Woringer-Kolopp disease (localized pagetoid reticulosis): a systematic review.

OBJECTIVE: Woringer-Kolopp disease (WKD), also known as localized pagetoid reticulosis, is a rare variant of mycosis fungoides as described by the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification system for cutaneous lymphomas. Our objective was to conduct a comprehensive review that describes and evaluates patient demographics, clinical presentation, immunohistochemical findings, management, and outcomes of WKD. METHODS: The databases PubMed, Embase, and Cochrane Library were searched for relevant literature. Patient demographics, imaging, treatments, and other clinical characteristics were obtained. The literature search was conducted on December 7, 2020. Studies were included if they contained primary data related to WKD. Non-pertinent studies, non-English studies, non-human studies, review articles, or studies with insufficient case information were excluded. The quality of the included studies and the risk of bias were evaluated using the Grades of Recommendation, Assessment, Development, and Evaluation Group (GRADE) criteria (2016), Oxford Centre for Evidence-Based Medicine: Levels of Evidence (OCEBM) (March 2009), and the Methodological Index for Non-Randomized Studies (MINORS) criteria (2003). RESULTS: A total of 84 studies with 143 patients met the inclusion criteria for this study. The most common chief complaint was an asymptomatic, slow-growing, and erythematous plaque. The average time from initial skin eruption to presentation was 97.6 months. Histologically, 128 cases of WKD displayed epidermotropism (97.7%). Various therapies, including radiotherapy, surgery, and local chemotherapy, were used. In total, 92 (78.6%) cases had complete remission and 11 (9.4%) cases recurred. CONCLUSIONS: WKD is a rare cutaneous T-cell lymphoma that often presents as a single plaque on the extremities with an indolent course and a favorable prognosis. However, it is often misdiagnosed, leading to delays in treatment. The diagnosis of WKD involves clinical appraisal, a biopsy of suspicious lesions, and immunohistochemistry. Monotherapy appears to be preferred compared to combination therapy for WKD with radiotherapy demonstrating among the highest cure rates and lowest recurrence. Future retrospective and prospective studies are needed to accurately determine the epidemiology, prognosis, and effective treatments for WKD. Limitations include a possibility of missed studies, heterogeneity in reporting methods, publication, and selection bias.

Ethnic distribution of populations in the highest and lowest dermatologist-dense areas: is there more to the story?

Several studies in the past decade have highlighted the lack of adequate dermatological care in skin of color (SOC) patients. This inquiry has led to further research to identify the sources of this disparity. Previous studies have highlighted the uneven geographic distribution of dermatologists, with a higher density of dermatologists in urban areas compared to other areas. However, the exact ethnic populations served by these dermatologists has remained largely uncharacterized. The purpose of this study was to compare the ethnic distributions in the ten highest and lowest dermatologist-dense areas across the United States to determine if there is equal access to dermatological care for minorities. Stratified by ethnicities, the highest dermatologist-dense areas consisted of 60% White alone (not Hispanic or Latino), 13% Hispanic or Latino, 13% Asian alone, and 12% Black or African American. Conversely, the least dermatologist-dense areas consisted of 45% White alone (not Hispanic or Latino), 28% Black or African American, 21% Hispanic or Latino, and 4% Asian alone. Our analysis highlights the presence of larger proportions of SOC patients in the lowest dermatologist-dense areas and this lack of access to dermatologists may contribute to inferior dermatological care and outcomes in Hispanic or Latino, and Black or African American minorities.

Ewing Sarcoma of the Craniofacial Bones: A Qualitative Systematic Review.

