Pigmented Nevi Research Articles

A Baby with Café-au-lait Macules and Aberrant Mongolian Spot: Report of Phakomatosis Pigmentopigmentaria

Abstract Phacomatosis pigmentovascularis (PPV) is a term used to describe the association of vascular nevi with different pigmentary nevi. There are a very few case reports describing the association of two pigmented nevi or pigmented anomalies of nevoid character. The pathogenetic mechanism of such association is hypothesized to be twin spotting. Wolf at al has coined the term phakomatosis pigmentopigmentaria for describing the association different pigmenatry nevi or nevoid disorders. We report a rare case of baby born with co-localized segmental CALMs with aberrant Mongolian spots on the trunk. Our case can be considered as an example of this term phakomatosis pigmentopigmentaria.

Epidemiological patterns and survival outcomes in 1666 cases of malignant melanomas arising from giant pigmented nevi

Epidemiological patterns and survival outcomes in 1666 cases of malignant melanomas arising from giant pigmented nevi

Photodynamic therapy for the treatment of port-wine stains in phakomatosis pigmentovascularis.

Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome. Only a few studies have reported the treatment of PPV, including a case using photodynamic therapy (PDT) to treat PPV-associated port-wine stains (PWS). To investigating the efficacy and adverse effects of hemoporfin-PDT in PPV-associated PWS. The efficacy and adverse effects in patients with PPV who underwent two sessions of hemoporfin-PDT from January 2019 to December 2022 were retrospectively analyzed. Twenty patients were included (13 females, 7 males, age range: 2-31 years; mean: 8.20 ± 8.92 years). Two, nine, seven, and two patients had PPV types Ia, IIa, IIb, and IIIa, respectively. After two treatments, the visual evaluation indicated the color of the PWS in 4, 5, 6, and 5 patients showed poor, fair, good, and excellent improvements, respectively. The combined good and excellent improvement rates in patients with PWS and pigmentary nevus overlapping in the same treatment area and in patients with PWS in the treatment areas only were 33.3% versus 87.5%, respectively, and were significantly different (p = 0.02). Minor side effects, such as edema, scabbing, hyperpigmentation, and blistering, were observed in some patients after PDT. Hemoporfin-PDT is an effective treatment for PPV-associated PWS. Patients with PWS and pigmentary nevus overlapping in the same treatment area showed poorer efficacy than patients with PWS in the treatment areas only.

Type I Diabetes Mellitus Suppresses Experimental Skin Carcinogenesis.

This study explores the previously uncharted territory of the effects of ultraviolet (UV) radiation on diabetic skin, compared to its well-documented impact on normal skin, particularly focusing on carcinogenesis and aging. Employing hairless SKH-hr2, Type 1 and 2 diabetic, and nondiabetic male mice, the research subjected these to UV radiation thrice weekly for eight months. The investigation included comprehensive assessments of photoaging and photocarcinogenesis in diabetic versus normal skin, measuring factors such as hydration, trans-epidermal water loss, elasticity, skin thickness, melanin, sebum content, stratum corneum exfoliation and body weight, alongside photo documentation. Additionally, oxidative stress and the presence of hydrophilic antioxidants (uric acid and glutathione) in the stratum corneum were evaluated. Histopathological examination post-sacrifice provided insights into the morphological changes. Findings reveal that under UV exposure, Type 1 diabetic skin showed heightened dehydration, thinning, and signs of accelerated aging. Remarkably, Type 1 diabetic mice did not develop squamous cell carcinoma or pigmented nevi, contrary to normal and Type 2 diabetic skin. This unexpected resistance to UV-induced skin cancers in Type 1 diabetic skin prompts a crucial need for further research to uncover the underlying mechanisms providing this resistance.

