Diagnostic study of actinic cheilitis by reflectance confocal microscopy / 口腔疾病防治

Objective @#To explore the clinical application value of reflectance confocal microscopy(RCM) in the diagnosis of actinic cheilitis(AC).@*Methods@#After approval by the hospital ethics committee and informed consent given by the patients, from October 2020 to July 2022, 17 patients who were diagnosed with actinic cheilitis in the Ninth People's Hospital affiliated with Shanghai Jiao Tong University School of Medicine were retrospectively analyzed. The white keratotic lesions of the lips were scanned with reflectance confocal microscopy, and the image characteristics were summarized and analyzed, including epithelial hyperplasia/atrophy, hyperkeratosis, inflammatory cell infiltration, blood vessel dilatation, solar elastosis, atypical keratinocytes, widening of intercellular spaces, degeneration of basal cell layer, and pigmentation. We used the sample compliance rate to measure the correlation between RCM parameters and histopathological diagnostic criteria for AC and kappa concordance analysis to calculate the concordance between RCM and pathological diagnosis. @* Results@# Under RCM, the sample correct rates for epithelial hyperplasia/atrophy, hyperkeratosis, inflammatory cell infiltration, vasodilation, and solar elastosis were 76.5%, 100%, 100%, 64.7%, and 70.6%, the sample accuracy compared with pathological diagnosis was 82.4%, 47.1%, 94.1%, 88.2% and 76.5%, respectively. We also observed that 100%, 88.2%, 76.5%, and 88.2% of AC patients showed RCM features of atypical keratinocytes, widening of intercellular spaces, degeneration of the basal cell layer, and pigmentation, respectively. The kappa value of hyperkeratosis and inflammatory cell infiltration was 1. The kappa value of blood vessel dilatation was 0.645. @* Conclusion @#Reflectance confocal microscopy is noninvasive and versatile and has clinical application value in the diagnosis of actinic cheilitis.

A Case Report of Annular Elastolytic Giant Cell Granuloma / 罕见病研究

Annular elastolytic giant cell granuloma(AEGCG) is a rare granulomatous skin condition. We present a case of a 67-year-old man with annular plaques on the face, neck, shoulder, back and upper limbs, and mildly pruritis exceeding one year. Histopathological examination demonstrated granulomatous inflammatory infiltration of lymphocytes, histiocytes and multinuclear giant cells in the middle and the upper dermis, with more local eosinophils. Elastic fiber staining showed that elastic fibers were absent in granuloma area and were engulfed by multinucleated giant cells. Based on the clinical and histopathological findings, a diagnosis of AEGCG was made. The etiology and pathogenesis of this condition are unclear, and atypical manifestations of non-exposed areas can also occur.It is usually related to systemic diseases lacks specific treatment at present.

Histopathological comparison of lesional and perilesional skin in melasma: A cross-sectional analysis

Background: Melasma is a common acquired hyperpigmentary disorder of the sun exposed skin, especially the face. The pathogenesis is unclear but interplay between genetic factors, hormones and ultraviolet radiation is important. We have evaluated the histological characteristics of melasma and compared the findings with adjacent normal skin. Methods: Skin biopsies were taken from both melasma and the surrounding perilesional normal skin in 50 Indian women. The sections were stained with hematoxylin and eosin, Fontana–Masson and Verhoeff–Van Gieson stains. Results: Biopsy from melasma showed significant epidermal atrophy, basal cell hyperpigmentation and solar elastosis when compared with the perilesional skin. We found that the proportion of pendulous melanocytes was significantly higher in the lesional biopsy compared with the perilesional biopsy (76% vs 42%, P < 0.001). Similarly, pigmentary incontinence and features of solar elastosis were significantly higher in the lesional skin compared with the perilesional skin. Conclusion: The characteristic histopathological features such as epidermal atrophy, basal cell hyperpigmentation and solar elastosis suggest the role of chronic sun exposure in the pathogenesis of melasma. Presence of pendulous melanocytes is a characteristic feature of melasma. The presence of pendulous melanocytes may have prognostic implications in melasma.

