Extrafacial Acquired Dermal Melanocytosis: An Exceptionally Rare Example With Bilateral Multifocal Disease.

Dermal melanocytoses are a group of cutaneous disorders characterized by the presence of ectopic melanocytes in the dermis; the most well-known example is the Mongolian spot. Acquired dermal melanocytosis (ADM) is a term used to describe the onset of dermal melanocytosis occurring after its usual age of presentation (i.e., birth and infancy). ADMs usually occur on the face and can less commonly affect extrafacial sites, such as the back and limbs. Purely extrafacial ADM is extremely uncommon and, when present, is usually unifocal. Herein, we present an exceptionally rare example of purely extrafacial ADM with extensive bilateral involvement in a 44-year-old female originally from the Philippines.

Advancements in Laser Therapies for Dermal Hyperpigmentation in Skin of Color: A Comprehensive Literature Review and Experience of Sequential Laser Treatments in a Cohort of 122 Indian Patients.

The heightened awareness of ethnic dermatology aligns with the growing prevalence of skin of color communities globally, where hyperpigmentation disorders pose a common dermatological challenge. Effectively addressing dermal pigmentation is challenging due to its resistance to conventional therapies and its association with impaired quality of life. This underscores the need for effective treatments and a thorough grasp of laser advancements. A relevant literature search spanning the last 7 years across the PubMed database reveals core studies, challenges, and the evolution of laser technologies tailored for various forms of congenital and acquired dermal hyperpigmentation in skin of color. This comprehensive review explores the mechanisms, applications, and recommendations for pigmentary laser technologies, highlighting the key role of Q-switched lasers in their established millisecond/ nanosecond forms and emerging picosecond lasers, fractional non-ablative and ablative lasers, Intense Pulsed Light, etc. The summary of evidence includes studies on dermal melanocytosis (nevus of Ota and Hori's nevus), tattoos, acquired dermal macular hyperpigmentation, etc., and also entities with mixed epidermal-dermal components, such as melasma and post-inflammatory hyperpigmentation. The review offers valuable insights for clinicians to make informed decisions based on diagnosis, skin type, and the latest technologies to optimize results and minimize complications, especially in darker Fitzpatrick skin types. In their five-year study with 122 Indian patients, the authors applied specific laser combinations for diverse dermal melanoses, including tattoos, dermal/mixed melasma, acquired dermal macular hyperpigmentation, and dermal nevi. Substantial pigmentation reduction, subjectively assessed by both physicians and patients, was observed across all groups. A one-way ANOVA indicated a significant difference in mean improvement scores across various pigmentary conditions (F = 3.39, p = 0.02), with melasma patients exhibiting a significantly higher improvement score than tattoos (p = 0.03). The results affirmed the safety and efficacy of sequential laser therapy for dermal pigmentation in skin of color, advocating for flexibility in approach while maintaining the rationale behind the laser sequences. Despite advancements, challenges persist, and gaps in the current literature are identified. In conclusion, this summary highlights the ongoing pursuit of optimal protocols in dermatological laser treatments for dermal melanoses, offering valuable insights for future research and clinical practice.

Extensive and Persistent Dermal Melanocytosis in a Male Carrier of Mucopolysaccharidosis Type IIIC (Sanfilippo Syndrome): A Case Report.

