[Translated article] Prurigiform Angiomatosis: A New Term for Describing the Prominent Dermal Angiomatoid Proliferation Underlying Epidermal Changes in Prurigo Nodularis/Lichen Simplex Chronicus.

Vascular hyperplasia is a common finding in prurigo nodularis/lichen simplex chronicus (LSC). The term prurigiform angiomatosis was recently proposed to describe a histologic pattern characterized by prominent vascular hyperplasia in patients with LSC. The aim of this study was to identify cases of LSC with this pattern and analyze associations with clinical and pathologic features and disease course. We reviewed 54 cases of histologically confirmed LSC and detected findings consistent with prurigiform angiomatosis in 10 (18.5%). The patients (7 men, 3 women) had a mean age of 59.7 years. The lesions were pruritic and predominantly located on the extremities and trunk. The most notable histologic finding was vascular proliferation in the superficial dermis associated with a lymphocytic inflammatory infiltrate. Recognition of prurigiform angiomatosis is important as it helps not only to distinguish LSC from other entities (mainly vascular tumors) but also to detect lesions that need to be surgically excised due to poor response to topical treatment.

[Vulvar lymphangioma circumscriptum: diagnosis and treatment]. / Het vulvaire lymfangioom.

BACKGROUND: The vulvar form of lymphangioma circumscriptumis a rare condition. It is part of the acquired lymphangiectasia and arises secondary, for example, after surgery, radiotherapy for malignancies in the pelvic region, inflammation in which vulvar lymphedema occurs or Morbus Crohn. CASE DESCRIPTION: A 44-year-old woman presented to the gynaecology outpatient department with a vulvar abnormality that was accompanied by pain and pruritus. Her medical history consisted of premalignant cervical abnormalities and a vulvar lichen simplex chronicus. A biopsy was taken and the diagnosis lymphangioma circumscriptum was made. Due to the growth and the complaints, the decision was made to remove the lesion in the operating room. CONCLUSION: Lymphangioma circumscriptum is a rare condition that is often misdiagnosed. This case may describe the development of lymphangioma circumscriptum from a lichen simplex chronicus, which has not been described before. It also demonstrates that surgical treatment appears to be a good treatment with few complications in the postoperative course.

Comparison of p53 immunohistochemical staining in differentiated vulvar intraepithelial neoplasia (dVIN) with that in inflammatory dermatoses and benign squamous lesions in the vulva.

AIMS: Differentiated vulvar intraepithelial neoplasia (dVIN), the precursor lesion to human papillomavirus-independent vulvar squamous cell carcinoma (VSCC), can be difficult to distinguish from vulvar inflammatory dermatoses. Our goal was to determine if p53 could be a useful biomarker for dVIN, by characterizing p53 percentage, intensity and patterns of staining in dVIN and its histological mimics. METHODS AND RESULTS: We studied p53 immunohistochemical staining patterns in 16 dVIN cases and 46 vulvar non-neoplastic squamous lesions [12 lichen sclerosus (LS); seven lichen simplex chronicus; three lichen planus (LP); six psoriasis; 13 spongiotic dermatitis (SPO); and five candidiasis]. dVIN cases were adjacent to a p16-negative invasive VSCC in resection specimens. All dVIN cases showed null-type or moderate to strong uniform p53 staining in >70% of basal cells, with moderate to strong continuous parabasal staining extending to two-thirds of the epidermis. This was in contrast to weak or weak to moderate patchy p53 staining in the majority of other lesions. Moderate to strong and increased basal p53 staining (≥70%) was also observed in a subset of LS cases (5/12, 42%), LP cases (1/3, 33%), and SPO cases (36%, 4/11); however, in all categories, this was limited to the basal layer, and any staining in the parabasal layers was patchy. CONCLUSION: Strong and uniform p53 staining of basal cells, extending into the parabasal layers, and a complete absence of staining (null type) is useful in distinguishing dVIN from other mimics in the vulva. p53 staining of lesser intensity or quantity, particularly basal overexpression only, overlaps with that in vulvar inflammatory lesions.

Cupping for neurodermatitis: A protocol of systematic review and meta-analysis.