Fibroelastolytic Papulosis of the Chest / 대한피부과학회지

Fibroelastolytic papulosis is an acquired elastic tissue disorder that includes the clinicopathological features of white fibrous papulosis of the neck (WFPN) and pseudoxanthoma elasticum-like papillary dermal elastosis (PXE-PDE). It is clinically characterized by discrete white papules occurring on the neck in middle-aged women and is histopathologically characterized by elastolysis and/or fibrosis in the upper dermis. An 80-year-old woman presented with multiple white papules on her anterior chest, which occurred 6 months prior to the presentation. Histopathological examination of a biopsy specimen revealed some degree of fibrosis and elastolysis in the upper dermis. Thus, the final diagnosis in this case was compatible with fibroelastolytic papulosis considering that this patient showed clinicopathological features of both WFPN and PXE-PDE. Notably, this is the first case report that describes fibroelastolytic papulosis confined exclusively to the anterior chest.

Elastosis en la queilitis actínica. Revisión de literatura / Elastosis in Actinic Cheilitis. Literature Review

Resumen La matriz extracelular (MEC) juega un papel importante en la regulación de los eventos biológicos, tales como, el desarrollo de la migración celular, proliferación y diferenciación. La exposición crónica a la luz ultravioleta (UV) provoca elastosis (en distintos grados), que corresponde a una degeneración basófila de la MEC. La queilitis actínica (QA) es una lesión potencialmente maligna del labio inducida por la exposición regular y prolongada a la luz UV, que afecta principalmente al bermellón del labio inferior. Las lesiones de QA tienen un estroma complejo, se observa siempre la presencia de elastosis, infiltrado inflamatorio crónico de distinta intensidad y la aparición de vasos sanguíneos telangiectásicos. Dentro de este infiltrado inflamatorio se ha descrito un aumento significativo de mastocitos (MCs), localizados especialmente alrededor de las zonas de elastosis y en la zona subepitelial. Se ha propuesto que la elastosis actínica se produce tanto por procesos degenerativos como de síntesis anormal de fibras elásticas por parte de fibroblastos con daño solar, lo que va acompañado de cambios morfológicos del colágeno. A pesar de que el fibroblasto tendría un rol preponderante en la formación de la elastosis actínica, diversos estudios sugieren que otros tipos celulares como el MC también contribuirían en forma significativa al daño actínico de la MEC. El propósito de esta revisión es analizar las características de la elastosis en la QA.

Abstract The extracellular matrix (ECM) plays an important role in the regulation of biological events, such as cell migration, proliferation and differentiation. Chronic exposure to ultraviolet (UV) light causes elastosis (to varying degrees), which corresponds to a basophilic degeneration of the ECM. Actinic cheilitis (AC) is a potentially malignant lip lesion induced by regular and prolonged exposure to UV light, which mainly affects the vermilion. AC lesions have a complex stroma characterized by the presence of elastosis, chronic inflammatory infiltrate of different intensity and the appearance of telangiectatic blood vessels. Within this inflammatory infiltrate a significant increase of mast cells (MCs) has been described, located especially around areas of elastosis and at the subepithelial zone. It has been proposed that actinic elastosis is produced both, by degenerative processes and by abnormal synthesis of elastic fibers by photodamaged fibroblasts, which is accompanied by morphological changes in collagen. Although the fibroblast would play a major role in actinic elastosis formation, several studies suggest that other cell types such as MCs also contribute significantly to actinic ECM damage. The purpose of this review is to discuss the characteristics of elastosis in AC.

Occurrence of squamous cell carcinoma in Milchschaf sheep in Uruguay / Ocorrência do carcinoma de células escamosas em ovinos Milchschaf em Uruguai

ABSTRACT: In this study, the epidemiological and pathological features of an outbreak of squamous cell carcinoma (SCC) in adult female sheep and a young ram, Friesian Milchschaf breed or crossbreed is described. Seven adult females belonging to an original flock nucleus of 20 animals were affected, as well as a young ram belonging to the same nucleus was affected in other establishment. Multiple verrucous neoplastic masses of several months of evolution were evident in the head region, with local invasion and regional lymph node metastases. Histological studies revealed a cancerous stage of invasion of the dermis and neoplastic proliferation characteristic of invasive SCC, and a pre-cancerous stage with solar elastosis and chronic solar keratosis lesions induced by sunlight. Decrease in latitude, increased level of solar radiation with long exposure to sunlight, and the phenotypic characteristics of the breed are the main factors responsible for the high prevalence of SCC, showing the susceptibility of the Friesian Milchschaf breed and crossbreed in temperate zones as Uruguay.