Congenital dermal melanocytosis (DM) represents a common birthmark mainly found in children of Asian and darker skin phototype descent, clinically characterized by an oval blue-grey macule or macules, commonly located on the lumbosacral area. In rare DM cases, when presenting with diffuse macules persisting during the first years of life, it could represent a cutaneous feature of mucopolysaccharidoses (MPS). Extensive congenital DM is actually associated with Hurler syndrome (MPS type I) and Hunter syndrome (MPS type II), although several reports also described this association with MPS type VI and other lysosomal storage disorders (LySD), including GM1 gangliosidosis, mucolipidosis, Sandhoff disease, and Niemann-Pick disease. Here, we present the case of a two-year-old boy presenting with extensive dermal melanocytosis, generalized hypertrichosis, and chronic itch, harboring a heterozygous variant of uncertain significance, NM_152419.3: c.493C>T (p.Pro165Ser), in the exon 4 of HGSNAT gene, whose mutations are classically associated with MPS IIIC, also known as Sanfilippo syndrome. This is the first report that highlights the association between extensive congenital DM and MPS type IIIC, as well as a pathogenetic link between heterozygous LySD carrier status and congenital DM. We speculate that some cases of extensive congenital DM could be related to heterozygous LySD carriers, as a manifestation of a mild clinical phenotype.

High-fluence 1064nm Q-switched Nd:YAG laser treatment for ectopic Mongolian spot.

Q-switched neodymium-yttrium aluminum-garnet (Q-switched Nd:YAG) laser has been reported as an effective treatment for nevus of Ota and acquired bilateral nevus of Ota-like macules (ABNOM). Data on ectopic Mongolian spots have rarely been reported.The present study was performed to investigate the treatment efficacy of a high-fluence 1064 nm Q-switched Nd:YAG laser without tissue whitening in ectopic Mongolian spots.We included 61 patients with ectopic Mongolian spots, and 70 lesions were examined. Thirty-three lesions were treated with a high-fluence 1064 nm Q-switched Nd:YAG laser, and 38 lesions were observed without treatment. The results were assessed using a 5-quantile grading scale and melanin index using a Mexameter®.Mean follow-up duration was 14.1 ± 6.8 months for the treatment group and 17.8 ± 10.0 months for the observation group. Mean 5-quintile grading scale at final follow-up was statistically different (p < 0.001) between the two groups (treatment: 2.85 ± 1.00, observation: 0.49 ± 0.73). There was a significant difference (p < 0.001) in the Δ melanin index (initial melanin index - final melanin index) between the observation (7.1 ± 62.7) and treatment (156.7 ± 78.4) groups.High-fluence Q-switched Nd:YAG laser without tissue whitening showed good results and was well-tolerated in treating ectopic Mongolian spots.

Melanoma sobre nevus azul en placa o sobre melanocitosis dérmicas: utilidad diagnóstica y pronóstica del BAP1 / Melanoma arising in plaque-type blue nevus and dermal melanocytosis: Diagnostic and prognostic value of BAP1

El melanoma sobre nevus azul o melanoma ex-blue nevus es una variedad de melanoma peculiar que tiene un perfil genético diferente al del resto de los melanomas cutáneos y sorprendentemente superponible al perfil del melanoma uveal. Aunque puede aparecer de novo, el melanoma ex-blue nevus se suele desarrollar sobre un nevus azul previo o sobre una melanocitosis dérmica. No todas las lesiones nodulares desarrolladas sobre un nevus azul o una melanocitosis dérmica son melanomas, y los hallazgos clínicos e histológicos pueden ser insuficientes para llegar a un diagnóstico de certeza. Así, cobran relevancia estudios adicionales, como la hibridación genómica comparada, pues la presencia de aberraciones cromosómicas favorece el diagnóstico de malignidad. Es de especial utilidad el estudio del gen BAP1, cuya pérdida de expresión orienta a melanoma en este espectro de lesiones. Presentamos 3casos del espectro nevus azul a melanoma ex-blue nevus con estudios de biología molecular (AU)

Melanoma arising in blue nevus, also known as melanoma ex blue nevus, is a specific form of melanoma whose genetic profile is different to that of other cutaneous melanomas and surprisingly similar to that of uveal melanoma. Although melanoma ex blue nevus can appear de novo, it usually arises in a preexisting blue nevus or dermal melanocytosis. Not all nodular lesions arising in association with blue nevus or dermal melanocytosis are melanomas, however, and because clinical and histologic findings may be insufficient for a definitive diagnosis, additional studies such as comparative genomic hybridization are important. Detection of chromosomal aberrations supports a diagnosis of malignancy. Studies of the BAP1 gene are particularly useful in this setting because loss of expression is indicative of melanoma. We present 3 cases on the spectrum of blue nevus to melanoma ex blue nevus that were studied using molecular biology techniques (AU)