Clinical study on the role of platelet-rich plasma in human acellular dermal matrix with razor autologous skin graft repair of giant congenital pigmented nevus in children

BackgroundNA ObjectiveEvaluate the safety and feasibility of platelet-rich plasma (PRP) in the treatment of giant congenital melanocytic nevi (GCMN) in children with human acellular dermal matrix (HADM) transplantation. Patients and MethodsA total of 22 children with GCMN were included in the study. They were divided into an experimental and a control group. The experimental group used the method of HADM with Razor Autologous Skin Graft combined with PRP to repair skin and soft tissue defects after giant nevus resection (Group A, n = 11). The control group was treated with HADM with Razor Autologous Skin Graft (Group B, n = 11) only. To compare the survival rate of skin grafts, we used the Vancouver Scar Scale (VSS) for the postoperative skin graft area and the Patient and Observer Scar Assessment Scale (POSAS) to compare the two groups of patients. ResultsThere was no statistically significant difference in age, gender, location of giant nevi, and pathological classification between Group A and Group (P > 0.05). The survival rate of skin grafting and the VSS and POSAS scores of scar tissue in group A were superior to those of group B (P < 0.05). ConclusionsPRP has improved the survival rate of composite skin grafting in children with GCMN, and long-term satisfactory prognosis of scar healing. Therefore, we consider this treatment method a valuable contribution to clinical practice.

Choroidal Nevus Associated with Vitelliform Deposition in a Patient with Autosomal Dominant Best Dystrophy.

To describe the clinical, optical coherence tomography (OCT), fundus autofluorescence and ultrasound findings of a patient with a choroidal nevus actively exuding vitelliform material in the setting of Autosomal Dominant Best dystrophy (BD). The patient's clinical course was followed over time with ophthalmic examinations and multimodal imaging. A 71-year-old male with BD was referred for evaluation of a choroidal nevus in the right eye. Dilated fundoscopic exam showed a small pigmented choroidal nevus in the temporal periphery. Over a 3-year period the nevus developed progressive deposition of vitelliform material along its inferior border. Meanwhile, OCT and fundus photography showed only slight growth. Ultrasound showed no change in height; basal measurements were confounded by the increased vitelliform deposits. Genetic testing confirmed a heterozygous mutation in the BEST1 gene and electrophysiology was consistent with BD. Dysfunction of the retinal pigment epithelium associated with BD may cause novel presentations of other conditions such as choroidal nevi. The implications for malignant transformation of a choroidal nevus associated with vitelliform deposit accumulation in this context is unknown.

Vitamin D status in children with congenital melanocytic nevi.

Congenital melanocytic nevi (CMN) are rare, pigmented birthmarks that can predispose patients to melanoma of the central nervous system and skin. Data from non-CMN melanoma cohorts suggest that vitamin D levels may be connected to outcome, prompting this study of 25-hydroxyvitamin D levels in plasma samples from 40 children with CMN. While 27% were insufficient and 13% deficient, this was representative of European populations, and UK supplementation guidelines are already in place. Our data support routine vitamin D supplementation for all CMN patients during winter months, without routine serum measurement.

Polarization-Diversity Optical Coherence Tomography Assessment of Choroidal Nevi.

The purpose of this study was to demonstrate the utility of polarization-diversity optical coherence tomography (PD-OCT), a noninvasive imaging technique with melanin-specific contrast, in the quantitative and qualitative assessment of choroidal nevi. Nevi were imaged with a custom-built 55-degree field-of-view (FOV) 400 kHz PD-OCT system. Imaging features on PD-OCT were compared to those on fundus photography, auto-fluorescence, ultrasound, and non-PD-OCT images. Lesions were manually segmented for size measurement and metrics for objective assessment of melanin distributions were calculated, including degree of polarization uniformity (DOPU), attenuation coefficient, and melanin occupancy rate (MOR). We imaged 17 patients (mean age = 69.5 years, range = 37-90) with 11 pigmented, 3 non-pigmented, and 3 mixed pigmentation nevi. Nevi with full margin acquisition had an average longest basal diameter of 5.1mm (range = 2.99-8.72mm) and average height of 0.72mm (range = 0.37mm-2.09mm). PD-OCT provided clear contrast of choroidal melanin content, distribution, and delineation of nevus margins for melanotic nevi. Pigmented nevi were found to have lower DOPU, higher attenuation coefficient, and higher MOR than non-pigmented lesions. Melanin content on PD-OCT was consistent with pigmentation on fundus in 15 of 17 nevi (88%). PD-OCT allows objective assessment of choroidal nevi melanin content and distribution. In addition, melanin-specific contrast by PD-OCT enables clear nevus margin delineation and may improve serial growth surveillance. Further investigation is needed to determine the clinical significance and prognostic value of melanin characterization by PD-OCT in the evaluation of choroidal nevi.

