Dermatologica Sinica

: 2019  |  Volume : 37  |  Issue : 1  |  Page : 50--52

Idiopathic lymphoplasmacellular mucositis of the vulva in a patient with partial interferon-γ receptor 1 deficiency

Kuan-Yu Chen1, Tseng-Tong Kuo2, Ya-Ching Chang1, Rosaline Chung-Yee Hui1, Ya-Hui Chuang1,  
1 Department of Dermatology, Chang Gung Memorial Hospital, Taipei; Department of School of Medicine, College of Medicine, Chang Gung University, Taoyuan, Taiwan
2 Department of Pathology, Chang Gung Memorial Hospital, Taipei, Taiwan

Correspondence Address:
Ya-Hui Chuang
Department of Dermatology, Chang Gung Memorial Hospital, 199, Tung-Hwa North Road, Taipei 105


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.

How to cite this article:
Chen KY, Kuo TT, Chang YC, Hui RC, Chuang YH. Idiopathic lymphoplasmacellular mucositis of the vulva in a patient with partial interferon-γ receptor 1 deficiency.Dermatol Sin 2019;37:50-52

How to cite this URL:
Chen KY, Kuo TT, Chang YC, Hui RC, Chuang YH. Idiopathic lymphoplasmacellular mucositis of the vulva in a patient with partial interferon-γ receptor 1 deficiency. Dermatol Sin [serial online] 2019 [cited 2022 Aug 9 ];37:50-52
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Full Text


Idiopathic lymphoplasmacellular mucositis (ILPM) is a unified nomenclature for Zoon-like lesions, suggested by Brix et al. in 2010,[1] with histopathological findings of dense plasma cell infiltrates in the dermis or submucosa. Those occurred on vulva had been termed as Zoon's vulvitis, plasma-cell vulvitis, or vulvitis circumscripta plasmacellularis. ILPM of the vulva is a rare and benign idiopathic chronic inflammatory disease, with <50 cases reported in the literature. It usually presents as well-defined erythematous macules, patches, or plaques.[2] Partial interferon-γ receptor 1 (IFN-γR1) deficiency is a primary immunodeficiency caused by mutations in the IFNGR1 gene.[3] We report a case of ILPM of the vulva with ulcerovegetative appearance occurring in a patient with partial IFN-γR1 deficiency.

 Case Report

A 48-year-old woman, who is a hepatitis B virus (HBV) carrier with partial IFN-γR1 deficiency (Mendelian susceptibility to mycobacterial disease),[3] was referred from Gynecology Department to Dermatology Department because of painful and pruritic vulvar lesions with mucinous discharge for 6 months. Previous biopsy at a gynecology clinic 3 months ago was reported as ulcer with acute and chronic inflammation. Tissue cultures for mycobacteria and other bacteria were negative. Laboratory tests for syphilis, herpes simplex virus (HSV), and human immunodeficiency virus (HIV) infection were also negative. Oral clarithromycin, doxycycline, and occasional use of topical acyclovir cream and topical neomycin ointment were ineffective. During physical examination, we found an extensive vulvar ulcer, involving the right labium majus and minus and partial left labium minus, with elevated granulation tissue and mucinous discharge [Figure 1]a. Owing to the chronic extensive presentation and the profound impact on this patient, we decided to re-perform a vulvar biopsy. Histopathological examination revealed mild parakeratosis, mild irregular acanthosis, focal pseudoepitheliomatous hyperplasia, neutrophilic exocytosis, and diffuse heavy infiltrates of numerous plasma cells with scattered lymphocytes and eosinophils in the submucosa [Figure 1]b1 and submucosa [Figure 1]b2 Immunohistochemical study showed that the plasma cells were positive for IgG [Figure 1]c with scattered IgG4-positive cells and IgA and IgM were negative. Both kappa and lambda light chains were positive. There were no spirochetes found with anti-Treponema pallidum antibody. The pathologic diagnosis was established as ILPM.{Figure 1}

She was treated with oral methylprednisolone (0.5 mg/kg/day), zinc gluconate, and topical dexamethasone oral paste with remarkable improvement of the lesion in size and clinical symptoms in 5 days. Due to her elevated liver enzymes and HBV carrier status, we discontinued oral steroids after a 10-day course and kept her other treatments. Her vulva returned to normal appearance in another 2 weeks [Figure 1]d. However, erosive and painful lesions recurred 1 month later. She required intermittent low-dose oral corticosteroids (0.4 mg/kg/day every other day) to remain free of symptoms, and regular follow-up of liver function was normal.


