Erosive Lichen Planus Treated With Tofacitinib: A Case Series of 23 Patients.The Australasian journal of dermatology • December 11, 2024
Vera Miao, Miranda Branyiczky, Rebecca Saunderson, Gayle Fischer
Erosive lichen planus (ELP) is a chronic inflammatory dermatosis manifesting as painful erosions and ulcers that can affect the oral, anogenital, oesophageal, and other mucosal sites. Erosive vulvovaginal lichen planus (EVLP) causes chronic symptoms and scarring, which lead to significantly diminished sexual function and quality of life (QoL) [1]. EVLP may be very difficult to treat, with up to 40% of cases not responding to first-line topical corticosteroid therapy and requiring systemic agents [2]. The varying efficacy and side effect profiles of traditional immunosuppressive agents such as prednisolone, methotrexate, mycophenolate mofetil, azathioprine, cyclosporine, and hydroxychloroquine highlight the need for more targeted therapies. Several molecular and clinical studies have evidenced the Janus kinase-signal transducer and activator of transcription (JAK–STAT) pathway as playing a crucial role in ELP pathogenesis [3]. Tofacitinib, a second-generation inhibitor of JAK1 and JAK3, has demonstrated promising responses in case reports of patients with ELP [4-7]. We aimed to determine whether tofacitinib can confer a favourable response in a larger patient group and present this case series.
Twenty-three adult women with EVLP, including six with concurrent refractory oral ELP and one with oral and oesophageal ELP, treated with oral tofacitinib 5 mg twice daily, were identified from the patient database of a vulvovaginal sub-specialty clinic. The mean age at diagnosis was 48.4 (range 20–69) years and the mean follow-up duration was 5.7 (range 2–9) years. In keeping with previously published data, histopathology is unreliable, and only 10 women had biopsy features suggestive of EVLP [8]. The remaining 13 women met previously described diagnostic criteria [8, 9]. The mean duration of disease before commencement of tofacitinib was 9.1 (range 3–34) years, during which various topical and systemic therapies were trialled with limited efficacy (Table 1).
Adult Vulval Psoriasis: Clinical Presentation, Management and Impact on Quality of Life.The Australasian Journal Of Dermatology • The Australasian Journal Of Dermatology
Michelle Wu, Gayle Fischer
Objective: To explore the clinical presentation, management and impact on quality of life in women with vulval psoriasis.
Methods: A retrospective, single-centre cohort study of women was conducted at a large dermatology practice from January 2016 to January 2024. Sequential Vulval Quality of Life Index scores and patient data were systematically collected and recorded in an online patient database. Treatment regimens were individualised and titrated to clinical response.
Results: The study included a total of 350 patients with vulval psoriasis over an eight-year period. 13.1% of patients required systemic treatment solely for vulval disease. The median VQLI score improved from 18.0 ± 9.4 at baseline to 9.7 ± 7.6 at the end of follow-up (p < 0.0001). All domains showed statistically significant improvements except for 'Sexual Function'. The domains with the greatest improvement were 'Future Health Concerns' (69.2%, p < 0.001), 'Feelings and Emotions' (63.4%, p < 0.001) 'Symptoms' (58.6%, p < 0.001) and 'Activities of Daily Living' (56.8%, p < 0.001).
Conclusions: Vulval psoriasis has a substantial impact on quality of life but remains underdiagnosed and undertreated. While treatment can significantly improve outcomes, issues related to sexual function and relationships often persist. Systemic therapy may be required for a subset of patients with vulval-only disease. Routine assessment and targeted management of vulval involvement are crucial to optimising patient well-being.
A Guide to Screening for Autoimmune Diseases in Patients With Vulvar Lichen Sclerosus.The Australasian Journal Of Dermatology • October 16, 2024
Annabel Guttentag, Marlene Wijaya, Gayle Fischer, Angela Lee, Ken Liu, Rebecca Saunderson
Background: The aetiology of vulvar lichen sclerosus (VLS) remains unknown. However, there is evidence that in addition to a genetic predisposition, autoimmunity contributes to the pathogenesis.
Objective: The objective of this study was to determine the prevalence of autoimmune disease and positive autoantibody serology in patients with VLS.