Scleritis in children: Etiology, pathogenesis, clinical features, diagnostic, and treatment algorithm

AIM: To analyze the etiology and pathogenesis and identify clinical and diagnostic features, development of a diagnostic algorithm, and personalized treatment of scleritis in children.&#x0D; MATERIALS AND METHODS: Twelve children with mono- and bilateral scleritis with disease duration of 39 months were observed. Biomicroscopy, ophthalmoscopy, and ultrasonography of the eyes were performed. The examination plan included consultations with a rheumatologist, otorhinolaryngologist, and dentist and laboratory blood analysis in the enzyme immunoassay to detect the presence of IgG and IgM antibodies to herpes viruses and markers of their reactivation.&#x0D; RESULTS: Chronic scleritis in 58.4% of the patients was associated with immunoinflammatory rheumatic diseases: 41.7% with juvenile idiopathic arthritis and 16.7% with psoriatic arthritis. In some cases scleritis was associated with chickenpox, surgical treatment of congenital pigmented nevus of the skin of eyelids,n on the conjunctiva and oculomotor muscles, otogenic neuritis of the facial nerve. Сlinical features of anterior deep scleritis and symptoms of bacterial scleritis are described. Personalized schemes for the diagnosis and treatment of scleritis in children have been developed. Conservative treatment included instillation of glucocorticoids and non-steroidal anti-inflammatory drugs. In addition, to anti-inflammatory therapy, antibacterial drugs are prescribed only in the presence of clinical signs of bacterial scleritis; in other cases, their use is inappropriate. The indications for antiviral therapy included laboratory confirmation of herpes infection reactivation. Personalized etiotropic therapy made it possible to achieve remission of scleritis in 914 days.&#x0D; CONCLUSION: This study analyzed the etiopathogenesis of scleritis, described the characteristic clinical features of anterior deep scleritis in children, and developed personalized diagnostic and treatment schemes.