BACKGROUND: Neurodermatitis is a common inflammatory and allergic disease, characterized by itching and lichenification plaque. Some studies have reported cupping therapy (CT) for the treatment of neurodermatitis. However, the effectiveness and safety are still uncertain. This study aims to evaluate the efficacy and safety of CT for the treatment of patients with neurodermatitis. METHODS: We will retrieve the following electronic databases systematically: Pubmed, Web of Science, Embase, the Cochrane Library, Chinese Scientific Journal Database, China National Knowledge Infrastructure Database, Chinese Biomedical Literature Database, and Wanfang database from their inception to December 2020. Other literature resources will be manually searched. Published randomized controlled trials (RCTs) and quasi-randomized controlled trials (q-RCTs) on the topic will be retrieved by 2 investigators independently. We will apply a fixed-effect model or random effect model basis on the heterogeneity test and employ with RevMan 5.3 software for data synthesis. The total clinical effective rate will be selected as the primary outcome, skin disease quality of life index score, recurrence rate, and adverse events as secondary outcomes. RESULTS: This study will comprehensively summarize the high-quality trials to determine the efficacy and safety of CT for the treatment of patients with neurodermatitis. CONCLUSION: Our systematic review will present evidence for the efficacy and safety of CT to neurodermatitis patients. OSF REGISTRATION NUMBER: DOI 10.17605/OSF.IO/6DCM3.

Human Papillomavirus-Independent Squamous Lesions of the Vulva.

The pathogenesis of vulvar squamous neoplasia has 2 pathways: human papillomavirus (HPV)-dependent and HPV-independent. The HPV-dependent pathway in the vulva follows the same progression as HPV-dependent lesions elsewhere in the gynecologic tract-HPV infection results in high-grade squamous intraepithelial lesion with subsequent progression to basaloid squamous cell carcinoma. The HPV-independent pathway is more complex, with a variety of precursor lesions and molecular alterations. Although the most recognized form of HPV-independent vulvar lesion is differentiated vulvar intraepithelial neoplasia, recent explorations have elucidated new precursors. This review provides an update on HPV-independent risk factors and precursor lesions for squamous cell carcinoma of the vulva.

Classic Vulvar Intraepithelial Neoplasia With Superimposed Lichen Simplex Chronicus: A Unique Variant Mimicking Differentiated Vulvar Intraepithelial Neoplasia.

High-grade vulvar intraepithelial neoplasia, a precursor lesion to vulvar squamous cell carcinoma, is subdivided into 2 types, classic or usual vulvar intraepithelial neoplasia (CVIN) and differentiated vulvar intraepithelial neoplasia (DVIN). CVIN, which is a human papilloma virus (HPV)-dependent lesion, is typically distinguished from DVIN, a p53 mutation-dependent process, by its distinct histomorphologic and immunohistochemical characteristics. However, distinguishing between the 2 entities becomes challenging in cases of CVIN with superimposed inflammatory changes, especially lichen simplex chronicus (LSC). Twelve cases of DVIN, 9 cases of LSC, and 9 cases of CVIN with superimposed LSC were assessed for a number of morphologic features, including hyperkeratosis, hypergranulosis, acanthosis, hypercellularity, abnormal maturation (i.e. abnormal keratinization close to the base and/or dyskeratosis), hyperchromasia, and basal atypia. Immunohistochemistry for p53, p16, and MIB-1 was performed for all cases. When sufficient tissue was available, HPV genotyping was performed for cases of CVIN with superimposed LSC. DVIN uniformly demonstrated abnormal maturation, and atypia involving the basal cell layer; they were all p16 negative and demonstrated p53 positivity of moderate to strong intensity in a basal and parabasal distribution. CVIN with superimposed LSC frequently displayed hyperchromasia involving the basal 3 to 4 cell layers, basal to full-thickness atypia, and apoptosis. CVIN with superimposed LSC demonstrated intense p16 positivity extending from the basal cells to the mid-epithelium and a reduction or loss of staining in maturing keratinocytes. P53 staining revealed a unique pattern of parabasal and mid-epithelial weak to moderate staining with sparing of the basal layer. Cases of LSC demonstrated heterogenous p53 positivity and were negative for p16. MIB-1 staining showed a similar range of positivity for all diagnoses. HPV genotyping revealed HPV 16 in all 5 cases of CVIN with LSC that underwent testing. We conclude that, although CVIN with superimposed LSC can closely resemble DVIN, morphologic features such as nuclear hyperchromasia uniformly involving the basal 3 to 4 cell layers, apoptosis, and absent or less pronounced cytoplasmic maturation are more suggestive of CVIN with superimposed LSC. In cases where the morphology remains ambiguous, immunohistochemistry for both p16 and p53 can be helpful. In particular, p53 parabasal and mid-epithelial staining without involvement of the basal layer appears to be a characteristic finding in CVIN with superimposed LSC. MIB-1 staining is of little utility in distinguishing between these entities and should not be routinely performed.