RESUMO: Neste estudo descrevem-se as características epidemiológicas e patológicas de um surto de carcinoma de células escamosas (SCC) em ovelhas adultas e ram jovem, raça Friesian Milchschaf ou cruza. Foram afetadas sete fêmeas adultas pertencentes a um núcleo de rebanho original de 20 animais, bem como um ram jovem pertencente ao mesmo núcleo em que foi afetado em outro estabelecimento. Múltiplas neoplásicas verrucosas de vários meses de evolução foram evidentes na região principal, com invasão local e metástases de linfonodos regionais. Estudos histológicos revelaram um estágio cancerígeno de invasão da derme e proliferação neoplásica característica do SCC invasivo e um estágio pré-cancerígeno com elastose solar e lesões de ceratose solar crônica induzidas pela luz solar. A diminuição da latitude, o aumento do nível de radiação solar com longa exposição à luz solar e as características fenotípicas da raça são os principais fatores responsáveis pela alta prevalência de SCC, mostrando a susceptibilidade da raça Friesian Milchschaf e cruzada em zonas temperadas como o Uruguai.

D-penicillamine Induced Elastosis Perforans Serpiginosa / 대한피부과학회지

No abstract available.

Elastosis perforante serpiginosa y pseudo-pseudoxantoma elástico secundarias y tratamiento con D- penicilamina en paciente con enfermedad de Wilson / Elastosis perforans serpiginosa and pseudo-pseudoxanthomaelasticum secondary to D-penicillamine in a patient with Wilson’s disease

La D-penicilamina es la opción terapéutica más utilizada en la enfermedad de Wilson, rara enfermedad genética, autosómica recesiva, en la cual existe una alteración en el metabolismo del cobre que se deposita en los tejidos (hígado, encéfalo y córnea). Presenta numerosos efectos adversos, la mayoría cutáneos, que se observan cuando la droga es utilizada en altas dosis y por largo tiempo; entre ellos se encuentran las dermatosis degenerativas, que incluyen elastosis perforante serpiginosa, cutis laxa, anetodermia y pseudo-pseudoxantoma elástico (también llamado pseudoxantoma elástico símil o pseudoxantoma elástico like). Se presenta una paciente de 29 años con antecedentes de enfermedad de Wilson asociada a elastosis perforante serpiginosa y pseudo-pseudoxantoma elástico, ambas secundarias al tratamiento con D-penicilamina.

Penicillamineis the most commonly used therapeutic option in Wilson's disease.This is a rare, genetic, autosomal recessive diseasein which there is an alteration inthe metabolism of copper that is deposited in the tissues (liver, brain and cornea).It has numerous adverse effects, most of them affecting skin, but they are onlyobserved when the drug is used in high doses and for a long time, such as perforatingelastosis serpiginosa, cutis laxa, anetodermia and pseudo-pseudoxantomaelasticum (also called elasticum pseudoxantoma simil or elasticum pseudoxantomalike). We present the case of a29 year-old woman with a history of Wilson's diseaseand two concomitant degenerative dermatoses: elastosis perforans serpiginosa andpseudo pseudoxanthoma elasticum, both of them, secondary to treatment with Dpenicillamine.

Solar elastosis in conjunctival squamous cell neoplasm.

Aim: To study the role of solar elastosis in the patient's with Conjunctival Squamous Cell Neoplasia (CSCN). Materials and Methods: Paraffin embedded 30 Conjunctival Squamous Cell Neoplasia tissues were studied for the presence of solar elastosis by Verhoeff's stain. Nineteen Squamous Cell Carcinoma (SCC), 6 Carcinoma In Situ (CIS) and 5 Conjunctival Intraepithelial Neoplasia (CIN) specimens were included in the study. Disease free conjunctiva and pterygium tissues were used as negative and positive control respectively. Results: Solar elastosis was found to be present in 19 of 30 (63.3%) Conjunctival Squamous Cell neoplasia specimens. Conclusion: Our study showed the presence of solar elastosis in conjunctival squamous cell neoplasia. Poor socioeconomic condition is an important factor as most of the elastosis positive patients were farmers.

Elastosis Perforans Serpiginosa

Elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers. This condition classically presents as small papules arranged in serpiginous or annular patterns on the neck, face, arms, or other flexural areas. While these lesions may spontaneously resolve, they often persist for longer periods of time. Though numerous treatment modalities have been described in the literature, most are not very effective. A 35-year-old man presented to Gangnam Severance Hospital with a 2-year history of skin eruptions on his neck, which were refractory to topical antifungal or steroid treatment. On examination, the patient showed multiple crusted and erythematous papules arranged in an annular pattern with central clearing. A biopsy specimen revealed acanthosis with notable transepidermal elimination of nuclear debris and eosinophilic degenerated elastic fibers from the dermis via an epidermal channel. Verhoeff-van Gieson staining showed dense clumps of altered elastic fibers in the papillary dermis. Based on these findings, a diagnosis of elastosis perforans serpisinosa was made. Treatment with topical 0.05% tretinoin application for 6 months resulted in no improvement.

