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Cutis verticis gyrata (CVG), characterized by cerebriform overgrowth of the scalp, is rarely observed in congenital melanocytic nevi (CMN). We describe a 13-year-old male with autism and a large CMN of the scalp with numerous satellite nevi whose scalp nevus exhibited evolution with poliosis and CVG. Given the potential association of CVG (independent of CMN) with seizures, neuropsychiatric, and ophthalmologic disorders, and nevus-associated CVG (cerebriform intradermal nevus) with melanoma, multidisciplinary evaluation of CMN patients with CVG is important to guide management and treatment., (© 2022 Wiley Periodicals LLC.)

Congenital melanocytic nevi (CMN) are nevi that are present at birth or arise within the first few weeks of life. They are often found on the trunk, head and neck and extremities. We report herein an unusual presentation of a CMN as a cerebriform tumour presenting as secondary cutis verticis gyrata on the scalp of an 18-year-old Filipino male., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Cutis verticis gyrata (CVG) describes a rare thickening and folding of the scalp resembling the outer surface of the brain. Secondary CVG is caused by a variety of diseases such as nevi. A 38-year-old woman presented with a long history of a deformed right parietofrontal scalp. Clinical and histopathologic criteria lead to the diagnosis of CVG secondary to a combined blue and congenital nevus. Reports of malignant melanoma developing in this clinical setting mean that each patient must be followed closely or considered for prophylactic excision.

Large or giant congenital melanocytic nevi are rare. They are associated with a risk of melanoma, which is poorly quantified, and often represent a major cosmetic problem. Surgical intervention is the most common treatment, although dermabrasion or curettage in the first weeks of life is used in some centers. When these nevi occur in the scalp, the risk of melanoma has been reported to be lower. Here we describe a series of children in whom large congenital melanocytic nevi on the scalp appeared to spontaneously involute. Therefore, our findings indicate that these nevi also have a better prognosis cosmetically. It is suggested that the decision about surgical treatment should be deferred until the maximal improvement in appearance has occurred during the first 2 years of life.

Basal cell carcinoma (BCC) occurs rarely in children and is most often associated with an underlying condition that predisposes patients to the development of malignancy. There have been numerous reports of BCC developing after puberty in nevus sebaceus; however, such occurrences have rarely been described in children. We report a 7-year-old boy with BCC forming in a nevus sebaceus.

Detection of fetal scalp cysts may pose diagnostic challenges in terms of differentiating between structures whose origin is ectodermal, so presumed benign, and neuroectodermal, potentially having neurological significance. We describe the first case of a fetal scalp dermoid cyst to be detected antenatally and discuss the concept of sequested meningoceles which may not have neurological consequence but can be associated with other structural abnormalities., (Copyright 1999 John Wiley & Sons, Ltd.)

A 74-year-old man presented with a tumor on his scalp that he had since birth. The authors observed a verrucous pink crusted plaque. The tumor was clinically diagnosed as nevus sebaceus and was removed. Histopathologic study showed, arising from nevus sebaceus, a lobular neoplasm with the classic features of trichilemmoma. Desmoplasia was present in the center of the trichilemmomatous area. This description is typical of a special, uncommon type of trichilemmoma that histologically mimics invasive carcinoma.

A newborn girl had some subcutaneous masses within a giant congenital melanocytic nevus in the left parietal area. Microscopic examination revealed melanoma. The tumor consisted of two different patterns, being composed partly of epithelioid cells and partly of spindle-shaped cells. Immunohistochemically the epithelioid cells showed a positive reaction for HMB-45 and NKI/C3, and a negative reaction for desmin and alpha-sarcomeric actin. The spindle-shaped cells showed a negative reaction for HMB-45 and NKI/C3, and a positive reaction for desmin and alpha-sarcomeric actin. Ultrastructurally the spindle-shaped cells had some schwannian features, namely, a basal lamina, interdigitating cell processes, and junctional complexes. The pathologic findings of our case suggest possible direct rhabdomyogenic differentiation of melanoma cells.

A male infant with the features of Adams-Oliver syndrome is described. These features included severe aplasia cutis congenita (ACC) of the scalp involving both skin and cranium, a shortened right foot with talipes equinovarus, extensive cutis marmorata telangiectatica congenita and also haemangiomas and ulceration of the abdominal skin. Exposure of the dural membrane was associated with infection, hyponatraemia and finally localised necrosis with herniation of brain and fatal cerebral haemorrhage.