A pigmented hairy nevus with limb reduction

A pigmented hairy nevus with limb reduction

CLINICAL CASE OF RECURRENT NEVUS

Abstract. Introduction. Recurrent nevus is a benign proliferation of melanocytes that occurs at the site of non-radical removal of a previously existing nevus. Recurrence rates range from 0.3% to 28% and most often occur within 6 weeks to 6 months after removal. Clinically recurrent nevus is characterized by uneven pigmentation with uneven edges in the area of the scar.&#x0D; Walton et al. began researching the phenomenon of recurrent nevus in 1957. In 1975, Kornberg and Ackerman reported that a pigmented recurrent nevus can resemble superficially spreading melanoma both clinically and histologically, and proposed the term pseudomelanoma to describe this benign phenomenon. Currently, the term is rarely used, but it motivates a thorough examination of patients with such a problem.&#x0D; Case presentation. Patient A. complained about the presence of a formation in the area of the outer surface of the right forearm, which has existed for many years, it was injured several times, but has recently changed the colour. On examination, a nodule up to 0.5 cm in size, heterogeneously pigmented, with peripheral erythema was found in the indicated place. During dermoscopy, chaotic unevenly distributed pigmentation, consisting of dots, globules, forming a cellular structure in a scattered manner is determined, zones of segmental radial lines are present as well. The vascular pattern in the central part of the formation is not defined, on the periphery it is represented by slightly noticeable point vessels. Digital post-processing using machine learning technology was carried out to improve visualization of blood supply. Multiple point, curved, and convoluted vessels with few branches around the formation, and several central vessels in the pigment cells are determined. Surgical removal of the tumor was performed with involvement of the erythema zone, taking into account the peculiarities of pigmentation and vascular pattern. The pathogistological structure is presented in the photomicrograph. The epidermis has a typical structure, with areas of slight acanthosis; hyperpigmentation of the basal layer is also observed. In the dermis, foci of brown pigment deposition, single lymphocytes, there are areas of intradermal lobular-horizontal fibrosis with angiomatosis and scattered lymphocytic infiltration. Taking into account the clinical data, there is a recurrence of a melanocytic nevus on the background of a skin scar (Recurrent neavus).&#x0D; Teaching point. Dermoscopy allows obtaining additional data for the differential diagnosis of recurrent nevus and recurrent melanoma. A recurrent nevus is characterized by relative symmetry of formation and a pattern of segmental radial lines. The formation is limited to the area of the scar and does not go beyond it. Also, in the case of recurrent melanoma, a continuous growth in dynamics and a later appearance of recurrence from the moment of intervention will be obvious.&#x0D; Conclusions. As clinical practice demonstrates, under certain circumstances, recurrent pigmentation can be observed in cases of nevi that have been traumatized. It will be visible both dermoscopically and pathohistologically. Sometimes it is difficult even for experts to distinguish between a highly irritated nevus and a melanoma.

Combining hyperspectral imaging techniques with deep learning to aid in early pathological diagnosis of melanoma

BackgroundCutaneous melanoma, an exceedingly aggressive form of skin cancer, holds the top rank in both malignancy and mortality among skin cancers. In early stages, distinguishing malignant melanomas from benign pigmented nevi pathologically becomes a significant challenge due to their indistinguishable traits. Traditional skin histological examination techniques, largely reliant on light microscopic imagery, offer constrained information and yield low-contrast results, underscoring the necessity for swift and effective early diagnostic methodologies. As a non-contact, non-ionizing, and label-free imaging tool, hyperspectral imaging offers potential in assisting pathologists with identification procedures sans contrast agents. MethodsThis investigation leverages hyperspectral cameras to ascertain the optical properties and to capture the spectral features of malignant melanoma and pigmented nevus tissues, intending to facilitate early pathological diagnostic applications. We further enhance the diagnostic process by integrating transfer learning with deep convolutional networks to classify melanomas and pigmented nevi in hyperspectral pathology images. The study encompasses pathological sections from 50 melanoma and 50 pigmented nevus patients. To accurately represent the spectral variances between different tissues, we employed reflectance calibration, highlighting that the most distinctive spectral differences emerged within the 500–675 nm band range. ResultsThe classification accuracy of pigmented tumors and pigmented nevi was 89% for one-dimensional sample data and 98% for two-dimensional sample data. ConclusionsOur findings have the potential to expedite pathological diagnoses, enhance diagnostic precision, and offer novel research perspectives in differentiating melanoma and nevus.

Problems and prospects in the treatment of various forms of pigmented nevi in children (a literature review)