[Translated article] Melanoma Arising in Plaque-Type Blue Nevus and Dermal Melanocytosis: Diagnostic and Prognostic Value of BAP1 / Melanoma sobre nevus azul en placa o sobre melanocitosis dérmicas: utilidad diagnóstica y pronóstica del BAP1

Melanoma arising in blue nevus, also known as melanoma ex blue nevus, is a specific form of melanoma whose genetic profile is different to that of other cutaneous melanomas and surprisingly similar to that of uveal melanoma. Although melanoma ex blue nevus can appear de novo, it usually arises in a preexisting blue nevus or dermal melanocytosis. Not all nodular lesions arising in association with blue nevus or dermal melanocytosis are melanomas, however, and because clinical and histologic findings may be insufficient for a definitive diagnosis, additional studies such as comparative genomic hybridization are important. Detection of chromosomal aberrations supports a diagnosis of malignancy. Studies of the BAP1 gene are particularly useful in this setting because loss of expression is indicative of melanoma. We present 3 cases on the spectrum of blue nevus to melanoma ex blue nevus that were studied using molecular biology techniques (AU)

El melanoma sobre nevus azul o melanoma ex-blue nevus es una variedad de melanoma peculiar que tiene un perfil genético diferente al del resto de los melanomas cutáneos y sorprendentemente superponible al perfil del melanoma uveal. Aunque puede aparecer de novo, el melanoma ex-blue nevus se suele desarrollar sobre un nevus azul previo o sobre una melanocitosis dérmica. No todas las lesiones nodulares desarrolladas sobre un nevus azul o una melanocitosis dérmica son melanomas, y los hallazgos clínicos e histológicos pueden ser insuficientes para llegar a un diagnóstico de certeza. Así, cobran relevancia estudios adicionales, como la hibridación genómica comparada, pues la presencia de aberraciones cromosómicas favorece el diagnóstico de malignidad. Es de especial utilidad el estudio del gen BAP1, cuya pérdida de expresión orienta a melanoma en este espectro de lesiones. Presentamos 3casos del espectro nevus azul a melanoma ex-blue nevus con estudios de biología molecular (AU)

Melanoma arising in plaque-type blue nevus and dermal melanocytosis: Diagnostic and prognostic value of BAP1. / Melanoma sobre nevus azul en placa o sobre melanocitosis dérmicas: utilidad diagnóstica y pronóstica del BAP1.

Melanoma arising in blue nevus, also known as melanoma ex blue nevus, is a specific form of melanoma whose genetic profile is different to that of other cutaneous melanomas and surprisingly similar to that of uveal melanoma. Although melanoma ex blue nevus can appear de novo, it usually arises in a preexisting blue nevus or dermal melanocytosis. Not all nodular lesions arising in association with blue nevus or dermal melanocytosis are melanomas, however, and because clinical and histologic findings may be insufficient for a definitive diagnosis, additional studies such as comparative genomic hybridization are important. Detection of chromosomal aberrations supports a diagnosis of malignancy. Studies of the BAP1 gene are particularly useful in this setting because loss of expression is indicative of melanoma. We present 3 cases on the spectrum of blue nevus to melanoma ex blue nevus that were studied using molecular biology techniques.

[Physiologic hyperpigmentation on darker skin]. / Physiologische Hyperpigmentierungen auf dunkler Haut.

