Vulvar Lichen Sclerosus Clinical Severity Scales and Histopathologic Correlation: A Case Series.

ABSTRACT: Several vulvar lichen sclerosus (VLS) clinical severity scales have recently been proposed. In this prospective case series, we characterized histopathology in the context of clinical severity in 6 treatment-naïve postmenopausal patients with VLS. The Vulvar Quality of Life Index (VQLI) and an adaptation of the 2018 International Society for the Study of Vulvovaginal Disease Delphi consensus VLS severity score were administered. Vulvar skin punch biopsies were obtained to measure inflammatory density, constituent inflammatory cells, thickness of the stratum corneum and other epidermal layers, dermal edema, and dermal sclerosis. Clinicopathologic correlations were assessed. Two cases demonstrated sparse inflammatory densities, 1 case demonstrated patchy and nodular inflammatory density, 1 case demonstrated dense lichenoid inflammatory density, and 2 cases demonstrated dense lichenoid and epitheliotropic inflammatory densities. Those patients who reported severe pruritus demonstrated the greatest lymphocytic inflammatory densities on histopathological examination. Both cases of ulceration or erosion were associated with severe VQLI scores. Severe VQLI scores were also associated with trends for higher average thickness of the epidermal layers and of dermal sclerosis. Altogether, histopathologic grading of biopsy sites may reflect clinical severity in patients with VLS.

Vulvovaginal manifestations in Stevens-Johnson syndrome and toxic epidermal necrolysis: Prevention and treatment.

The prevalence of acute vulvovaginal involvement in toxic epidermal necrolysis can be as high as 70%; up to 28% of female patients will also develop chronic vulvovaginal sequelae. There is little consensus regarding prevention and treatment of the gynecologic sequelae of both Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN). We review acute and chronic sequelae, including erosions, scar formation, chronic skin changes, urethral complications, adenosis, malignant transformation, vulvodynia, and dyspareunia. We provide comprehensive recommendations for acute and long-term vulvovaginal care in adult and pediatric SJS/TEN patients. Treatment should include an ultrapotent topical steroid, followed by a nonirritating barrier cream applied to vulvar and perineal lesions. A steroid should be used intravaginally along with vaginal dilation in all adults (but should be avoided in prepubertal adolescents) with vaginal involvement. Menstrual suppression should be considered in all reproductive age patients until vulvovaginal lesions have healed. Last, referrals for pelvic floor physical therapy and to surgical subspecialties should be offered on a case-by-case basis. This guide summarizes the current available literature combined with expert opinion of both dermatologists and gynecologists who treat a high volume of SJS/TEN patients.

Vulvar diseases: Conditions in adults and children.

The most problematic vulvovaginal conditions are familiar to dermatologists but may exhibit distinct clinical features or medication management because of the anatomic location. The second article in this continuing medical education series focuses on management pearls for treating vulvar diseases. We highlight key conditions, such as lichen sclerosus, erosive lichen planus, and vulvodynia. In addition, we review conditions that dermatologists may be less familiar with, such as plasma cell vulvitis, desquamative inflammatory vaginitis, vulvar aphthae, and low estrogen states. Nearly 1 in 6 women experience undiagnosed and untreated vulvovaginal discomfort at some point in their lives. Physicians who treat vulvar disorders will improve the quality of life of countless women.

Vulvar diseases: Approach to the patient.

Patients with vulvar dermatoses often delay seeking medical treatment because of anxiety and embarrassment. Moreover, women frequently self-treat with various home remedies and see multiple clinicians before presenting to a dermatologist. Despite serving as the primary providers for patients with vulvovaginal symptoms, gynecologists typically receive limited training in the causes and management of these conditions. Dermatologists are experts in the evaluation and management of cutaneous disease and should be the caretakers of all skin, including the genitalia. Vulvar disorders are underrecognized by dermatologists for numerous reasons: inadequate training, lack of comfort with both interview and examination techniques, and unfamiliarity with normal anatomic variations. The first article in this continuing medical education series on vulvar dermatoses reviews the fundamentals, approach, and techniques that can be used to ensure a successful visit for both patient and provider.

Cutaneous extranodal NK/T-cell lymphoma mimicking cellulitis an HIV positive patient without lymphopenia.

