Fibroadenoma of the vulva with pseudoangiomatous stromal hyperplasia: A common neoplasm in uncommon site.

Mammary-type tissue in the vulva was first described in 1872 but has been rarely reported in the literature. This tissue was previously considered as ectopic breast tissue that occurs as a result of incomplete regression of the milk line. Similar to native breast tissue, ectopic mammary tissue is hormone-sensitive and can develop benign changes, such as fibroadenoma, as well as malignant changes. A more recent theory suggests that these benign and malignant mammary-type entities arise from mammary-like anogenital glands, which constitute normal vulvar components. We report a case of a 41-year-old woman who presented with a chronic asymptomatic cyst on the left vulva that eventually became uncomfortable, especially on standing. The cyst was located on the labium minus, measuring 1.0 × 0.5 cm, with no identified erythema or other skin abnormalities. Excision of the lesion and subsequent microscopic examination showed a circumscribed mass with a nodular overgrowth of epithelial and stromal components, resembling a mammary fibroadenoma with pseudoangiomatous stromal hyperplasia. We bring to attention this rare diagnosis and the importance of considering it in the presence of a vulvar lesion. The malignant and recurrence potential of mammary-type tissue necessitates excision with clear margins and close monitoring of these patients.

Cutaneous crystal storing histiocytosis: A case series with review of literature.

Crystal-storing histiocytosis (CSH) is a rare condition in which crystals accumulate in the cytoplasm of histiocytes and is usually associated with a lymphoplasmacytic neoplasm. Cutaneous CSH is extraordinarily rare and limited to case reports in the literature. We report two cases of this disease with cutaneous involvement. Case 1 was a 65-year-old male with a 4-month history of a pruritic eruption that started as a solitary pink to skin-colored indurated plaque on the anterior neck before progressing to involve the whole neck, chest wall, and face. Case 2 was a 54-year-old woman with a history of unspecified "lymphoma" who presented with a soft nodule on the forearm. Biopsies from both cases had similar findings and showed a proliferation of epithelioid cells with pink cytoplasm and intracellular crystalline structures infiltrating the dermis and subcutaneous fat. In the first case, the cells were positive for CD43, CD45, CD68, and IgG kappa, and in the second case, the crystals were positive for IgG lambda. Based on these findings, the patients were diagnosed with cutaneous CSH. We highlight this rare diagnosis and the importance of investigating an underlying lymphoplasmacytic neoplasm.

Patient-Reported Outcome Measures for Health-Related Quality of Life in Patients With Acne Vulgaris: A Systematic Review of Measure Development and Measurement Properties.

Importance: Multiple patient-reported outcome measures (PROMs) for health-related quality of life (HRQoL) exist for patients with acne. However, little is known about the content validity and other measurement properties of these PROMs. Objective: To systematically review PROMs for HRQoL in adults or adolescents with acne. Data Sources: Eligible studies were extracted from PubMed and Embase (OVID). Study Selection: Full-text articles published in English or Spanish on development, pilot, or validation studies for acne-specific, dermatology-specific, or generic HRQoL PROMs were included. Development studies included original development studies, even if not studied in acne patients per Consensus-Based Standards for the Selection of Health Measurement Instruments (COSMIN) recommendations. If a study included several diagnoses, the majority (ie, over 50%) of patients must have acne or acne-specific subgroup analyses must be available. Abstract and full-text screening was performed by 2 independent reviewers. Data Extraction and Synthesis: Two independent reviewers assessed study quality applying the COSMIN checklist and extracted and analyzed the data. For each distinctive PROM, quality of evidence was graded by measurement property. Main Outcomes and Measures: PROM properties (target population, domains, recall period, development language), PROM development and pilot studies, content validity (relevance, comprehensiveness, comprehensibility), and remaining measurement properties (structural validity, internal consistency, cross-cultural validity, reliability, measurement error, criterion validity, construct validity, and responsiveness). Quality of evidence was assigned for each measurement property of included PROMs. An overall recommendation level was assigned based on content validity and quality of the evidence of measurement properties. Results: We identified 54 acne PROM development or validation studies for 10 acne-specific PROMs, 6 dermatology-specific PROMs, and 5 generic PROMs. Few PROMs had studies for responsiveness. The only acne-specific PROMs with sufficient evidence for content validity were the CompAQ and Acne-Q. Based on available evidence, the Acne-Q and CompAQ can be recommended for use in acne clinical studies. Conclusions and Relevance: Two PROMs can currently be recommended for use in acne clinical studies: the Acne-Q and CompAQ. Evidence on content validity and other measurement properties were lacking for all PROMs; further research investigating the quality of remaining acne-specific, dermatology-specific, and generic HRQoL PROMs is required to recommend their use.

