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.

Tumescent local anaesthesia for the surgical therapy of congenital nevi in the first year of life.

BACKGROUND: The indication for surgical treatment of congenital nevi must be made after individual consideration of the expected benefit and risk and requires careful information of the parents as well as interdisciplinary psychological support. In addition to suspected malignancy, a relevant indication is the risk of stigmatization depending on the size and localization of the nevus. Objective was to show which size reduction of the congenital nevus can be achieved by surgical therapy under tumescent local anaesthesia (TLA) in infancy and how often complications of anaesthesia or surgery occur. METHODS: All infants up to 12 months of age who underwent surgery for a congenital nevus under TLA at the University Dermatological Clinic Tuebingen between January 2015 and December 2021 were included; surgeries were performed using serial excisions (powerstretching technique), whereby the incisions were made inside the nevus and mobilization was strictly limited to the skin side without nevus. RESULTS: Overall, a cumulative total area of 38.65 cm2 could be removed on average within the first year of life (trunk: 67 cm2 , head: 21.2 cm2 , legs: 21.6 cm2 , arms: 13.2 cm2 ). A cumulative maximum area reduction of 406.9 cm2 could be achieved. We evaluated 363 surgical sites (123 children) on all body regions. The median age of the children at the first surgery was 3.5 months (0.46-10.7 months). Complications occurred in 2.3% (seven procedures). All these complications were reversible in the course of the operation and did not lead to a prolonged hospital stay. No anaesthesia-related complications occurred. CONCLUSION: We were able to show that a reduction of large areas of congenital nevi is possible in the first year of life with the combination of serial excisions using powerstretching technique, TLA, and intracutaneous butterfly sutures.

Care of Congenital Melanocytic Nevi in Newborns and Infants: Review and Management Recommendations.

A pediatric dermatology expert working group performed a narrative review to describe care related to congenital melanocytic nevi (CMN) in neonates and infants. There are no published guidelines for most aspects of care, including routine skin care and visit intervals. Few guidelines exist for surgical management; newer recommendations favor conservative practice. Emerging evidence contributes to recommendations for screening MRI to evaluate for neural melanosis and related central nervous system complications, however, more research is needed. Risk for melanoma is generally low, but those with large, giant, or multiple CMN have a higher risk. Multidisciplinary care, with a focus on family and patient preferences, is of paramount importance. Without standardized screening and management guidelines, questions abound regarding appropriate physical examination intervals, potential treatment including full or partial excision, timing and frequency of imaging, melanoma risk, and assessment for neural melanosis. This review highlights the current state of knowledge concerning care of patients with CMN, reveals gaps in the literature surrounding skin care, and provides management recommendations. We additionally discuss cutaneous complications of CMN, such as pruritus, hypertrichosis, and wound healing. Resources and references for families and providers can help patients navigate this sometimes challenging diagnosis. Finally, we contribute expert care recommendations to the current body of literature as a foundation for the development of future, more comprehensive care guidelines.

Cutaneous lasers and skin camouflage make-up: a useful alternative intervention for periorbital hairy congenital melanocytic naevus.

Periorbital congenital melanocytic naevi can be very disfiguring and difficult to treat effectively. Although surgical excision and reconstruction is the most widely accepted treatment strategy, we describe a case in which cutaneous lasers treatment followed by the application of cosmetic skin camouflage make-up provided an alternative solution delivering a good cosmetic improvement.

Infantile hemangioma: treatment with short course systemic corticosteroid therapy as an alternative for propranolol.

Infantile hemangiomas (IHs) are increasingly being treated with propranolol or other beta-blockers, but before this therapeutic option was available, oral glucocorticosteroids (GCSs) were the criterion standard treatment and are still the alternative modality in problematic cases. Nevertheless, there is no standard treatment protocol for the dose and duration of GCSs. Long-term treatment with GCSs is associated with unwanted side effects such as growth suppression, behavioral changes, and reflux. Twenty-one children with troublesome IHs were treated according to an algorithm with 3 mg/kg/day of oral prednisolone divided three times per day with varying duration and number of GCS courses. Two blinded investigators independently interpreted therapy results using the Hemangioma Activity Score (HAS). Side effects were determined according to reports in patient charts and parental questionnaires. The median duration of a short course of GCSs was 2 weeks (range 1-6 weeks). The number of courses was 2 (range 1-5). The median cumulative dose was 91 mg/kg. Growth stabilized in all patients, with a good response (>50% reduction in HAS) in 62% and a favorable response (30-50% reduction is HAS) in 23%. Twelve of the 21 children (57%) had minor side effects. Persistent side effects did not occur. Intermittent short course, systemic, high-dose GCS therapy is an effective and safe treatment modality for IH, with a substantially lower cumulative dose of GCSs compared to prolonged therapy and no major side effects. This treatment is an alternative in cases in which propranolol fails or is contraindicated.