OBJECTIVES: To conduct a systematic review on the demographics, characteristics, management, treatment, complications, and outcomes of Ewing sarcomas in the craniofacial bones. DATA SOURCES: Using Cochrane Library, EmBase, and PubMed, the authors identified 71 studies to be included. REVIEW METHODS: The Cochrane Library, EmBase, and PubMed databases were used to identify literature relating to Ewing sarcomas in the craniofacial bone to conduct a systematic review. Patient demographics, clinical characteristics, and treatment strategies were extracted. RESULTS: Seventy-one studies encompassing 102 patients were identified. The most common craniofacial locations involved were the frontal bone (16.7%, n = 17), nasal cavity (16.7%, n = 17), and temporal bone (14.7%, n = 15). Stratified by location, the most common presenting symptoms were frontal bone (palpable mass, n = 8, 47.1%), nasal cavities (epistaxis, n = 9, 52.9%), and temporal bones (headache, n = 5, 33.3%). The 3 most commonly used treatment strategies were a combination of surgical intervention/radiotherapy/chemotherapy (n = 43, 43%), a combination of radiotherapy/chemotherapy (n = 18, 18%), and a combination of surgical intervention/chemotherapy (n = 15, 15%). Patients who received a combination of surgical intervention/radiotherapy/chemotherapy experienced local recurrence rate of 16.6%. However, other combinations of therapies such as surgical intervention/chemotherapy and radiotherapy/chemotherapy had a lower local recurrence rate but were limited by small sample size. Most patients (79.0%) were disease free without evidence of recurrence. CONCLUSION: Ewing Sarcoma of the craniofacial bones has a good prognosis when treated appropriately. Given that our study was limited by retrospective data, we advise clinicians to use the findings of this article with their own clinical judgment to determine which treatment strategy they should pursue.

Individual Typology Angle and Fitzpatrick Skin Phototypes are Not Equivalent in Photodermatology.

Individual typology angle (ITA) measures constitutive pigmentation while skin phototypes (SPT) categories are based on sun reactiveness. However, the association between the two, ITA and SPT, is yet to be established. Since both systems provide six categories, recent studies have used ITA classifications as synonymous to SPT. The results of this study indicate that these cannot be utilized interchangeably. In conclusion, poor correlation between the six objective individual typology angle categories and the subjective Fitzpatrick skin phototype categories was established along with highlighting ITAs potential in photobiologic studies and objective standardization of skin type classifications.

Prospective protocol registration should be required for systematic reviews in dermatology literature.

Recently, the authors of this study undertook a systematic review, and during the data collection phase, a systematic review was published on the same topic, despite not being registered on Prospective Registration of Systematic Reviews (PROSPERO). As a result we sought to perform an evidence-based review of the dermatology literature evaluating PROSPERO registration for published systematic reviews. Prospective systematic review protocol registration can help optimize resources, time, and the efforts of research teams rather than committing unplanned duplication. Our data on journals' author guidelines showed that although 38.8% of journals mentioned systematic reviews in their author guidelines, only 2.5% (n = 2) required PROSPERO registration. Further analysis revealed that only 13.7% (n = 204 of 1,492) of published systematic reviews in dermatology literature were registered in PROSPERO. Our study highlights the paucity of PROSPERO-registered systematic reviews in dermatology journals, as well as the need to require prospective protocol registration and require submission of a Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist. We believe these measures will ultimately improve the quality of systematic reviews in dermatology literature.

Malignant Proliferating Trichilemmal Tumor of the Scalp: A Systematic Review.

OBJECTIVES: Malignant proliferating trichilemmal tumors of the scalp can exhibit aggressive presentation and recurrences. Our objective was to perform an evidence-based systematic review evaluating clinical presentation, tumor characteristics, and treatment modalities used to determine which treatment strategies had the best outcomes. METHODS: The databases PubMed, Embase, and Cochrane Library were searched for relevant literature by the authors. Patient demographics, imaging, treatments, and other clinical characteristics were obtained. The results were reported using the Preferred Reporting Systems for Systematic Reviews and Meta-Analysis guidelines. RESULTS: Thirty-nine studies with a total of 65 patients were identified. The most common presentation was a history of slow-growing, painless swollen mass on the scalp. In total, 10 patients (15.4%) presented with spread to the regional lymph nodes and 6 (9.2%) additional patients presented with metastasis to distant locations. In total, 61 patients (93.8%) underwent surgery. Various chemotherapy and radiation therapy regimens were used. Of the 45 cases with documented follow-up, 11 (24.4%) patients had one or multiple instances of local, lymph node or metastatic tumor recurrence. CONCLUSIONS: Surgery is favored, and the exact approach should be based on clinical judgment. However, Mohs micrographic surgery should strongly be considered because of its superior margin control against such an invasive tumor. Radiotherapy and chemotherapy have been used as adjuvant therapy in aggressive cases or recurrence. Patients should be followed closely and examined often to frequently assess recurrence or metastasis. Randomized controlled trials are needed to further clarify these findings.