Many, even common, normal variations and nuances of skin of color are less frequently described or often misinterpreted as pathological. Recognizing these physiologic changes is gaining relevance in our increasingly diverse patient population and enables the physician to encourage the acceptance by the patients as well as to manage our resources wisely. Therefore, we explain common hyperpigmentation on dark skin such as demarcation lines, mucosal hyperpigmentation, melanonychia striata, and circumscribed dermal melanocytosis. The aim of this article is to facilitate the classification of these phenomena in clinical practice.

Blue naevi and the blue tumour spectrum.

Blue naevi (BN) form a wide group of benign dermal melanocytic proliferations. They are genetically distinct from common and Spitz naevi with frequent hotspot mutations occurring in Gαq genes. Clinically, BN display a female predominance, elective sites of emergence and a great variety of subtypes related to specific regions of the skin linked to early embryological genetic events. Histologically, most BN are located in the dermis with small, bland, spindled and dendritic pigmented melanocytes within a fibrous background. Variation in tumour volume, fibrosis, and melanin pigment load can be broad. A growth in size and cellularity can occur within a subset of tumours as they acquire the morphological features of cellular blue naevi, with a biphasic architecture associating a dendritic blue naevus morphology near the surface, and deep vertical cellular expansions of medium-sized, bland melanocytes often reaching the subcutis. Sclerosing and myxoid variants can be observed either as individual or combined modifications that can add complexity to an otherwise straightforward diagnosis. Malignant progression of a cellular blue naevus is exceptional with an intermediate stage named atypical cellular blue naevus. Malignant blue melanomas are fast growing, large, pigmented tumours with most often obvious features of malignancy. However, they are difficult to separate from other malignant dermal melanocytic proliferations. Herein, we will extensively detail and illustrate the clinical, histological and genetic features of the vast spectrum of blue naevi and related entities in the skin.

Angiotropic melanocytes: Possibly a distinctive finding in extra-facial acquired dermal melanocytosis.

BACKGROUND: Acquired dermal melanocytosis is a heterogenous group of hyperpigmented lesioins which predominantly involve the face. AIM: The aim of this study was to study the clinical presentation and histopathology of cases with extra-facial acquired dermal melanocytosis. METHODS: Retrospective record analysis was performed between May 2016 to August 2019 to retrieve cases of extra-facial acquired dermal melanocytosis seen at the out-patient department of dermatology at the All India Institute of Medical Sciences, Jodhpur. Consecutive cases with histopathologically proven diagnosis of acquired dermal melanocytosis were included. Documentation of variation in clinical presentation and histopathologic findings was done in light of the existing literature. RESULTS: Overall, four cases of extra-facial acquired dermal melanocyosis (female:male = 1:3) were seen during the study period. The lone case on head and neck involved the ear lobule and peri-auricular area. The other three cases had involvement of the hand. The histopathology confirmed the diagnosis of dermal melanocytosis but revealed peculiar findings of angiotropic melanocytes and dilated capillaries. LIMITATIONS: Small sample size and lack of comparison with perilesional normal skin were the limitations of this study. CONCLUSION: The findings of angiotropic melanocytes may be unique to extra-facial acquired dermal melanocytosis. This might indicate interaction between dermal melanocytes and capillary endothelial cells. This finding along with dermal capillary ectasia may indicate a possible role for vascular lasers in the management of these disorders.

Disorders of hyperpigmentation. Part I. Pathogenesis and clinical features of common pigmentary disorders.

Disorders of hyperpigmentation are common and, depending on the extent and location of involvement, can affect the quality of life and pose a significant psychologic burden for patients. Given the similarities in presentation of the various causes of hyperpigmentation, it is often difficult to elucidate the etiology of these conditions, which is important to guide management. Furthermore, certain disorders, such as lichen planus pigmentosus and ashy dermatosis, have similar clinical and/or histologic presentations, and their classification as distinct entities has been debated upon, leading to additional confusion. In this review, the authors selected commonly encountered disorders of hyperpigmentation of the skin, subdivided into epidermal, dermal, or mixed epidermal-dermal disorders based on the location of pigment deposition, along with disorders of hyperpigmentation of the mucosa and nails. Melanocytic nevi, genetic disorders, and systemic causes of hyperpigmentation were largely excluded and considered to be outside the scope of this review. We discussed the pathogenesis of hyperpigmentation as well as the clinical and histologic features of these conditions, along with challenges encountered in their diagnosis and classification. The second article in this 2-part continuing medical education series focuses on the medical and procedural treatments of hyperpigmentation.

