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Abstract Secondary osteoma cutis is a phenomenon that may occur in several conditions. When it occurs in a melanocytic nevus it is named osteonevus of Nanta, an event considered uncommon and characterized by the presence of bone formation adjacent or interposed with melanocytic cells. There are reports of its occurrence in various melanocytic lesions, being more frequently associated with intradermal nevus. We report a case of osteonevus of Nanta in combined nevus, possibly the first description of this association.

Pigmented epithelioid melanocytoma is a rare, newly described melanocytic tumor that encompasses lesions previously classified as animal type melanomas and epithelioid blue nevus of the Carney complex. Pigmented epithelioid melanocytoma is a specific clinicopathological entity with particular clinical presentation and histological features. We present the case of a 5 year old female patient with a heavily pigmented papule on her right thigh that showed histological findings compatible with pigmented epithelioid melanocytoma and discuss the relevance /clinical significance of sentinel lymph node biopsy as a staging procedure in this particular neoplasm.

Pediatric melanoma of the scalp has the highest mortality of any anatomic location. We describe five pediatric patients with a diagnosis of scalp melanoma receiving care at Massachusetts General Hospital and/or Boston Children's Hospital from 2018 through 2022. Melanoma presented in diverse contexts: cellular blue nevus-associated, compound nevus-associated, spitzoid, nodular, and superficial spreading subtypes. This study describes a range of melanoma presentations and emphasizes the need for additional compilation of data on pediatric scalp melanomas to promote their recognition and improve patient care., (© 2023 Wiley Periodicals LLC.)

Abstract: Blue nevus is a benign melanocytic lesion, typically asymptomatic and of unknown etiology. Many histological subtypes are recognized, the most commons being: common blue nevus, cellular blue nevus, and combined blue nevus. New rare variants have been described in the literature, with emphasis on eruptive blue nevus, plaque, agminate, linear, with satellitosis, disseminated, familial and targetoid. The diagnosis of blue nevus usually presents no difficulties, however, the presence of structures such as irregular edges or satellitosis, are highly suggestive of malignancy, and the differential diagnosis with malignant blue nevus and melanoma with peripheral spread should be considered. We report a case of blue nevus with satellitosis in a 15-year-old female patient.

Abstract: Blue nevus is a benign melanocytic lesion whose most frequent variants are dendritic (common) blue nevus and cellular blue nevus. Atypical cellular blue nevus presents an intermediate histopathology between the typical and a rare variant of malignant blue nevus/melanoma arising in a cellular blue nevus. An 8-year-old child presented a pigmented lesion in the buttock since birth, but with progressive growth in the last two years. After surgical excision, histopathological examination revealed atypical cellular blue nevus. Presence of mitoses, ulceration, infiltration, cytological atypia or necrosis may occur in atypical cellular blue nevus, making it difficult to differentiate it from melanoma. The growth of blue nevus is unusual and considered of high-risk for malignancy, being an indicator for complete resection and periodic follow-up of these patients.