Aneurysmal Bone Cysts of the Craniofacial Origin: A Systematic Review.

OBJECTIVE: Aneurysmal bone cysts (ABCs) are blood-filled, locally destructive, benign bone tumors. Our objective was to conduct a systematic review outlining patient demographics, clinical characteristics, management, and outcomes of those with ABCs of the craniofacial bones. DATA SOURCES: Using PubMed, Cochrane, and Embase databases, 116 studies were included. REVIEW METHODS: Following Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines, a systematic review was conducted. Data including patient demographics, clinical characteristics, treatment strategies, and patient outcomes were collected. RESULTS: A total of 127 patients from 116 studies were identified. Age ranged from 8 months to 90 years, with a mean age of 19.0 years. The most commonly affected craniofacial locations were the mandible (n = 31, 24.4%), temporal bone (n = 21, 16.5%), and occipital bone (n = 14, 11.0%). The most common presenting symptoms included a nontender mass (n = 51, 40.2%), a tender mass (n = 31, 24.4%), and generalized headache (n = 30, 23.6%). Imaging modalities included computed tomography (CT) and magnetic resonance imaging (MRI) (n = 77, 60.6%), CT alone (n = 31, 24.4%), and MRI alone (n = 8, 6.2%). All patients underwent surgical resection, with 1 patient requiring adjuvant radiation in addition to surgery. In total, 121 patients were disease-free and symptom-free without evidence of recurrence (17.4-month mean follow-up, 5.4 months average time to first recurrence). CONCLUSION: The current literature's characterization of ABCs in craniofacial bones is limited to case reports and case series. Given the rarity of these tumors, head and neck surgeons may rely on systematic reviews such as the present analysis to guide management.

Ovarian Cystic Teratoma in Pregnant Women: Conservative Management or Prophylactic Oophorectomy?

Acute abdominal pain in pregnancy is common and the differential diagnosis is vast. Mature cystic teratomas are rarely the cause of adnexal torsion during pregnancy and can be difficult to diagnose. Timely surgical intervention is required to avoid ovarian infarction. We report a 22-year-old patient presenting with sudden right lower abdominal pain. Imaging including bedside Doppler ultrasonography and MRI were negative for signs of acute ovarian torsion. Despite no definitive imaging findings, due to severe pain, we made the decision for diagnostic multi-port laparoscopic examination with possible oophorectomy. The right cystic ovary was noted to be torsed three times around the utero-ovarian ligament. A right oophorectomy was performed. Grossly, cystic teratoma was confirmed with a large amount of hair and sebum, and pathological analysis also confirmed a benign mature teratoma. The patient recovered well and delivered without any complications. Bedside ultrasonography is a highly accessible tool; however, imaging can be uncertain. Despite the rarity of ovarian torsion due to mature teratomas in second- and third-trimester pregnancies, physicians should be aware of the possibility of acute ovarian torsion in a pregnant patient even with uncertain imaging results, especially those with a documented ovarian mass. Early prophylactic surgical intervention preferably with laparoscopy should be pursued for ovarian masses between 5 cm and 10 cm.

Longest Survival of Expectantly Managed Twin Gestation Complicated by Previable Preterm Premature Rupture of Membranes at 13 Weeks' Gestation.