Chronic Wound Telemedicine Models Before and During the COVID-19 Pandemic: A Scoping Review.

GENERAL PURPOSE: To present the results of a scoping review exploring chronic wound care telemedicine before and during the pandemic, including the characteristics of the models implemented. TARGET AUDIENCE: This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care. LEARNING OBJECTIVES/OUTCOMES: After participating in this educational activity, the participant will:1. Identify the characteristics of the studies the authors examined for their scoping review of chronic wound care telemedicine.2. Choose the electronic methods commonly used for wound care telemedicine in the studies the authors examined.3. Recognize the implications for the patients who participated in chronic wound care telemedicine in the studies the authors examined. ABSTRACT: OBJECTIVETo explore different chronic wound telemedicine models and identify current research on this topic.METHODSThe authors searched the MEDLINE and EMBASE databases on August 10, 2021 and identified 58 articles included in the analysis.RESULTSIncluded studies were published between 1999 and 2021, with more than half of the studies published between 2015 to 2019 (25.9%, n = 15/58) and 2020 to 2021 (25.9%, n = 15/58). There were 57 models identified, of which 87.7% (n = 50/57) used a blended model of care. Image assessment was the most common element in blended care (66.0%, n = 33/50), followed by video consultation (46.0%, n = 23/50), text (44.0%, n = 22/50), and telephone consultation (22.0%, n = 11/50). Purely virtual care was used in 12.3% (n = 7/57) of models, 85.7% (n = 6/7) of which were implemented during the COVID-19 pandemic. Most studies conducted a quantitative analysis (62.1%, n = 36/58); 20.7% (n = 12/58) conducted a qualitative analysis, and 17.2% (n = 10/58) conducted both. The most frequently assessed results were wound outcomes (53.4%, n = 31/58) and patient opinions (25.9%, n = 15/58).CONCLUSIONSChronic wound care-related telemedicine has common elements: image assessment, video and telephone consultation, and text-based information that can be combined in a variety of ways with unique implementation barriers. Blended care models are more common than purely virtual alternatives. Heterogeneity among outcomes and reporting methods make the results difficult to synthesize.

Verrucous esophageal carcinoma is a unique indolent subtype of squamous cell carcinoma: a systematic review and individual patient regression analysis.

BACKGROUND AND AIMS: Verrucous esophageal carcinoma (VEC) is a rare malignancy that presents a diagnostic challenge. We aim to characterize the clinical and genomic features, tumor behavior, and treatment outcomes of VEC to guide clinical practice. METHODS: We performed a systematic review of the literature and identified additional cases from Massachusetts General Hospital records and The Cancer Genome Atlas (TCGA). We obtained individual VEC patient data and analyzed publicly available clinicogenomic data from TCGA. We performed a regression analysis comparing cases of VEC to esophageal squamous cell carcinoma (ESCC) to identify factors influencing survival. RESULTS: A total of 135 patients were reported in 82 publications, and four unpublished cases from Massachusetts General Hospital (median age 65 years, 69% males, 48% smokers, 33% consumed alcohol). Symptoms were present at diagnosis in 95% of patients, most commonly dysphagia and weight loss. Median symptom onset to diagnosis time was 11.5 months with frequent misdiagnosis as Candida esophagitis. Among VEC cases with pathologic staging, lymph node metastases were rare (5%) compared to ESCC (40%). VEC was genomically characterized by enrichment of SMARCA4 missense mutations and a lack of pathogenic TP53 mutations. Despite its diagnostic elusiveness, in a multivariate regression analysis, VEC was detected at earlier stages (p = < 0.001) compared to ESCC, and advanced stage was the only significant factor affecting survival (p = 0.013). CONCLUSIONS: VEC is a rare, clinically and genomically distinct subtype of ESCC. Recognition and diagnosis of this lesion may allow the pursuit of curative and less morbid treatment strategies.