Methods: A VLS database in Sydney, Australia, was retrospectively reviewed. A diagnosis of VLS was required for inclusion in the study. Data collected included demographics, comorbidities including any personal history of autoimmune disease, family history of autoimmune disease, and the results from autoantibody testing. A total of 2243 females with VLS were included in this study.
Results: Autoimmune disease was found in 24.5% and 34.6% of children and adults with VLS, respectively. The most prevalent autoimmune conditions were psoriasis, Hashimoto's thyroiditis, lichen planus, and vitiligo. Antinuclear antibodies were common and found in 31.0% of patients. Thyroid peroxidase and thyroglobulin antibodies were present in 16.1% and 18.9% of cases, respectively. Thyroid function, determined by thyroid stimulating hormone, was abnormal in 8.2% of patients. 5.3% of patients had positive parietal cell antibodies, and 5.9% had low vitamin B12 levels.
Conclusions: This work provides support that VLS is of an autoimmune aetiology, and that there is an association between VLS and autoimmune diseases. The high proportion of patients with an abnormal thyroid test, positive thyroid antibodies, and intrinsic factor and gastric parietal cell antibodies with low vitamin B12 levels, warrants screening for thyroid disease and pernicious anaemia in patients with VLS. Initial autoimmune screening in VLS can be rationalised to TSH, vitamin B12 levels, intrinsic factor and parietal cell antibodies. Thyroid antibodiy testing shouls be performed in hypothyroid patients.
Severe Vulvovaginal Candidiasis Associated With Sodium-Glucose Cotransporter 2 Inhibitor Use in Postmenopausal Women.Journal Of Lower Genital Tract Disease • September 02, 2024
Vera Miao, Marlene Wijaya, Gayle Fischer, Rebecca Saunderson
Objective: The authors present a case series of severe vulvovaginal candidiasis in postmenopausal women using sodium-glucose cotransporter-2 inhibitor (SGLT2i) medications for the management of their diabetes mellitus.
Methods: Twenty-four cases from a private vulvovaginal specialist clinic are described.
Results: All 24 patients were referred with severe and persistent vulvar pruritus, pain, and erythema. Examination findings varied between patients and included erythema, edema, erosions, adherent white discharge, and fissuring, which were extensive and often involved the mons pubis, labia majora, and extended to the perineum and perianal region, mimicking psoriasis and/or irritant dermatitis. The clinical presentation in this postmenopausal group hindered a timely diagnosis, resulting in a delay in appropriate management. Fortunately, all patients improved on oral antifungal treatment, and in those that ceased their SGLT2i medication, there was resolution of the condition.
Conclusions: While candidiasis is reported to occur with SGLT2i, severe genital mycotic infections are not yet a well-recognized adverse effect and may be missed. The presentation in these cases was persistent and severe. Clinicians should have a high index of suspicion in postmenopausal women presenting with vulvar pain, pruritus, and extensive erythema that mimics psoriasis or irritant dermatitis, if they are on SGLT2i therapy.
Understanding and Managing Vulvar Psoriasis in Girls: Findings From a Cohort Study.Pediatric Dermatology • April 26, 2024
Michelle Wu, Gayle Fischer
Objective: Pediatric vulvar psoriasis in girls is under-recognized and under-treated due to its nonspecific clinical appearance. This paper aims to describe the signs and symptoms of pediatric vulvar psoriasis and treatment strategies used by our group.
Methods: A retrospective cohort study was undertaken at a private pediatric dermatology referral practice from January 2016 to December 2022. Clinical data were prospectively collected and recorded in an online patient database. Treatment regimens were individualized and titrated to clinical response.
Results: In 100 girls with vulvar psoriasis, the most common presentation was an erythematous vulvar eruption (97%) which was well-demarcated in 52% of children and appeared as a plaque in one-fourth. The perianal skin was involved in 48% of cases. Extragenital psoriasis was present in 69% of patients. Most children responded to initial induction treatment with moderate-potency topical corticosteroid ointment followed by maintenance with topical tar solution. Systemic treatment was not required for purely vulvar psoriasis compared with 3% of children with extragenital psoriasis.
Conclusions: Vulvar psoriasis in girls presents as a chronic erythematous vulvitis, with perianal involvement in half the cases, but without vaginitis. It is a remitting and relapsing skin condition that requires long-term topical management.
