Giant Cerebriform Intradermal Nevus with Follicular Pigmented Macules: A Rare Presentation
Year: 2026
Type: article
Study design: Case Report
Abstract: Sir, A 3-year-old girl presented with an asymptomatic swelling over her scalp since birth. The lesion started as a small macule with loss of hair on the left side of the scalp, which enlarged gradually. She was born of a nonconsanguineous marriage. The family history was negative for melanoma or any congenital melanocytic nevi. Examination showed multiple nodules over the left parieto-occipital region of scalp coalescing to form a single hyperpigmented plaque of size, 22 cm × 12 cm × 2.5 cm, which resembles gyri and sulci with alopecia [Figure 1a]. Multiple follicular blackish macules were also present over hyperpigmented swelling [Figure 1b]. Dermoscopic examination showed brown color macules in a globular pattern over tan background, resembling a “Leopard appearance,” with each macule showing central hypopigmentation and peripheral network suggestive of a benign melanocytic nevus [Figure 2]. A 3 mm punch biopsy was done from plaque. Histopathology showed diffuse proliferation of nested melanocytes in reticular dermis and subcutaneous tissue with patchy perivascular and periadnexal patterns. Melanocytes were oval to spindle-shaped without cell atypia [Figure 3]. On the basis of clinical, dermoscopic, and histopathological examination, she was diagnosed as a case of cerebriform intradermal nevus (CIN) with spotted grouped pigmented naevi type-1. The patient was asked to visit OPD for regular close follow-up, and no malignant change was observed on clinical and dermoscopic examination after 1 year.Figure 1: (a) Hyperpigmented plaque over scalp with corrugated pattern, (b) Follicular blackish macules over plaqueFigure 2: Brown macules in a globular pattern with central hypopigmentation were seen on dermoscopy (Leopard appearance)Figure 3: Nest of melanocytes in dermis with patchy periadenexal pattern (H and E, ×100)Discussion Melanocytic nevi are a diverse group of benign melanocytic proliferations which vary in clinical appearance, progression, and associated risk of melanoma.[1] CIN is a rare type of congenital melanocytic nevus with a corrugated appearance.[2] Hammond and Ranson were the first to describe a cerebriform nevus resembling cutis verticis gyrata (CVG) in 1937.[3] CIN is a rare cause of secondary CVG, characterized by hyperpigmented to skin colored tumor with folds and furrows over the surface, usually localized over the scalp.[3] The mechanism of cerebriform appearance on the surface is unknown. There may be multifocal hyperplasia of epidermis and nevus cells.[2] Histological examination shows intradermal nevus cells in the full dermis which can be well delineated or irregular.[3] Globular pattern by hypopigmented macules with cerebriform-like image in a few lesions on dermoscopy was mentioned in one case report.[4] Malignant transformation can be seen in patients of CIN, 3 out of 77 cases (3.9%).[5] Surgical excision is preferable; regular close monitoring can be considered in asymptomatic patients. Spotted grouped pigmented naevi is a rare form of congenital pigmented naevi consisting of spotted lesions, first described by Scholtz in 1932. It was further subclassified into three subtypes: type 1 (follicular papules forming a plaque), type 2 (agminated naevi), and type 3 (naevus spilus). Histological examination shows periappendageal localization of naevus cells, with follicular centric in type 1 and eccrine predominance in type 2 and 3. The present patient had hyperpigmented plaque with fold and furrow, which was suggestive of CIN, along with follicular pigmented macules. These clinical findings were supported by histopathological examination, diffuse proliferation of melanocytes in the reticular dermis with a significant number of melanocytes around adnexal tissue. We have observed follicular pigmented macules over the hyperpigmented plaque, which were not described in the literature to the best of our knowledge. Dermoscopic examination showed brown macules in a globular pattern over a tan background. Although malignancy has been reported in cases of CIN, regular close monitoring with clinical and dermoscopic examination should be considered for asymptomatic benign lesions. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Authors’ contributions Literature search: A.M, Manuscript preparation: A.G, Manuscript editing: B.B,,; Manuscript review: S.K.S. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Source: Clinical Dermatology Review
Institutions: Institute of Medical Sciences, Banaras Hindu University
Language: English
Subfield: Oncology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W7119497025