ILPM of the vulva is an uncommon chronic inflammatory disease of unknown etiology. Hypotheses with chronic irritation (warmth, friction, poor hygiene, trauma),[2] persistent infection,[4],[5] human papillomavirus)[6], or autoimmune disease[7] have been proposed. As described in the English literature, it usually presents as asymptomatic or symptomatic (burning, dyspareunia, pruritus, pain, dysuria, and bleeding) well-defined erythematous macules, patches, or plaques, sometimes with pinpoint purpuric “cayenne pepper spots.”[2],[8] Differential diagnosis includes infection (candidiasis, herpes, syphilis, chancroid, lymphogranuloma venereum, HIV infection, tuberculosis), trauma (factitial lesions), hypersensitivity (allergic contact dermatitis, fixed drug eruption), immune-mediated dermatoses (lichen planus, pemphigus, Crohn's disease, systemic lupus erythematosus, Behcet disease), and neoplasms (extramammary Paget disease, erythroplasia of Queyrat, intraepithelial vulvar neoplasia, squamous cell carcinoma, and plasmacytoma).[2],[5],[9],[10] Histopathological examination is crucial in making the diagnosis. It is characterized by a heavy infiltrate of plasma cells in the submucosa with scattered lymphocytes and neutrophils.[1] In the case of our patient, immunohistochemistry study was also done to exclude other plasma cell diseases, including IgG4-related disease, syphilis, and plasma cell neoplasm.

Various treatment options have been reported,[9] including topical agents (mid-to-high-potency steroids, calcineurin inhibitors,[11] imiquimod cream,[4],[6],[12],[13] antibiotics, and misoprostol)[7], intralesional agents (steroids, interferon-α,[5] and platelet-rich plasma)[14], systemic therapy (antibiotics, steroids), destruction of the tissue (liquid nitrogen, CO2 laser,[15] and electrocoagulation), surgical excision,[8],[16] and radiation therapy. None of the treatments showed consistent effectiveness.

Deficiency of the IFNGR gene interferes the transformation of naive CD4 T-cells to Th1 cells.[17],[18] Patients with partial IFN-γR1 deficiency are then prone to systemic infection with mycobacteria,[19]Salmonella,[19],[20] and also viruses, including herpes virus (HSV, varicella zoster virus, and cytomegalovirus), respiratory syncytial virus, and parainfluenza virus.[21],[22]

To the best of our knowledge, our patient was the first reported ILPM case with partial IFN-γR1 deficiency. The ulcerovegetative lesion seen in our case is very different from the typical erythematous macules, patches, or plaques found in ILPM. Similar appearance could only be found in another patient reported by dos Reis et al. in 2013.[10] Interestingly, the two patients shared similar clinical characteristics and courses. Both of them had immunodeficiency related to impaired function of CD4-T cells, our patient had partial IFN-γR1 deficiency (primary immunodeficiency) and dos Reis et al.'s patient had HIV infection (acquired immunodeficiency) under stable highly active antiretroviral therapy for 3 years. They were similar in ages (48 and 52 years) and the time from onset to confirmed diagnosis was both 6 months. Both patients had received biopsy with the diagnosis of nonspecific inflammation originally and treated with topical and oral antibiotics without clinical improvement. After correct diagnosis, both patients received topical and oral steroids with remarkable improvement. dos Reis et al.'s patient received topical clobetasol propionate ointment 0.05% three times a day and oral betamethasone 6 mg per day. Ulcer healed in ten weeks, and she remained disease-free during a 13-month follow-up.


ILPM should be considered in the differential diagnosis of chronic vulvar erosive/ulcerative lesions. Immunodeficiency might be a contributory factor of the ulcerovegetative appearance, but it might also be a coincidence. The number of cases might be underestimated. The awareness of histopathologic features is essential for establishing the correct diagnosis and management of ILPM.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


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