Treatment of Nevus of Ota in Black patients with the 1064 nm QS or picosecond laser and nonablative fractional photothermolysis.

OBJECTIVES: Nevus of Ota is a benign melanocytic lesion that presents as a unilateral blue gray to brown facial patch favoring the distribution of the first two branches of the trigeminal nerve. Incidence is highest in Asian and Black populations, however, the overwhelming majority of studies are limited to diagnosis and treatment in Asian patients. We herein present 10 Black patients with Fitzpatrick skin types (FST) V and VI who underwent laser treatment for Nevus of Ota. METHODS: We performed a retrospective review of Black patients presenting with Nevus of Ota. Race was self-designated by all patients and documented in the medical record at the time of initial consultation. Primary outcomes were based on improvement using before and after photographs which were graded by three independent board-certified dermatologists using a 5-point visual analog scale. RESULTS: Ten FST V or VI patients with an age range of 9 months to 45 years were treated for Nevus of Ota. All patients were treated with the 1064 nm Q-switched neodymium doped yttrium aluminum garnet (QS Nd:YAG) and on average received 4.7 treatments at 2-10 month intervals. Fluence ranged from 1.8 to 2.3 J/cm2 , and total pulse count ranged from 510.9 to 776.6. 2/10 patients were additionally treated with 1550 nm nonablative fractional resurfacing (NAFR), and 1/10 patients underwent combination therapy with both NAFR and 1064 nm picosecond laser therapy. Overall, patients saw a mean improvement of 51%-75% at follow-up 5-254 weeks (mean 51.5 weeks) after treatment. Three patients experienced mild guttate hypopigmentation in treated areas. No other long-term adverse events were encountered. CONCLUSION: 1064 nm QS Nd:YAG laser therapy is a safe and efficacious treatment for Nevus of Ota in patients with FST V and VI. When patient improvement plateaus, combining therapy with 1550 nm NAFR or transitioning to 1064 nm picosecond laser may be of benefit. Patients should be counseled on the risk of guttate hypopigmentation. This is the largest case series to date of Black patients with Nevus of Ota, highlighting the need for further investigation to determine optimal device settings and treatment parameters for this population.

Nevus of Ota on the auricle successfully treated with Q-switched ruby laser.

Nevus of Ota is a dermal melanocytosis that consists of blue-brown spots, patches and plaques along the distribution of the first and second branches of trigeminal nerve. The efficacy of Q-switched ruby laser treatment against nevus of Ota on dark skin has not been described. The present case, a 2-month-old Indonesian girl, showed rare auricular involvement. Because ear has complicated steric structure, whose skin is sensitive and thin, pain and inflammatory reaction are inevitable. We discussed the difficulty of laser treatments on auricular lesions.

A Report of Unusual Case of Acquired Dermal Melanocytosis on the Scalp of a Caucasian Man with a Literature Review.

Dermal melanocytosis includes several benign pigmented lesions which present as blue-gray color which is a result of the color transmission of melanin pigment through the dermis. While some types are present at birth, there is an acquired variant, acquired dermal melanocytosis (ADM), which usually involves faces of middle-aged Asian women. To the best of our knowledge, there are limited reports of extra facial ADM which all are on the trunk and extremities. Herein, we report a unique case of extra facial ADM affecting the scalp of a middle-aged man and provide a review of all extra facial ADM cases that have been reported.