New therapies in pediatric dermatology.

PURPOSE OF REVIEW: There have been many new developments in therapeutic modalities for the treatment of pediatric dermatological diseases in the past year. Advances in the treatment of atopic dermatitis, psoriasis, infantile hemangiomas and dystrophic epidermolysis bullosa will be discussed. The following review will update the reader on these exciting new possibilities for patient care and future directions for research to improve the lives of children suffering from skin diseases. RECENT FINDINGS: This review will discuss recent articles describing the use of topical tacrolimus for maintenance of remission in atopic dermatitis, utility of nurse educators in atopic dermatitis, safety and efficacy of etanercept for the treatment of psoriasis in children, narrow band ultraviolet B phototherapy for atopic dermatitis and psoriasis, use of topical timolol for infantile hemangiomas and bone marrow transplantation for dystrophic epidermolysis bullosa. SUMMARY: There are many new interesting, potentially useful therapeutic modalities emerging in pediatric dermatology. New treatments for atopic dermatitis, psoriasis, infantile hemangiomas and dystrophic epidermolysis bullosa are reviewed.

Early excision of congenital melanocytic nevi under tumescent anesthesia and skin expansion by intracutaneous double butterfly sutures.

BACKGROUND: Excision of large congenital melanocytic nevi is recommended because of the risk of malignant transformation and for aesthetic reasons. Our treatment concept includes 1) excision in one or more steps preferably before the age of one year, 2) skin expansion through high-tension wound closure with intracutaneous double butterfly sutures and 3) performing surgery with tumescent or general anesthesia. PATIENTS AND METHODS: A retrospective survey was conducted among all parents with children treated for congenital melanocytic nevi in 2004 or 2005 with a maximum age of 6.0 years. The parents were asked to evaluate the trauma associated with their child's surgery and the aesthetic result. RESULTS: Fifty-nine families were contacted, of whom 51 replied. Forty-two parents assessed surgery-associated trauma as light or very light. Thirty-nine parents rated the aesthetic results as good to very good. Severe complications did not occur. CONCLUSION: The proposed surgical concept is a safe procedure with good aesthetic results and low surgery-associated trauma for the child.

Congenital high-grade sarcoma presenting as skin nodules and respiratory distress in a neonate.

We report, to our knowledge, the first case of a congenital, widespread, aggressive high-grade sarcoma, presented as multiple skin nodules and respiratory distress in a neonate that had a t(9;22)(q22;q11-12) cytogenetic abnormality suggestive of a more indolent extraskeletal myxoid chondrosarcoma (EMC). EMC is generally thought of as a slow-growing tumor that presents between the fourth and sixth decades of life. Our patient was a 45,XY, t(13;14) newborn who presented at birth with subcutaneous nodules involving the face, scalp, back and extremities, as well as multiple intrathoracic, intraabdominal and intracranial masses. Diagnosis was made using electron microscopy and immunohistochemical and cytogenetic studies. Despite attempts to control rapid growth of lesions using high-dose steroids and cis-retinoic acid, patient's clinical status continued to deteriorate and life support was withdrawn at the 26 day of life.

[Therapy of congenital melanocytic nevi. Excision, dermabrasion, laser]. / Therapie kongenitaler melanozytärer Nävi. Exzision, Dermabrasion, Laser.

Congenital melanocytic nevi are rare lesions which depending on their size and location may cause major cosmetic and psychological problems. Large congenital melanocytic nevi may undergo malignant change and can also be associated with neurocutaneous melanosis. The different treatment approaches reach different levels of the skin. Complete excision is the treatment of choice, but is not always possible with giant nevi. Superficial treatment can reduce the pigmentation, but repigmentation is not uncommon. Incomplete removal of melanocytic nevi does not reduce the melanoma risk. Surgical intervention must be carefully planned; the advantages and disadvantages of the different modalities must be discussed with the parents. The risk of malignant transformation must be weighed up against the expected aesthetic and functional outcomes.

