Congenital Blue Nevus Research Articles

Nail Unit Blue Nevi: A 11-Case Series and Review of the Literature

Background: Blue nevus of the nail apparatus is a rare entity and only isolated cases are reported in the literature. Objective: The aim of this study was to better characterize blue nevus at the nail unit. Methods: Retrospective analysis of all nail unit blue nevi from the Nail Group of the French Society of Dermatology was compared to the literature. Results: Eleven cases were retrieved from 2002 to 2019 with an average age of 45 years. The majority were women (9/11) and acquired (10/11). Hands were more frequently involved (9/11) with a predilection for the thumb and 2 cases were located on the hallux. Nail unit blue nevus mostly presented as a well-delimited blue spot of the lunula (9/11) and histologically was of the common type (10/11). There was no malignancy. Conclusion: Nail unit blue nevus is a rare asymptomatic benign entity, mostly acquired on the thumb or the hallux of women. The most frequent presentation is a painless blue spot on the lunula. Congenital blue nevi seem to only affect the paronychium. Main differential diagnosis is melanoma and histopathological examination is mandatory.

Dermal Melanocytosis of the Scalp Associated to Intracranial Melanoma

Intracranial invasion of cellular blue nevus is extremely rare, and its malignant transformation is even less common. The differential diagnosis includes neurocutaneous melanosis and neurocristic cutaneous hamartoma. A 50-year-old female presented with intracranial melanoma in contiguity with a congenital blue nevus on the scalp. The patient showed a wide pigmented lesion on the scalp that had grown in the last few years over the congenital blue nevus. Magnetic resonance imaging revealed an intracranial tumor lying contiguous to the nevus. Despite aggressive surgery, the tumor relapsed and the patient developed systemic metastases. We report a rare case of cellular blue nevus showing an unexpected aggressive behavior with extensive extra- and intracranial expansion and distant metastases.

Malignant Blue Nevus Arising in a Giant Congenital Cellular Blue Nevus in an Infant

Giant congenital blue nevus (GCBN) is rare and usually occurs on the scalp. Malignant blue nevus (MBN) is also rare and has a poor prognosis. We report a case of MBN arising in a GCBN on the back. There have been three previous reports of MBN associated with GCBN on the trunk; our case had the earliest onset of MBN arising in a GCBN.

Giant congenital cellular blue nevus of the scalp: neonatal presentation and approach

Giant congenital cellular blue nevus of the scalp neonatal presentation and approach Martine Debyser1, Sofie De Schepper2, James D'haese1, Veerle De Ketelaere3, Luc Cornette11Neonatal Intensive Care Unit AZ St Jan Bruges-Ostend AV, Bruges, Belgium; 2Department of Dermatology, Ghent University Hospital, Ghent, Belgium; 3Department of Paediatrics, St Rembert Hospital, Torhout, BelgiumAbstract: The authors report the case of a massive vaginal bleeding during labor and delivery, resulting in the birth of a newborn infant in hypovolemic shock. The origin of the bleeding was a giant congenital cellular blue nevus (CBN) of the scalp. The report illustrates the crucial role of immediate and aggressive volume resuscitation, suturing of the skin dehiscence, and firm external compression. This therapy was combined with whole-body hypothermia. The neurological outcome of the patient was normal. A shiny blue mass on the scalp should alert neonatologists to the possibility of a blue nevus of the scalp, rather than a simple scalp hematoma. Final diagnosis is made by biopsy and histologic examination. Early surgical intervention is necessary to prevent malignant degeneration and metastasis. A multidisciplinary approach involving a pediatrician/neonatologist, dermatologist, and surgeon is thus needed for diagnosis, treatment, and long-term follow-up.Keywords: neonate, cellular blue nevus, giant blue nevus, giant congenital blue nevus, hemorrhagic shock, whole-body hypothermia

Congenital melanocytic nevi—when to worry and how to treat: Facts and controversies