A 38-year-old Hispanic man without comorbidities presented to our dermatology clinic for the evaluation of an asymptomatic dark macule on his left hand, which had gradually grown since he was a child. The hyperpigmentation involved the dorsum and palm (Figure 1). The patient was right-handed and denied previous trauma, inflammation, occupational exposure to chemicals, or using any medications. During physical examination, no other similar pigmentation was found on the rest of his body. An incisional biopsy of the left palm was performed (Figure 2). The histopathology revealed the presence of spindle-shaped cells with melanin granules in the superficial and middle dermis, surrounding the blood vessels, and between collagen bundles, which are findings compatible with acquired dermal melanocytosis (1,2). On dermoscopy, we found a pattern of regular pigment with a gray-brown tone and whitish spots within. We discussed the benignity of this rare entity with the patient, and he decided not to pursue treatment. Acquired dermal melanocytosis (ADM) is a rare condition, with isolated presentation on the hand and with less than 10 cases reported (1). Dermal melanocytosis includes several benign pigmented lesions histologically characterized by the presence of melanocytes in the dermis, which are spindle-shaped dendritic cells containing brown melanin pigment. Melanocytes can also be identified with immunoperoxidase staining for S100 and Fontana-Masson melanin stain (2). The physiopathology of ADM remains unclear, but it has been proposed that it involves reactivation of latent dermal melanocytes due to external factors such as trauma, inflammation, chemical exposure, sunlight, drugs, and hormonal treatment with estrogen and/or progesterone (3). ADM with hand involvement usually appears in the Asian population without sex predilection. The lesions develop in adolescence or young adulthood and tend to affect both hands and other body areas such as the face or the legs; there have also been two reported cases in the Hispanic population (both by Fitzpatrick III) (3,4). ADM must be differentiated from ectopic Mongolian spots, plaque-type blue nevi, tinea nigra, or other pigmented neoplasms. A biopsy is mandatory to establish a proper diagnosis. Ectopic Mongolian spots and plaque-type blue nevi are both congenital dermal melanocytoses that may present as bluish macules on the hand. However, these lesions show deep and more widely scattered distribution of melanocytes (1). There have also been some reports of malignant melanoma and acquired dermal melanocytosis that appeared on congenital nevus spilus (5). ADM is a benign condition, and reassurance should be offered to these patients.

Blue rubber bleb nevus syndrome (BRBNS) is a rare condition characterised by vascular malformations mostly of the skin and gastrointestinal tract and less commonly of the central nervous system, liver, thyroid, spleen and lungs. We report a rare case of BRBNS in a patient on anticoagulation who presented with gastrointestinal bleeding and no cutaneous or other organ involvement. We discuss the difficulty in balancing bleeding and clotting risks in this patient who developed two episodes of venous thromboembolism while off anticoagulation to minimise gastrointestinal bleeding. We also highlight the potential role of somatostatin analogues such as lanreotide in decreasing gastrointestinal bleeding risk in BRBNS, particularly in the setting of anticoagulation. The occurrence of two episodes of venous thromboembolism within a short time frame in this case, in conjunction with known associations between other vascular anomalies and venous thromboembolism, raises the question of whether BRBNS may be associated with a prothrombotic state., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Gastrointestinal bleeding of obscure origin accounts for less than 5% of gastrointestinal hemorrhages. It is typically difficult to diagnose due to limited accessibility through standard endoscopic techniques and generally requires a significant number of procedures to reach a diagnosis. The "blue rubber bleb nevus syndrome" is a rare condition, of a probably hereditary origin, characterized by the presence of multiple hemangiomatous lesions, which can manifest as gastrointestinal bleeding of obscure origin. These lesions are generally nodular, rubbery to the touch, and have a submucosal appearance, primarily affecting the skin and gastrointestinal tract. We present the case of a 72-year-old male who was investigated for iron deficiency anemia with upper and lower gastrointestinal endoscopies conducted on two occasions, without revealing any findings that could explain the condition. Subsequently, a study with video-capsule endoscopy was performed, which revealed multiple submucosal and vascular lesions, measuring between 3-5 mm, located in the distal duodenum and jejunum, consistent with "Blue rubber bleb nevus syndrome".

Abstract: Blue nevi are benign melanocytic lesions located in the deeper reticular dermis, consequence of failure of melanocytic migration into the dermal-epidermal junction from the neural crest. Lesions are usually asymptomatic and solitary, but may present in a multiple or agminated (grouped) pattern. The agminated subtype is formed when bluish-pigmented lesions cluster together in a well-defined area. Lesions can be flat or raised. We report the case of a patient who presented multiple bluish macules (1-3 mm in diameter) grouped on the left upper back. Dermoscopy and anatomic pathological examination were consistent with blue nevus.