Introduction. Pigmented nevi (PN) are formations in the skin layers which consist of accumulated melanocytes of various differentiation degrees and located in different layers of the skin. In modern society, this pathology causes serious medical and psychological problems; not infrequently , one can see unsatisfactory clinical and aesthetic outcomes. There is no any clear tactics which could take into account PN forms, location and child’s age. Purpose. To study modern problems and to outline prospects for treating various PN forms in children using the information obtained after literature search for the period 2005–2022. Material and methods. The authors made the literature search in domestic and foreign databases for the period 2005–2022. The following key words were used for the search: benign melanocytic skin lesions; pigmented nevus; laser radiation. 1627 literature sources were studied, 87 works were included in the review. Results. Despite of various techniques used for PN removal, unsatisfactory results are still noted in 41 % of patients with a complication rate of up to 50 %. After implementation laser scalpel for PN removal, complications in the form of hypertrophic and keloid scars were noted in 2–5 % of patients, postoperative hyperemia- in 21 % of cases, hyperpigmentation in 13 % and hypopigmentation in 34 %. The present review outlines advantages and disadvantages of various modalities used for removing PN. The authors also underline that the most promising ones are laser techniques in which laser light has a specific selectivity to pigmented tissues. The article has no information on parameters of laser irradiation which are required for treating definite forms of PN in children. Conclusion. Further research is needed to optimize existing and to develop new laser light techniques for treating various PN forms which should bring a significant improvement in outcomes.

Functional Involvement of ADRA1D in Cutaneous Melanoma Progression and Angiogenesis.

Cutaneous melanoma is a highly aggressive and malignant skin cancer, and its high recurrence rate and drug resistance increase the difficulty of treating advanced-stage patients. Studies have revealed that treatment via stimulation of alpha-1 adrenergic receptor (ADRA1) subtypes inhibits melanoma growth in mice. However, the associations between alpha-1D adrenergic receptor (ADRA1D) and cutaneous melanoma are poorly understood. Tissue specimens from 16 pairs of patients with a pigmented nevus and cutaneous melanoma were analyzed for ADRA1D expression using immunohistochemical staining. Western blotting and RT-qPCR were carried out in order to detect ADRA1D expression levels in melanoma cells and human epidermal melanocytes (HEMs), hypoxia-inducible factor-1α (HIF-1α), and vascular endothelial growth factor (VEGF) levels in HUVECS. A375 cells were transfected with a lentivirus overexpressing ADRA1D. Wound-healing, Transwell, and cell proliferation assays were utilized to identify the ADRA1D effect on the migration, invasion, and proliferation of the two groups of A375 cells in vitro. In order to evaluate the function of ADRA1D in vivo, a melanoma xenograft model was developed in immunodeficient mice. ADRA1D was low expressed in cutaneous melanoma tissues. Overexpression of ADRA1D inhibited the tubulation and migration of HUVECs in vitro. Overexpression of ADRA1D significantly decreased the HIF-1α and VEGF expression. Overexpression of ADRA1D inhibited the invasion and proliferation of A375 melanoma cells in vitro and reduced its angiogenesis in vivo. ADRA1D inhibits cutaneous melanoma growth and angiogenesis. It attenuates melanoma cell proliferation and invasion. Meanwhile, its anti-angiogenic effect is achieved by negatively regulating the HIF-1α/VEGF axis in melanoma tissue, thereby attenuating the growth of cutaneous melanoma and reducing the potential of metastasis.

Morphological characterization of nevi on the caruncle conjunctiva under in vivo confocal microscopy.

This study aimed to investigate the microscopic structure and characteristics of nevi on the conjunctiva of the lacrimal caruncle by in vivo confocal microscopy. In total, four patients with nevi growing on the lacrimal caruncle conjunctiva were recruited. The morphological characteristics of the nevi were evaluated by in vivo confocal microscopy before excision surgery; the results were compared with histopathological analyses of the surgical specimens. The nevi of the four patients were all located at the conjunctiva of the lacrimal caruncle, with a slightly nodular surface, mixed black and brown color, and clear boundary. The nevi were round and highly protruded on the surface of the lacrimal caruncle, with an average diameter of 4.5 ± 1.29 mm. Under in vivo confocal microscopy, the pigmented nevus cells on the conjunctiva of the lacrimal caruncle were observed to be clustered in nests with irregular boundaries. The cells were round or irregular, with clear cell boundaries, hyper-reflective at the periphery, with low reflectivity in the center. Vascular crawling was observed in some regions. Histopathological analysis showed that nevus cells were roughly equal in size and distributed in a nodular pattern. Melanin granules were observed in the cytoplasm. No atypia or mitotic figures of the cells were found. This study revealed that the microstructure of nevi growing on the conjunctiva of the lacrimal caruncle can be identified by in vivo confocal microscopy.