Lichen simplex chronicus on the scalp: exuberant clinical, dermoscopic, and histopathological findings.

Lichen simplex chronicus is a skin disease that mostly affects female patients, with a peak incidence between ages 35 and 50 years. On the scalp, it appears as a single or multiple oval lesions, showing scaling and hair shaft loss or breakage. An important dermoscopic feature of the disease are the "broom fibers." Histopathology reveals the "hamburger" and the "gear wheel" signs. The aim of this report is to demonstrate a case of lichen simplex chronicus on the scalp with typical and exuberant clinical, dermoscopic, and histopathological findings.

Glucose Monitoring Dermopathy.

A 75-year-old man had been monitoring his glucose using a blood glucose monitoring system at the same body site for at least 20 years (>7300 needlesticks). The asymptomatic skin lesion had been present for many years. He used the same site because it hurt less than the fingers and bled well. His medical history was remarkable for diabetes mellitus, hypertension, coronary artery disease, and a pacemaker. His medications included glipizide, metformin, carvedilol, furosemide, lisinopril, amlodipine, clopidogrel, and aspirin. Physical examination revealed a brown, slightly raised, rough plaque with focal, punctate hemorrhagic crusts, on the distal area of the right thigh (Figure 1). The clinical differential diagnosis was more likely to be lichen simplex chronicus than pigmented Bowen's disease. A skin biopsy demonstrated an acanthotic epidermis with coarse collagen bundles in a thickened papillary dermis with extravasated erythrocytes, consistent with a dermal reparative reaction (Figure 2).

Lichen simplex chronicus on the scalp: exuberant clinical, dermoscopic, and histopathological findings

Abstract: Lichen simplex chronicus is a skin disease that mostly affects female patients, with a peak incidence between ages 35 and 50 years. On the scalp, it appears as a single or multiple oval lesions, showing scaling and hair shaft loss or breakage. An important dermoscopic feature of the disease are the "broom fibers." Histopathology reveals the "hamburger" and the "gear wheel" signs. The aim of this report is to demonstrate a case of lichen simplex chronicus on the scalp with typical and exuberant clinical, dermoscopic, and histopathological findings.

Obsessive-compulsive skin disorders: a novel classification based on degree of insight.

Individuals with obsessive-compulsive features frequently visit dermatologists for complaints of the skin, hair or nails, and often progress towards a chronic relapsing course due to the challenge associated with accurate diagnosis and management of their psychiatric symptoms. The current DSM-5 formally recognizes body dysmorphic disorder, trichotillomania, neurotic excoriation and body focused repetitive behavior disorder as psychodermatological disorders belonging to the category of Obsessive-Compulsive and Related Disorders. However there is evidence that other relevant skin diseases such as delusions of parasitosis, dermatitis artefacta, contamination dermatitis, AIDS phobia, trichotemnomania and even lichen simplex chronicus possess prominent obsessive-compulsive characteristics that do not necessarily fit the full diagnostic criteria of the DSM-5. Therefore, to increase dermatologists' awareness of this unique group of skin disorders with OCD features, we propose a novel classification system called Obsessive-Compulsive Insight Continuum. Under this new classification system, obsessive-compulsive skin manifestations are categorized along a continuum based on degree of insight, from minimal insight with delusional obsessions to good insight with minimal obsessions. Understanding the level of insight is thus an important first step for clinicians who routinely interact with these patients.

Lesions With an Epidermal Hyperplastic Pattern: Morphologic Clues in the Differential Diagnosis.

In this report, we review the most common entities that show a epidermal hyperplastic pattern in the biopsy. These entities include inflammatory, reactive, infectious, hamartomatous, and tumoral conditions, each with a very different prognosis. Therefore, an approach based on the classic "patterns and clues" tools used in dermatopathology can bring a lot of information to the dermatopathologist or the general pathologist evaluating these lesions.