Mongolian spots with involvement of mandibular area.

A 1-year-old boy had grayish pigmentation on the left side of his face over the area supplied by the mandibular branch of the trigeminal nerve. Upon further examination, the lesion was also found on the left side of the neck, shoulder, upper arm, right lower back and buttock. The pigmentation was uniform in intensity. This case report demonstrates that Mongolian spots can occur on the face in the area supplied by the mandibular branch of the trigeminal nerve. These spots should not be misdiagnosed as nevus of Ota.

Treating vascular lesions.

The treatment of acquired vascular lesions is one of the most commonly requested and performed cutaneous laser procedures. Furthermore, every year, 40,000 children are born in the United States each with congenital vascular lesions and malformations. Laser treatment of vascular lesion is based on the principle of selective photothermolysis, conceived in the 1980s. A variety of different lasers and light sources have since been used in the treatment of vascular lesions: lasers with wavelengths between green and yellow, near infrared lasers, and broadband light sources. Despite limitations, this remains the treatment of choice today. This publication addresses acquired and congenital vascular lesions as different entities and proposes a separation of vascular lesions into those that can easily be treated from those where clearance is difficult. Different treatment modalities and the various endpoints of individual vascular lesions will be discussed.

Ruby laser treatment of congenital melanocytic nevi: a review of the literature and report of our own experience.

The authors describe the possibilities and advantages of ruby laser treatment of large congenital nevi as an alternative to surgical excision. The literature (from 1980 to 2002) is reviewed and a case report is presented. Literature and the authors' experience show good cosmetic results after ruby laser treatment. Skin texture is improved and there is a considerable reduction in pigmentation and unsightly hair growth. Ruby laser treatment does not result in scarring, mutilation, or functional impairments, in contrast to surgical treatment. Only short outpatient sessions are required, recovery periods are extremely short, and no rehabilitation period is needed. No malignant changes have been reported after treatment with the ruby laser, even after 8 years of follow-up. The results imply that ruby laser treatment could be a valuable new treatment modality for large congenital nevi. It should be considered when patients refuse to undergo surgery, or when surgery would cause severe morbidity. More research should be initiated to assess possible risks and long-term results.

Recurrent pigmented macules after q-switched alexandrite laser treatment of congenital melanocytic nevus.

BACKGROUND: Q-switch-mode laser treatment of congenital nevi does not result in complete histological clearance, and many patients have partial repigmentation within several months. In addition, the number of recurrent pigmented macules (RPMs) may increase, a major drawback to good cosmetic results. While the mechanism of recurrence is not known. OBJECTIVE: To help elucidate the mechanism of RPM development, we evaluated the expression of TNF-alpha and E-cadherin on RPM after treatment of congenital nevi with a Q-switched alexandrite laser (QSAL). METHODS: Thirteen Korean subjects with congenital nevi received QSAL treatment at intervals ranging from 2 to 6 months (mean, 4.5 treatments). Two-millimeter punch biopsy specimens were obtained at their first visit and from RPMs 3-6 months after the last treatment. Expression of E-cadherin and TNF-alpha were determined histochemically in the original nevi and RPM. In addition, one RPM was examined by electron microscopy. RESULTS: Reduced pigmentation in the treated areas was seen in all cases, but partial repigmentaion was seen as black spots within 6 months after the last QSAL treatment. Compared to the original nevi, the RPMs had increased numbers of melanocytes in the epidermis and reduced nevomelanocytic nests in the dermis. The expression of TNF-alpha and E-cadherin was downregulated in the RPMs compared to the original nevi. Electron microscopy confirmed the increase in melanocytes in the epidermis of RPMs. CONCLUSION: Our findings suggest that the down-regulation of E-cadherin and TNF-alpha may induce the proliferation of melanocytes, resulting in the formation of RPMs.

A case of giant congenital nevocytic nevus with neurotization and onset of vitiligo.

The authors report the case of a 21-year-old woman with a giant congenital nevocytic nevus (GCNN) who developed vitiligo at the age of 16 years on skin areas remote from the GCNN. This is the first reported case of GCNN developing neurotization combined with vitiliginous changes within the GCNN lesion. Treatment with PUVA achieved repigmentation of the vitiligo lesions, except for the hypochromic areas within the area of the nevus that were shielded from UVA radiation.