The Gingival Mole – Rare Case Report

Pigmented Nevi are benign tumors of the skin and mucosa characterized by the presence of melanin-producing, neuroectodermal derived nevus cells. Pigmented lesions usually pose an aesthetic concern to the patients and hence a clinical expertise is of utmost requirement for appropriate diagnosis and treatment for improved esthetics and patient satisfaction.

Noninvasive imaging exploration of phacomatosis pigmentokeratotica using high-frequency ultrasound and optical coherence tomography: Can biopsy of PPK patients be avoided?

Phacomatosis pigmentokeratotica (PPK) is a distinct and rare type of epidermal nevus syndrome characterized by coexisting nonepidermolytic organoid sebaceous nevus (SN) with one or more speckled lentiginous nevi (SLN). Atypical nevi including compound Spitz and compound dysplastic may manifest within regions of SLN. Patients with PPK, or similar atypical nevus syndromes, may be subject to a significant lifetime number of biopsies, leading to pain, scarring, anxiety, financial burden, and decreased quality of life. The current literature includes case reports, genetics, and associated extracutaneous symptoms of PPK, but use of noninvasive imaging techniques have not been explored. We aim to investigate the value of high-frequency ultrasound (HFUS) and optical coherence tomography (OCT) in discriminating morphological features of pigmented lesions and nevus sebaceous within one patient with PPK. Two modalities, (1) HFUS imaging, based on acoustic properties and (2) OCT imaging, based on optical properties, were used to image a patient with PPK. Benign pigmented lesions, which may raise clinical suspicion for significant atypia, and nevus sebaceous, were selected on different areas of the body to be studied. Five pigmented lesions and one area of nevus sebaceous were imaged and analyzed for noninvasive features. Distinct patterns of hypoechoic features were seen on HFUS and OCT. HFUS provides a deep view of the tissue, with ability to differentiate gross structures beneath the skin. OCT provides a smaller penetration depth and a higher resolution. We have described noninvasive features of atypical nevi and nevus sebaceous on HFUS and OCT, which indicate benign etiology.

Stardust Pattern as Evolution of Pigmented Spitz Nevi During Childhood.

Spitz nevi (SN) are benign melanocytic proliferations frequently occurring in children. Some pigmented SN with a starburst pattern evolve into the "stardust" one, which is characterized by a central, black to gray, hyperpigmented area and remnants of a brown network at the periphery. These dermoscopy changes are often the first alert to induce excision. The aim of this study is to enlarge the case series of stardust SN in children, in order to increase confidence with this new dermoscopic pattern and reduce unnecessary excisions. This retrospective observational study was conducted with SN cases received from IDS members. The inclusion criteria were: clinical and/or histopathologic diagnosis of Spitz naevus with starburst appearance in children <12 years old, availability of a dermoscopic image at baseline and after follow-up of at least 1 year, availability of patient data. The dermoscopic images and their changes over time were assessed by three evaluators in consensus. 38 SN were enrolled, with a median age of 7 years and a median FUP duration of 15,5 months. Comparing the evolution with time of FUP, no significant differences were found between growing and involuting lesions in terms of patient age and sex, location and palpability of lesions. The long follow-up reported in our study could really support the concept of benignity of changing SN. A conservative approach is acceptable for nevi showing the stardust pattern, because it may be considered a physiological evolution of pigmented Spitz nevus, and urgent surgeries could be avoided.

Provocation of Pigment (Melanocytic) Nevus Occurrence by Tattoo Procedure

Provocation of Pigment (Melanocytic) Nevus Occurrence by Tattoo Procedure

Dermoscopic Findings of the Evolving Pigmented Spitz Nevus in a Child.

According to the Authors Guidelines in case of image letters no abstract should be provided.