Congenital melanocytic nevi (CMN) are evident in 1% to 6% of neonates. In some studies, nevi with clinical, dermatoscopic, and histologic features identical to CMN have had a prevalence of more than 15% in older children and adults, possibly reflecting the “tardive” appearance of nevi programmed from birth. There is ongoing debate about the magnitude of the risk of melanoma and other complications associated with CMN of various sizes and the best approach to management of these lesions. We review the natural history of CMN, including proliferative nodules and erosions during infancy, neurotization, and spontaneous regression, and features of variants such as speckled lentiginous and congenital blue nevi. The risk of melanoma arising within small-sized (<1.5 cm) and medium-sized CMN is low (likely <1% over a lifetime) and virtually nonexistent before puberty. Recent data suggest that melanoma (cutaneous or extracutaneous) develops in approximately 5% of patients with a large (>20 cm) CMN, with about half of this risk in the first few years of life. Melanoma and neurocutaneous melanocytosis (NCM) are most likely in patients with CMN that have a final size of >40 cm in diameter, numerous satellite nevi, and a truncal location. One-third of individuals with NCM have multiple medium-sized (but no large) CMN. In patients at risk for NCM, a screening gadolinium-enhanced magnetic resonance imaging, preferably before age 6 months, and longitudinal neurologic assessment are recommended. Management of CMN depends on such factors as the ease of monitoring (more difficult for large, dark, thick nevi) and cosmetic and psychologic benefits of excision or other procedures. CMN require lifelong follow-up. Periodic total body skin examinations are necessary for all patients with large CMN, even when complete resection (often impossible) has been attempted.

Intratumoral Therapy With Interferon-α in a Locoregional Advanced Malignant Blue Nevus

Malignant blue nevi are a rare variant of cutaneous melanoma often associated with metastatic disease and poor prognosis. Information on the treatment of this condition is limited. This case study reports the use of intralesional interferon -alpha2b (IFN-alpha2b) as a salvage option in a male patient with a congenital blue nevus on the right buttock. After initial diagnosis, the primary lesion (lesion 1) was partially resected and the patient received aggressive treatment with radiotherapy and concomitant polychemotherapy (cisplatin, vinblastine, and dacarbazine). However, the lesion progressed and the patient was treated second-line with carboplatin, paclitaxel, and sorafenib. Although this treatment stabilized the primary lesion, at cycle 6, the patient developed a measurable locoregional metastasis (lesion 2). At this stage, the patient was treated with intralesional IFN-alpha2b twice weekly at lesion 2, followed by weekly intralesional IFN-alpha2b therapy to both lesions, which resulted in partial disease regression. Biweekly maintenance therapy with IFN-alpha2b administered to both lesions maintained a partial response (ongoing at 23+ mo) and allowed complete occupational rehabilitation. Thus, intralesional therapy with IFN-alpha2b may be a viable treatment option in patients with locally advanced melanoma who have progressed on prior radiotherapy, polychemotherapy, and/or multikinase antiangiogenic-based therapy.

Congenital smooth muscle hamartoma on the scalp

Case Report An 8-day-old male infant was referred to our neonatal unitfor prematurity. It was observed that his scalp color wasdarker than the rest of his skin color when the scalp hair waspartially shaved for intravenous access. Dermatologic exam-ination revealed a slightly elevated, hyperpigmented plaqueconfined to the whole scalp. The margin of the lesion did notextend to the face and neck. His hair was abundant and dark(Fig. 1). There were no areas of alopecia and no overlyingfolds of skin. There were no dysmorphic features, such asMichelin tire baby. Pseudo-Darier’s sign was negative.A punch biopsy specimen was obtained from the lesionwith a diagnosis of congenital melanocytic nevus or congeni-tal blue nevus. The result of histopathologic examinationshowed increased smooth muscle bundles in the reticular der-mis running in various directions under a normal epidermis(Fig. 2a); immunohistochemical stain revealed desmin-positive (Fig. 2b) and S100-negative proliferation. Based onthese clinical and histopathologic findings, a diagnosis ofcongenital smooth muscle hamartoma was made. No changesin this lesion were observed during a 9-month follow-up period. Discussion Congenital smooth muscle hamartoma (CSMH) is a rarecutaneous dysembryoplasia characterized by a disorganizedproliferation of normal muscle fibers of arrector pili. Thedisease usually presents as a localized, skin-colored ormildly hyperpigmented, irregularly shaped patch or plaquewith prominent vellus hairs located on the lumbosacral area.