Argyria is a rare disease caused by prolonged skin contact with silver. Localized cases have been described regarding the use of topical medications, and trauma with objects containing this metal such as acupuncture needles and jewelry. Clinically, a macule or a patch, round or oval, appears in the infected area, with a characteristic bluey-gray color. To our knowledge this is the first time that this clinical condition has been described through the use of dermoscopy.A argíria é uma doença rara, causada pelo contato prolongado da pele com prata. Foram descritos casos localizados relacionados ao uso de medicamentos tópicos e traumas com objetos que contem esse metal, como agulhas de acupuntura e jóias, por exemplo. Clinicamente, aparecem máculas ou manchas redondas ou ovais na área afetada, com uma característica cor azul-acinzentada. Até onde sabemos esta é a primeira vez que este quadro clínico foi descrito através da dermatoscopia.

O nevo é uma má-formação congênita pigmentada, raramente encontrado na mucosa bucal. Cerca de 1/3 dos casos localizados nesta região anatômica são do tipo azul, uma variante histológica com considerável tendência à malignização. Este artigo relata o caso de um paciente do sexo masculino, tabagista crônico, portador de um nevo azul de 5 cm de diâmetro no palato duro. A excisão da lesão sem biópsia prévia foi a conduta terapêutica de eleição para o caso, uma vez que ainda existe controvérsia na literatura a respeito da realização de biópsia incisional em lesões pigmentadas malignas ou com potencial de malignização. O paciente foi acompanhado por um período de 2 anos, sem sinais de recorrência ou transformação malignaNevus is a congenital pigmented malformation rarely found in the oral mucosa. Around one third of cases located in this anatomical region are of the blue type, a histological variant with considerable tendency to malignancy. This study reports the case of a male patient, chronic smoker, with a blue nevus measuring 5cm in diameter on the hard palate. Since controversy exists in the literature regarding the incisional biopsy of pigmented lesions with malignant or malignant potential, excision without previous biopsy of the lesion was the therapy of choice for this case. The patient was followed-up for two years with no sign of recurrence or malignant transformation

Background: Blue rubber bleb nevus syndrome (BRBNS) is a rare systemic vascular anomaly mainly affecting the skin, central nervous system and gastrointestinal tracts. Its clinical presentation and characteristics in adult patients are unclear., Aim: To clarify the characteristics of BRBNS in adult patients with a focus on gastrointestinal symptoms., Methods: Following the PRISMA Extension for Scoping Reviews, we searched MEDLINE and EMBASE for all peer-reviewed articles using keywords including 'Blue rubber bleb nevus syndrome' from their inception to 28 December 2021., Results: Ninety-nine articles, including 3 observational studies and 101 cases from case reports and series, were included. Observational studies were consistently with small sample sizes, and there was only one prospective study to show the effectiveness of sirolimus in BRBNS. Common clinical presentations included anemia (50.5%) and melena (26.5%). While skin findings were known to be representative of BRBNS, only 57.4% had known vascular malformation. The diagnosis was primarily made clinically, with only 1% being diagnosed with BRBNS through genetic sequencing. Distribution of BRBNS-related lesions were variable, but predominantly oral (55.9%), followed by small bowel (49.5%), colorectal (35.6%) and stomach (26.7%) vascular malformations., Conclusion: While it has been underrecognized, adult BRBNS could be a culprit for refractory microcytic anemia or occult gastrointestinal bleeding. Further studies are critical to establish a uniform understanding of diagnosis and treatment for those with adult BRBNS. The utility of genetic testing in adult BRBNS diagnosis and the patient characteristics that may benefit from sirolimus, a potentially curative agent, remain to be clarified.