Previable preterm premature rupture of membranes (PV-PPROM) is defined as rupture of membranes prior to 24 weeks and is a rare phenomenon with an estimated prevalence of 0.5% of all pregnancies. Given that this phenomenon is even more rare in patients with dichorionic diamniotic (DCDA) twin pregnancies, there is no clear consensus in the literature on outcomes and management of DCDA PV-PPROM due to the scarcity of reports. We report a case of a rare successful prolongation of first trimester DCDA PV-PPROM pregnancy with rupture of the amniotic sac of one twin with survival of both twins without major complications. A 20-year-old female gravida 1 para 0 at about 13 weeks and three days presented with vaginal watery discharge mixed with vaginal bleeding. Abdominal ultrasound revealed a live twin dichorionic diamniotic (DCDA) spontaneous intrauterine gestation and a significantly low amniotic fluid volume involving fetus A. At 23 weeks gestational age, she experienced increased leaking of clear fluid, and she was admitted to the hospital for continuous monitoring with daily non-stress tests (NST), and ultrasounds every four weeks, and received antibiotics, betamethasone, and magnesium. Preterm labor occurred at 30w1d, and a primary low transverse cesarean section was performed on the 114th day after PPROM. Though, twin A required prolonged hospitalization both twins recovered and progressed well without complications. To the best of our knowledge, this is the longest case of successful expectant management of both twins with PV-PPROM yet reported.

A Rare Presentation of Acute Respiratory Distress Due to Diffuse Large B-Cell Lymphoma of the Tongue Base.

Primary diffuse large B-cell lymphoma of the tongue base (BOT) is an extremely rare entity with only a few cases described in the English literature to date. The incidence of BOT non-Hodgkin's lymphoma (NHL) increases with age, most commonly after the sixth decade of life with no observed gender differences. Our patient presented with a six-month history of right neck swelling, one-month history of dysphagia, a change in voice, and ultimately acute airway distress, which led to a tracheostomy. We report an extremely rare case of a diffuse large B-cell lymphoma presenting with airway distress. The patient was treated using rituximab-cyclophosphamide-doxorubicin-vincristine-prednisone (R-CHOP) chemotherapy, a five-day steroid course, and one intrathecal methotrexate. The patient recovered completely and is alive at the time of this writing. NHLs occur more commonly in patients like ours with a prior history of congenital immunodeficiency and celiac disease, exposure to radiation, acquired immune deficiency syndrome, rheumatoid arthritis, or Sjögren's syndrome. Most reported cases of BOT NHLs may cause dysphagia, pharyngeal foreign body sensation, or progressive dyspnea. This case highlights that although NHL of the tongue is a very rare entity, it should not be overlooked and should always be in the differential diagnosis among various benign and malignant tumors and may cause rapid respiratory deterioration.

Hidradenocarcinoma of the Abdominal Wall Treated With Wide Surgical Excision and Adjuvant Radiotherapy.

Hidradenocarcinomas are rare malignant sweat gland tumors that typically arise in the head and neck area. To the best of our knowledge, this is the only reported instance of hidradenocarcinoma of the abdominal wall as well as the first case arising from a region of prior trauma. A 72-year-old female presented with a left abdominal wall lesion, which she had first noticed after an injury to the area. Initially, the lesion remained stable in size, after which it became mildly pruritic, progressive in size, and expressive of a clear, non-odorous discharge. Imaging demonstrated a heterogeneous cystic density. Surgical pathology revealed a malignant dermal adnexal neoplasm composed of pleomorphic polygonal cells and focal intracytoplasmic lumina lined by eosinophilic cuticles, as well as areas of ductal differentiation, apocrine differentiation, and mucinous metaplasia. Surgical excision of the mass was performed, followed by adjuvant external beam radiotherapy (EBRT). The patient had no long-term toxicities or clinical evidence of local disease recurrence as of one year post-surgery and six months post-EBRT. Early diagnosis and treatment are essential to improving outcomes in patients with hidradenocarcinomas. Frequent follow-up is equally important, as these tumors have high recurrence rates.