.ds_18_18

M3 - 文章

AN - SCOPUS:85152298321

SN - 1027-8117

VL - 37

SP - 50

EP - 52

JO - Dermatologica Sinica

JF - Dermatologica Sinica

IS - 1

ER -

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

Abstract

Bibliographical note

Keywords

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