A 42-year-old woman presented with a small pigmented lesion of the palpebral conjunctiva that had been present for a few months. Because of the possibility of melanoma, the lesion was resected. Microscopic examination displayed an intratarsal blue nevus at the level of the meibomian glands comprised of bland nonpigmented and pigmented cells that enveloped a sebaceous gland and its ducts. The cells were of admixed spindle and epithelioid configuration and were immunoreactive for Melan-A. The Ki67 proliferative marker was negative in these cells, contrasting with the epithelium of the overlying conjunctiva and the sebaceous ducts, and thereby militating against the diagnosis of melanoma. Clusters of melanophages were also present. Although an intratarsal blue nevus has been described as a component of a combined nevus, the current lesion demonstrates the occurrence of a sole tarsal blue nevus. Palpebral pigmented lesions should be customarily excised because many are melanomas., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2022 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

The histopathological diagnosis of melanoma is fraught with potential pitfalls. In the setting of cutaneous metastatic melanoma, it is important to recognise the various histological patterns that can be encountered from the more common to the rare, including epidermotropic, folliculotropic, naevoid, and blue naevus-like. In addition, melanoma is notorious for phenotypic plasticity. Thus, there are many different subtypes and cytomorphological variations that can be difficult to recognise as melanoma, particularly in the recurrent or metastatic setting. Select melanoma variants including primary dermal, clear cell, plasmacytoid, signet ring cell, small cell, myxoid, rhabdoid, and dedifferentiated melanoma will be discussed, in addition to composite melanocytic neoplasms. This review is intended to remind the practitioner of key concepts of metastatic disease and select rare melanoma variants, while providing practical guidelines for accurate diagnosis., (Copyright © 2022 Royal College of Pathologists of Australasia. Published by Elsevier B.V. All rights reserved.)

Combined nevi are melanocytic lesions composed of two or more distinct types of melanocytic populations with-in the same lesion. Different types of combined nevi may form bizarrely shaped, multicolored skin lesions, making them one of the greatest melanoma mimickers. We report a 48-year-old female patient with suspicious skin lesion in the left lumbar region. Clinically, there was an oval, slightly asymmetrical lesion measuring 6 x 4 mm, showing multiple colors and shades of brown and black. A dermoscopic examination revealed a brown-bluish coloration in the right part of the lesion, while a fine pigment network with perifollicular halo was found in the left part of the lesion, suggesting the diagnosis of a combined nevus. Histological examination showed a poorly circumscribed proliferation of dendritic melanocytes in the superficial and deep dermis and proliferation of melanocytes in the dermoepidermal junction. A surgical excision of the tumor was performed, in order to confirm the dermoscopic findings. In conclusion, dermoscopy is useful in differentiating combined nevi from other melanocytic lesions. [ABSTRACT FROM AUTHOR]

Carney complex is a rare familial multineoplastic syndrome predisposing to endocrine and nonendocrine tumors due to inactivating mutations of PRKAR1A, leading to perturbations of the cAMP‒protein kinase A signaling pathway. Skin lesions are the most common manifestation of Carney complex, including lentigines, blue nevi, and cutaneous myxomas in unusual locations such as oral and genital mucosa. Unlike endocrine disorders, the pathogenesis of skin lesions remains unexplained. In this study, we show that embryonic invalidation of the Prkar1a gene in steroidogenic factor-1‒expressing cells leads to the development of familial skin pigmentation alterations, reminiscent of those in patients with Carney complex. Immunohistological and molecular analyses, coupled with genetic monitoring of recombinant cell lineages in mouse skin, suggest that familial lentiginosis and myxomas occur in skin areas specifically enriched in dermal melanocytes. In lentigines- and blue nevi‒prone areas from mutant mice and patients, Prkar1a/PRKAR1A invalidation occurs in a subset of dermal fibroblasts capable of inducing, under the influence of protein kinase A signaling, the production of promelanogenic EDN3 and hepatocyte GF signals. Our model strongly suggests that the origin of the typical Carney complex cutaneous lesions is the result of noncell-autonomous promelanogenic activity of a dermal fibroblast population sharing a community of origin with steroidogenic factor-1 lineage., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

There are elements in nature that may be compared to some dermatological lesions, such as the black pearl, which is very similar to a cellular blue nevus observed in the gluteus region of a 31-year-old male patient.A natureza pode oferecer elementos passíveis de comparação com algumas lesões dermatológicas, como a semelhança entre a pérola negra e o nevo azul celular observado na região glútea de paciente do sexo masculino, 31 anos.