The serine protease inhibitor of Kazal-type 9 (SPINK9) is expressed in lichen simplex chronicus, actinic keratosis and squamous cell carcinoma.

The serine protease inhibitor of Kazal-type (SPINK) 9 was reported to be exclusively expressed in palmoplantar skin. SPINK9 is a specific inhibitor of the serine protease kallikrein-related peptidase 5 (KLK5), which contributes to the desquamation process of the stratum corneum. Herein, we demonstrated that SPINK9 is also expressed in lichen simplex chronicus. Moreover, we observed expression of SPINK9 in 51 % of lesions of actinic keratosis and squamous cell carcinoma. In conclusion, we demonstrate that SPINK9 is not only expressed in healthy palmoplantar skin, but also in dermatologic disorders like lichen simplex chronicus, actinic keratosis and squamous cell carcinoma.

Grape Cells (Multinucleated Keratinocytes) in Noninfectious Dermatoses: Case Series and Review of the Literature.

Multinucleated keratinocytes (also known as multinucleated epidermal giant cells) are a frequently overlooked histological finding in noninfectious inflammatory dermatoses. They are sometimes found in conditions characterized by chronic rubbing and pruritus, such as lichen simplex chronicus or prurigo nodularis, and may be a helpful clue in making the clinical diagnosis. This finding must be differentiated from other conditions characterized by multinucleated keratinocytes on histopathology, specifically herpes simplex, varicella zoster, or measles viral infections. The authors present a case series of 2 patients with unique clinical noninfectious diagnoses but similar histopathologic findings on biopsy. The histopathologic findings on both cases demonstrated multinucleated keratinocytes, which were related to manipulation of the epidermis.

Vulval pruritus: the experience of gynaecologists revealed by biopsy.

Pruritus of the vulva is a common symptom among patients attending to outpatient clinics. In the present study, we aimed to assess pathologies causing vulval pruritus in the reliability of biopsy in a tertiary referral centre. A total of 137 patients undergoing vulval colposcopy because of vulval pruritus were reviewed from the hospital records retrospectively. The mean age of the patients was 47.61 ± 11.88 years and 36.5% of the patients were postmenopausal. In 101 (73.7%) of the patients, macroscopic lesions were present. In 88 (64.2%) of the patients, tolidine-positive stained areas were determined under colposcopy. In total, 68 (49.6%) of the lesions were plain, whereas 51 (37.2%) of them were depigmented. Lichen simplex chronicus, lichen sclerosis and chronic inflammation were the major pathologies associated with vulval pruritus (25.5%, n = 35; 20.4%, n = 28; 14.6%, n = 20). In conclusion, several pathologies out of vulvovaginal candidiasis may lead to vulval pruritus and clinicians should be aware of the importance of biopsy in determining the underlying pathology.

An update on vulvar intraepithelial neoplasia: terminology and a practical approach to diagnosis.

There are two distinct types of vulvar intraepithelial neoplasia (VIN), which differ in their clinical presentation, aetiology, pathogenesis and histological/immunophenotypical features. One form driven by high-risk human papilloma virus infection usually occurs in young women and has been termed classic or usual VIN (uVIN). The other, not related to viral infection, occurs in postmenopausal women with chronic skin conditions such as lichen sclerosus and lichen simplex chronicus and is termed differentiated or simplex-type VIN. The latter is the precursor lesion of the most common type of squamous cell carcinoma (SCC) in the vulva, namely keratinizing SCC (representing 60% of cases). In contrast, uVIN usually gives rise to basaloid or warty SCC (40% of cases). The histological features of uVIN are similar to those of high grade lesions encountered in other lower anogenital tract sites (hyperchomatic nuclei with high nuclear to cytoplasmic ratios and increased mitotic activity). However, differentiated VIN has very subtle histopathological changes and often escapes diagnosis. Since uVIN is driven by high-risk human papilloma virus infections, p16 immunohistochemistry is diffusely positive in these lesions and is characterized with a high Ki-67 proliferation index. In contrast, differentiated or simplex-type VIN is consistently negative for p16 and the majority of the cases harbour TP53 mutations, correlating with p53 positivity by immunohistochemistry.