A Clinicopathologic Study of Thirty Cases of Acquired Perforating Dermatosis in Korea

BACKGROUND: Acquired perforating dermatosis (APD) is histopathologically characterized by transepidermal elimination of materials from the upper dermis. APD can be divided into four diseases: Kyrle's disease, perforating folliculitis, elastosis perforans serpiginosa, and reactive perforating collagenosis. APD is usually associated with systemic diseases, especially diabetes mellitus or chronic renal failure. So far, there have only been a few Korean studies of APD, which have a limited number of patients. OBJECTIVE: The aim of this study is to evaluate the clinical and histopathologic characteristics of 30 cases of APD and to examine the association with systemic diseases. METHODS: We retrospectively reviewed the medical records and biopsy specimens of 30 patients who were diagnosed with APD. RESULTS: The mean age was 55.5 years, and the average duration of the lesion was 7.8 months. The lower extremities (73.3%) were the most frequently occurring sites of the lesion. Twenty-five patients (83.3%) had pruritus, and Koebner's phenomenon was present in 11 patients. Patients of 63.3% had at least one systemic disease. Diabetes mellitus (n=17, 56.7%) and chronic renal failure (n=10, 33.3%) were the most commonly associated conditions. Most patients received topical steroids (93.3%) and antihistamines (80.0%). The most common histopathologic type was reactive perforating collagenosis (n=23, 73.3%). CONCLUSION: In this study, most patients had a systemic association to the diseases. Therefore, we suggest that further evaluation is necessary for patients who present with APD. This includes reviewing patient's comprehensive past medical history, clinical exam, and additional diagnostic testing to check for the possibility of associated systemic diseases.

Could a Growth Spurt Cause Linear Focal Elastosis Like Striae Distensae?

Linear focal elastosis (LFE) is characterized by several asymptomatic, yellow, palpable, irregularly indurated, striae-like lines extending horizontally across the middle and lower back. A focal increase in elastic fibers is a hallmark of the disease as seen from biopsy specimens. The pathogenesis of LFE is unclear, as is the association between LFE and striae distensae (SD). However, the prevailing opinion is that LFE represents an excessive regenerative process of elastic fibers and is analogous to keloidal repair of SD. Although the timing of onset of LFE and SD was not synchronous in our patient, the triggering factor was the same, which was the growth spurt. This case is supporting the putative association between LFE and SD.

Penicillamine-induced elastosis perforans serpiginosa with abnormal "lumpy-bumpy" elastic fibers in lesional and non-lesional skin.

Four types of elastosis perforans serpiginosa (EPS) have been described in literature: 1) idiopathic EPS, 2) reactive perforating elastosis associated with connective tissue disorders, 3) in some instances of pseudoxanthoma elasticum (PXE), disease-specific calcified elastic tissue is extruded, producing a clinical picture indistinguishable from other types, may also be seen in patients undergoing hemodialysis and 4) EPS induced by long-term treatment with D-penicillamine is observed in patients suffering from Wilson's disease. Long term D-penicillamine therapy causes an alteration in the dermal elastic tissue. D-penicillamine induced EPS has a distinctive histopathologic feature - serrated appearance of elastic fibers due to perpendicular budding from their surface giving a "lumpy-bumpy" look. D-penicillamine induced elastic fiber alteration may not always manifest clinically as EPS. We report a case of D-penicillamine induced widespread alteration in skin elastic tissue with distinct histopathologic features.

Linear Focal Elastosis Following Striae Distensae: Further Evidence of Keloidal Repair Process in the Pathogenesis of Linear Focal Elastosis

Linear focal elastosis (LFE) is a rare dermal elastosis characterized by hypertrophic yellowish linear plaques and increased abnormal elastic tissues in the lumbosacral area. Although the pathogenesis of this disorder remains unknown, it may be associated with keloidal repair process (KRP) of elastic tissues in striae distensae (SD), because there have been some reported cases of LFE accompanied by SD. We herein report a 14-year-old boy with LFE following SD in the lumbar region. Our case supports the hypothesis of KRP in the pathogenesis of LFE. Immunohistochemical study for transforming growth factor-beta (TGF-beta) was negative. Therefore, we assume that the pathogenesis of KRP in LFE is different from that of keloid development, which is the TGF-beta signaling pathway.