We present the case of a 28-year-old male with a history of human immunodeficiency virus (HIV) with a 1-month history of a steadily enlarging, firm painful lesion on the right posterior shoulder. The patient was initially treated for cellulitis given his clinical picture. Histopathologic examination revealed an angiocentric and dermal proliferation of markedly atypical lymphoid cells with numerous mitoses and apoptotic bodies along with broad zones of necrosis. Biopsy revealed the presentation to be consistent with NK/T-cell lymphoma. The cutaneous lesions from NK/T-cell lymphoma can often be initially mistaken for cellulitis, therefore this malignancy should be included on the differential in a patient HIV/acquired immune deficiency syndrome (AIDS).

Geriatric Genital Dermatology.

As women age, hormonal changes set the stage for a variety of vulvovaginal pathologies. Health care providers in long-term care facilities should be able to recognize and treat these conditions, especially because residents may be unable to communicate their discomfort. The objective of this article is to highlight the major vulvovaginal conditions affecting older women and provide up-to-date information on treatment for providers in long-term care facilities.

Assessment of the Cutaneous Hormone Landscapes and Microbiomes in Vulvar Lichen Sclerosus.

Vulvar lichen sclerosus (VLS) is a progressive skin disease of unknown etiology. In this longitudinal case-control exploratory study, we evaluated the hormonal and microbial landscapes in 18 postmenopausal females (mean [SD] age: 64.4 [8.4] years) with VLS and controls. We reevaluated the patients with VLS after 10-14 weeks of daily topical class I steroid. We found that groin cutaneous estrone was lower in VLS than in controls (-22.33, 95% confidence interval [CI] = -36.96 to -7.70; P = .006); cutaneous progesterone was higher (5.73, 95% CI = 3.74-7.73; P < .0001). Forehead 11-deoxycortisol (-0.24, 95% CI = -0.42 to -0.06; P = .01) and testosterone (-7.22, 95% CI = -12.83 to -1.62; P = .02) were lower in disease. With treatment, cutaneous estrone (-7.88, 95% CI = -44.07 to 28.31; P = .62), progesterone (2.02, 95% CI = -2.08 to 6.11; P = .29), and 11-deoxycortisol (-0.13, 95% CI = -0.32 to 0.05; P = .15) normalized; testosterone remained suppressed (-7.41, 95% CI = -13.38 to -1.43; P = .02). 16S ribosomal RNA V1-V3 and ITS1 amplicon sequencing revealed bacterial and fungal microbiome alterations in disease. Findings suggest that cutaneous sex hormone and bacterial microbiome alterations may be associated with VLS in postmenopausal females.

Sudden Conjunctivitis, Lymphopenia, and Rash Combined With Hemodynamic Changes (SCoRCH) After Trimethoprim-Sulfamethoxazole Use: A Case Series Study of a Hypersensitivity Reaction.

Importance: Trimethoprim-sulfamethoxazole (TMP-SMX) hypersensitivity reaction, ranging from circulatory shock to aseptic meningitis and respiratory failure, is a potentially life-threatening condition with dermatologic relevance. Objective: To describe the mucocutaneous findings and clinical features of TMP-SMX hypersensitivity reaction. Design, Setting, and Participants: This was a retrospective case series study of 7 patients who developed a characteristic rash, hemodynamic changes, and end-organ dysfunction after treatment with TMP-SMX at a large university hospital system during January 2013 to March 2022. Exposures: Treatment with TMP-SMX within 2 weeks of the reaction. Main Outcome and Measures: Descriptions of the condition, including the demographic information of the affected population, the reaction timeline, and mucocutaneous and clinical features. Results: The cohort comprised 7 patients (median [range] age, 20 [15-66] years; 4 female and 3 male). The most common mucocutaneous findings were generalized sunburn-like erythema without scale, conjunctivitis, and mild facial and acral edema. Three patients had previous exposure to TMP-SMX and developed symptoms in 1 day or less, while those without prior exposure presented from 4 to 11 days after drug initiation. Among the 7 patients, 6 had fever, 7 had hypotension, and 7 had tachycardia. All patients had lymphopenia and evidence of end-organ dysfunction with either kidney or liver involvement. Median (range) time to resolution was 72 (48-96) hours. Conclusions and Relevance: This retrospective case series indicates that SCoRCH (sudden conjunctivitis, lymphopenia, and rash combined with hemodynamic changes) should be considered in the differential diagnosis of patients presenting with acute generalized sunburn-like erythema, conjunctivitis, systemic symptoms, and hemodynamic changes in the setting of recent TMP-SMX use.