Purpose: To review the clinical features and treatment-associated outcomes of primary orbital melanoma among cases reported in the literature and to present a case treated with orbital exenteration and post-operative radiotherapy. Methods: Case reports and case series on primary orbital melanoma published in the literature between 1980 and 2020 were reviewed. Data collected included patient demographics, presenting ocular symptoms, diagnostic imaging, histology, management, and outcomes. Results: Eighty-eight cases of primary orbital melanoma were reviewed. The average age at presentation was 45 years and 58% of patients were male. The most common presenting symptoms and signs were proptosis (73%), decreased visual acuity (32%), pain (14%), diplopia (15%), and palpable mass (9%). Imaging frequently showed a well-circumscribed enhancing lesion. Diagnosis was made by histology in all cases, and orbital blue nevus was identified in 42%. In the majority of cases, treatment consisted of orbital exenteration (54%) or excision (38%). Adjuvant radiotherapy was given in 47% of cases. For the 72 patients with reported outcomes, 36% had metastases, 15% had local recurrence, and 32% died of metastatic disease. Patients who received surgery and radiotherapy had improved survival compared to those who received surgery alone ( p = .01). There was no difference in survival between those who underwent orbital exenteration or excision ( p = .16). Conclusions: Primary orbital melanoma is a rare malignancy and should be considered in patients with a history of unilateral proptosis and a well-defined orbital mass on imaging. Surgery remains the mainstay of treatment. Adjuvant radiotherapy may improve patient survival.

Background: Digital clubbing is a well-known clinical sign characterized by thickening of the distal phalanges of the fingers and toes. Unilateral clubbing occurs less frequently. A previous report showed for the first time two cases of unilateral clubbing as a clinical manifestation of lower limb venous malformation. The objective of the present study is to describe a series of 13 patients with a low-flow vascular malformation where a clubbing-like unilateral digital thickening is also observed., Methods: All patients were retrospectively included after reviewing clinical photographs from a vascular malformations database., Results: A total of 13 patients with low-flow vascular malformations were included in this study. The mean age at diagnosis was 11 years (range 5-26 years) with a female predominance (nine patients). The most frequent vascular malformation collected was a blue rubber bleb nevus syndrome in four patients, followed by common venous malformations in three patients. All patients characteristically exhibited a clubbing-like digital thickening. Seven patients had foot involvement and six patients hand involvement., Conclusions: Although the number of cases is limited, our study is the first series of cases where a clubbing-like digital thickening is described in patients with a low-flow vascular malformation. The unilateral presence of clubbing or pseudoclubbing should lead to the suspicion of an underlying vascular malformation., (© 2021 the International Society of Dermatology.)

Blue rubber bleb nevus syndrome (BRBNS) is a rare systemic syndrome characterized by venous malformations usually found in the skin and visceral organs. To date, 11 case reports describing BRBNS during pregnancy have been published. To our knowledge, this is the first report describing intracranial, airway, epidural, and birth canal involvement of venous malformations in the same parturient. Key lessons learned include clinical presentation, workup, team management, and care of obstetric patients with this disorder., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the International Anesthesia Research Society.)

Objective:To highlight the important clinical and histological features of sinonasal blue naevi.Methods:A case of blue naevus of the nasal cavity is described (including endoscopic and histological pictures) and the existing literature is reviewed.Results:There have been five reported cases (including the presented case). Clinically, sinonasal blue naevi are heavily pigmented lesions that are small and asymptomatic. Histopathologically, blue naevi exhibit heavily pigmented dendritic melanocytes that are never abnormal in form.Conclusion:Blue naevus should be a differential diagnosis for pigmented lesions within the sinonasal cavity, despite its rarity. It may be initially suspected by its small size and asymptomatic nature. However, histological examination is required for definitive diagnosis, looking for the key microscopic features described above. [ABSTRACT FROM PUBLISHER]