Malignant Melanoma Arising in a Pregnant African American Woman with a Congenital Blue Nevus

The incidence of cutaneous melanoma in African-Americans is relatively low. Despite the slightly greater occurrence of congenital melanocytic nevi in black persons compared with white persons, the cumulative risk of melanoma arising in these lesions is very small. In addition, the overwhelming majority of melanomas in black persons occur on nonglaborous skin where congenital melanocytic nevi are rare. The objective was to describe and an unusual case of melanoma arising in a congenital nevus with combined features of a blue nevus on the scalp of a pregnant African-American woman. Histologic examination revealed a polypoid malignant melanoma arising in association with a congenital blue nevus in a young African-American woman. The lesion was located on the right parietal scalp and had been enlarging over the course of her pregnancy. Pathology from parotidectomy and neck dissection confirmed metastatic melanoma involving two intraparotid lymph nodes and 3 of 26 cervical lymph nodes. Despite aggressive chemotherapy, she died in 1 year after the diagnosis. A case is reported and the literature is reviewed. Clinicians must take great care in documenting and following pigmented lesions in all patients including African-American persons. Excision is indicated for lesions that undergo significant change during pregnancy.

Malignant Melanoma Arising in a Pregnant African American Woman with a Congenital Blue Nevus

Malignant Melanoma Arising in a Pregnant African American Woman with a Congenital Blue Nevus

Comparative study of dermabrasion, phenol peel, and acetic acid peel

Six areas of the face and forehead of a 36-year-old white female presenting with a benign congenital blue nevus of the skin were treated by dermabrasion, bichloracetic acid, and the classic phenol peel. Comparative results at six months showed each of these methods to be approximately equal in the depth of penetration and in the quality of skin on healing. However, in these small test sites, dermabrasion appeared to remove pigment slightly more efficiently. Therefore, her forehead and cheeks were treated with dermabrasion and subsequently with chemical peel. An attempt was made to touch up the dermabraded areas with acetic acid. Full thickness burns occurred, which resulted in thick scarring that required many months to finally heal. We conclude that at least in the treatment of pigmented lesions, the modalities of phenol, acetic acid, and dermabrasion are approximately equal.

Neoplasms arising in congenital giant nevi

Congenital giant nevi are complex cutaneous malformations composed of melanocytic and occasionally neural supportive elements. Malignant neoplasms arising in this setting are not uncommon, and their histologic appearances often differ significantly from the typical pattern of malignant melanoma. We report six patients with neoplasms arising in congenital giant nevi and one patient with a neoplasm arising in an extensive congenital blue nevus, and present a description of the neoplastic patterns encountered. These patterns include 1) poorly differentiated small round cell cancer, 2) malignant cellular blue nevus, 3) spindle-cell malignant tumor with lamellar cell (pseudomeissnerian) differentiation, 4) so-called minimal deviation melanoma, 5) heterologous malignant mesenchymal differentiation including rhabdomyosarcoma and liposarcoma, and 6) undifferentiated spindle cell cancer. We have reviewed the literature in order to address the question of frequency of malignant transformation in congenital giant nevi, the reported experience with the morphology of these cancers, and the histogenesis of these sometimes complex neoplasms as it is illuminated by our current understanding of the embryology of the neural crest.