Systemic Therapy for Lichen Sclerosus: A Systematic Review.

BACKGROUND: Lichen sclerosus (LS) is a chronic, inflammatory process affecting predominantly anogenital skin, with extragenital involvement in up to 20% of cases. The mainstay of therapy for anogenital LS is topical immunosuppression. However, in treatment-refractory cases, severe, or hypertrophic disease, systemic modalities may be used. Currently, there are no guidelines for systemic therapy in LS. OBJECTIVE: This study aimed to provide a review of the current literature on use of systemic therapies for LS, including demographic and clinical features of LS, as well as reported outcomes. METHODS: A primary literature search was conducted using the following databases: PubMed, Ovid, Scopus, and Web of Science, from the year the journal was published until June 2022. RESULTS: Ultimately, 71 studies consisting of 392 patients were included. Of these, 65% (n = 254) had anogenital disease, 9% (n = 36) had extragenital disease, and 19% (n = 73) had both anogenital and extragenital disease, and in 7% (n = 29) of cases, location was not specified. The most frequent therapies, stratified by total cases, included oral retinoids (n = 227), methotrexate (n = 59), hydroxychloroquine (n = 36), and systemic steroids (prednisone, methylprednisolone, prednisolone, oral triamcinolone, and other systemic steroids) (n = 60). Overall, 76% (n = 194) of anogenital, 94% (n = 34) of extragenital, and 81% (n = 59) of patients with both anogenital and extragenital involvement were reported to have clinical or symptomatic improvement. CONCLUSION: Overall, we found many therapies that have been used with reported success for extragenital and genital LS. However, future studies are needed to better define treatment outcomes and directly compare efficacy of different therapies for LS.

Dermatomyositis with Eosinophils.

Dermatomyositis is an idiopathic inflammatory myopathy that often presents with symmetric proximal skeletal muscle weakness and characteristic skin findings. Typical skin biopsy findings include vacuolar changes of the basal layer, increased dermal mucin, and a predominantly lymphocytic infiltrate. We report a case of dermatomyositis presenting as intensely pruritic papules and plaques, with initial histopathology being atypical of dermatomyositis due to the presence of eosinophils. The initial biopsy demonstrated a superficial dermatitis with eosinophils, initially thought to represent a drug eruption. A second biopsy of the same cutaneous manifestation was performed at a later time given high clinical suspicion for dermatomyositis and demonstrated a more classic vacuolar interface dermatitis with increased mucin and an absence of eosinophils. Notably, increased pruritus was specifically associated with the lesion that demonstrated tissue eosinophilia. The case illustrates the importance of considering tissue eosinophilia in the histologic presentation of dermatomyositis.

Eosinophils in Traditionally Noneosinophil-Rich Dermatoses.

ABSTRACT: The presence or absence of tissue eosinophilia has previously aided in the diagnosis of inflammatory skin conditions. However, recent studies have elucidated the presence of eosinophils in traditionally eosinophil-poor inflammatory skin diseases, such as dermatomyositis (DM), psoriasis, and lichen sclerosus (LS). This systematic review of the literature explores previous studies of tissue eosinophilia in skin biopsies of dermatoses that are believed to be classically poor in eosinophil. We identified 26 studies, the majority of which were retrospective reviews. The percent of specimens with increased eosinophils in psoriasis ranged from 18%-73%, pityriasis rubra pilaris (PRP) 22%-63%, LS 29%-53%, DM 15%-44%, morphea 8%-45%, hypertrophic lichen planus (LP) 0%-21%, and oral LP 0%-4%. These reports of tissue eosinophilia in reputed eosinophil-poor dermatologic conditions present a diagnostic pitfall and suggest that tissue eosinophilia itself should not be used to rule out a diagnosis of one of these conditions.

Acquired vulvar lymphangioma: risk factors, disease associations, and management considerations: a systematic review.

Acquired vulvar lymphangioma (AVL) is not well-characterized. Diagnosis is delayed and the condition is often refractory to therapy. Objective: The objective of this study was to provide a systematic review of AVL including risk factors, disease associations, and management options. Methods: A primary literature search was conducted using 3 databases: PubMed, CINAHL, and OVID, from all years to 2022. Results: In total, 78 publications with 133 patients (48 ± 17 years) were included. Most studies were case reports/series. The most common disease association was prior malignancy (70 patients, 53% of cases) and inflammatory bowel disease (6 patients, 5% of cases). The most common malignancy was cervical cancer (57 patients, 43% of cases). Most patients had prior radiation or surgery, with 36% (n = 48) treated with radiation, 30% (n = 40) with lymph node dissection, and 27% (n = 36) with surgical resection. Common presenting symptoms included discharge/oozing, pain, and pruritus. Most patients underwent surgical treatment for AVL with 39% treated with excision, 12% with laser therapy (the majority used CO2), and 11% with medical therapies. Most patients had failed prior therapies and there was a diagnostic delay. Limitations: Retrospective nature. Most studies were limited to case reports and case series, with interstudy variability and result heterogeneity. Conclusion: AVL is an underrecognized entity and should be considered in patients with a history of malignancy or radiation to the urogenital area. Treatment should include multidisciplinary care and address underlying lymphatic changes, manage any existing inflammatory conditions, and utilize skin-directed therapies and barrier agents while addressing symptoms of pruritus and pain. Prospective studies are needed to further characterize AVL and develop treatment guidelines.

Epithelioma Cuniculatum (Plantar Verrucous Carcinoma): A Systematic Review of Treatment Options.

Epithelioma cuniculatum (EC) is a subtype of verrucous carcinoma (VC) that affects the feet. Treatment involves complete tumor removal by wide local excision (WLE) or Mohs micrographic surgery (MMS). Extensive local destruction may require amputation. We sought to compare reported treatment methods for EC and determine their efficacy by assessing for tumor recurrence and treatment-associated complications. A systematic review of the literature spanning multiple databases was performed. To date, surgical excision is recommended as the standard of care for treatment of EC, with amputation considered in more advanced cases. Mohs micrographic surgery appears to be a promising treatment modality for EC and may have lower recurrence rates than WLE but requires further investigation.