Novel Application of the 730 and 785 nm Picosecond Titanium Sapphire Lasers for the Treatment of Nevus of Ota.

BACKGROUND AND OBJECTIVES: Nevus of Ota is a benign dermal melanocytosis that may pose significant psychosocial distress to patients. Q-switched nanosecond lasers have traditionally been considered the first-line treatment but pain, bleeding, and postinflammatory pigmentary alteration are common adverse effects. Picosecond devices have been increasingly used to treat nevus of Ota with promising results. We present two cases demonstrating novel applications of the 730 and 785 nm picosecond titanium sapphire lasers for the treatment of nevus of Ota in two patients with types III and IV skin. STUDY DESIGN/MATERIALS AND METHODS: A 730 and 785 nm picosecond titanium sapphire laser with pulse durations of 250 and 300 picoseconds, respectively, were used to treat two cases of nevus of Ota. Four to seven treatment sessions were conducted at monthly intervals, and follow-up evaluation was performed 1-3 months following the final treatment session. RESULTS: Both cases demonstrated greater than 75% clearance following treatment. There were no adverse events or pigmentary alteration noted as a result of picosecond titanium sapphire laser treatment. CONCLUSIONS: The 730 and 785 nm picosecond titanium sapphire lasers are safe and effective for the treatment of nevus of Ota. Lasers Surg. Med. 00:00-00, 2021. © 2021 Wiley Periodicals LLC.

Reimagining the Terms Mongolian Spot and Sign.

The term "Mongolian Spot" rather than the preferred descriptive name congenital dermal melanocytosis (CDM) continues to be used despite compelling objections to the contrary. Terms that stigmatize a culture, region, people, country, communities, and ethnic group should be replaced by their more descriptive counterparts. Herein, we clarify terminology, discuss the historical significance, and provide a recommendation about naming this disease.

[Treatment of nevus of Ota and Ito and epidermal nevus syndrome]. / Behandlung des Naevus Ota und Ito und des Pigmentnävussyndroms.

Nevus of Ota, nevus of Ito and nevus of Hori are special melanocytic nevi that have a slate-brown or blue/grey coloring. They are pigmented disorders characterized by its heterotopic melanocytic dermal location and by blue/brown unilateral and sometimes bilateral facial patch in case of nevus of Ota, and in the supraclavicular, scapular, and deltoid region in case of nevus of Ito. It is more common in patients with Asian and dark-skinned ethnic backgrounds. Histologically, elongated, dendritic melanocytes are seen scattered mainly throughout the upper third of reticular dermis. An acquired variant is called Hori's nevus with more bilateral facial distribution, similar to melasma. Dermal melanocytosis can also occur elsewhere on the body, including inside the mouth. Despite its benign nature, patients frequently seek therapy because of its facial involvement. QS lasers are used effectively to treat these lesions. The number of treatment sessions correlates with clinical improvement. Post laser hypo- and hyperpigmentation are common side effects mainly affecting patients with darker skin.

Superficial dermal melanocytosis of the elderly: An exceptional occurrence?

The development of flat pigmented lesions on chronically sun-damaged (CSD) skin of the face may represent the clinical manifestation of a wide variety of hyperplastic/neoplastic melanocytic proliferations. We report the exceptional case of an acquired pigmented patch occurring on CSD skin, histopathologically characterized by diffuse hyperplasia of dendritic/spindled melanocytes in the superficial dermis within a widened band of actinic elastosis. This lesion was associated with a small focus of early invasive lentigo maligna melanoma (LMM). We show the melanocytic nature of the population of dermal pigmented cells by means of single and double immunohistochemical staining for melanocytic and histiocytic markers. The biologic significance of the focus of LMM within the hyperpigmented lesion (whether random collision phenomenon or causally related occurrence), as well as the pathogenesis of the whole dermal lesion are difficult to elucidate. Our case emphasizes the need for a better understanding of the pathophysiology of so-called dermal melanocytes.