A Case of Linear Focal Elastosis with a Family History

Linear focal elastosis is an uncommon disorder typically occurring in the back region, which clinically presents as band-like striae, having a histological focal increase in abnormal elastic fibers. Until now, linear focal elastosis occurring in patients with a family history have been rarely reported. Here, we present one such case, of linear focal elastosis which occurred in a brother and sister.

Penicillamine-induced Elastosis Perforans Serpiginosa and Cutis Laxa in a Patient with Wilson's Disease

Elastosis perforans serpiginosa (EPS) is a rare reactive perforating dermatosis that is characterized by the transepidermal elimination of abnormal elastic fibers. Penicillamine, which is one of the clear triggers for EPS, is a heavy metal chelator that is primarily used for disorders such as cystinuria and Wilson's disease. It may cause alterations in the dermal elastic tissue such as pseudo-pseudoxanthoma elasticum, acquired cutis laxa, EPS and anetoderma. Herein we present a case of cutis laxa and EPS in a 34-year-old man who was previously on a long-term, high-dose of penicillamine for Wilson's disease. The combination of EPS and cutis laxa induced by penicillamine has rarely been reported and we report the first such case in Korea.

Melasma: A clinicopathological study of 43 cases.

Melasma is a symmetrical hypermelanosis of the exposed skin characterized by brown macules on the sun-exposed areas of the skin. The present study was carried out on 43 patients to analyze the correlation between histopathological features with clinical and Wood's light examination. The study showed a clinical and histopathological discordance of 16.2%. Solar elastosis (55.8%) was the single most common histological finding apart from increased melanin concentration, epidermal flattening and dermal lymphomononuclear inflammation.

Periumbilical perforating pseudoxanthoma elasticum.

A 58-year-old, gravida 6, obese woman presented with a pruritic yellowish plaque around the umbilicus, which first appeared about 3 years ago. She also had flat yellow papules on the axilla and neck. After a burn from a heating device, a few perforating papules and a violaceous hue occurred on the plaque. Histological examination revealed pathological elastic fibers with a keratotic plug and perforation in the deep dermis, which was consistent with perforating pseudoxanthoma elasticum. This case indicates that perforating pseudoxanthoma elasticum, a variant of hereditary pseudoxanthoma elasticum may perforate due to mechanical factors.

Colitis ulcerativa y colangitis esclerosante asociadas a elastosis perforante: presentación de un caso / Ulcerative colitis and sclerosing cholangitis associated with elastosis perforans: a case review

La eliminación transepidérmica es un mecanismo mediante el cual material extracelular o fibras alteradas de la dermis se eliminan a través de la epidermis. La elastosis perforante es un padecimiento raro caracterizado por eliminación transepidérmica de fibras elásticas alteradas. Clínicamente se manifiesta como pápulas queratósicas del color de la piel, que pueden confluir formando placas de aspecto arciforme o serpiginoso. Predomina en pacientes masculinos y menores de 30 años. Se presenta, hasta nuestro conocimiento, el primer caso de un paciente masculino con colitis ulcerativa crónica inespecífica y colangitis esclerosante asociadas a elastosis perforante.

Transepidermal elimination is a mechanism by which altered dermal extracellular material is discharged through the epidermis. Perforating elastosis is a rare disease characterized by the transepidermal elimination of abnormal elastic fibers. Clinically it manifests as skin-colored keratotic papules that may coalesce and form arcuate or serpiginous plaques. It affects mostly males, predominantly younger than 30-years-old. We present a case of a male patient with inflammatory bowel disease and sclerosing cholangitis associated with perforating elastosis. To our knowledge, this is the first description of inflammatory bowel disease associated with perforating elastosis.

A Case of Pseudoxanthoma Elasticum-Like Late-onset Focal Dermal Elastosis / 대한피부과학회지

Late-onset focal dermal elastosis is a recently described disorder afflicting elderly patients with yellow papules on the neck and flexural areas. Although there are similar clinical findings to pseudoxanthoma elasticum, no stigmata of pseudoxanthoma elastimum are not evident. Histologically the papules are characterized by increased elastic fibers of normal appearance in the mid and deep dermis and no evidence of solar elastosis. Late-onset focal dermal elastosis may be caused by focal accumulation of normal appearing elastic tissue due to loss of age related homeostatic growth regulating gene control mechanism. We report a case of pseudoxanthoma elasticum-like late-onset focal dermal elastosis and suggest that this condition may be more common than has been reported.