Cerebriform Intradermal Nevus Research Articles

Cerebriform Intradermal Nevus Presenting as Cutis Verticis Gyrata: A Rare Presentation

Abstract Cerebriform intradermal nevus (CIN) is one of the causes for secondary cutis verticis gyrata (CVG). Here, we report a 9-year-old boy with well-demarcated swelling over the left temporal area of his scalp since birth. The swelling was about 15 cm × 10 cm in size with cerebriform folds. It was soft in consistency and loss of hair was present. Histopathology confirmed CIN. This report serves as an example of a rare case of secondary CVG due to a CIN.

Cerebriform intradermal nevus manifesting as cutis verticis gyrata

Cutis verticis gyrata (CVG) is a rare scalp condition characterized by scalp skin redundancy that manifests as thickening of scalp skin with furrows and convoluted folds, giving out a cerebriform pattern of external appearance. CVG might manifest either as primary or secondary to an underlying cause. This case demonstrates the typical imaging findings of CVG that was provisionally diagnosed on imaging and confirmed on histopathology as secondary to cerebriform intradermal nevus in a healthy adolescent girl presenting with history of focal irregular scalp thickening since birth.

Dynamic evolution of a scalp congenital melanocytic nevus with poliosis and cutis verticis gyrata.

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.

A rare cutis verticis gyrata secondary to cerebriform intradermal nevus: case report and literature review

BackgroundCutis verticis gyrate (CVG) is a rare morphologic syndrome that presents with hypertrophy and folding of the scalp. CVG can be classified into three forms: primary essential, primary non-essential, and secondary. Cerebriform intradermal nevus (CIN) is a rare cause of secondary CVG. We are here to report a rare case of CVG with an underlying CIN and discuss the clinical course, treatment options, and critical screening guidelines for these patients.Case presentationA 25 year-old male patient presented with a chief complaint of generalized hair loss in the scalp parietaloccipital region for a duration of 1 year and the hair loss area was occasionally accompanied by mild itching. The hair loss started gradually and worsened over time. In addition, he had scalp skin folds resembling the ridge and furrow of the cerebral cortex in the parietaloccipital region since birth. Physical examination revealed hypertrophy and formation of folds in the parietal-occipital area, forming 5 to 6 furrows and ridges. The size of the cerebriform mass was about 12.0 cm × 8.5 cm, without other skin lesions. Diffuse non-scarring hair loss was distributed on the posterior-parietal scalp, mid-parietal scalp and superior-occipital scalp. The diseased tissue of the patient's parietaloccipital area was excised under general anesthesia. The postoperative pathological examination of the tissue excised showed that there were dense intradermal melanocytic nevus, so the patient was diagnosed with secondary CVG caused by CIN. At the 2 year follow-up, there were no obvious changes in the lesions.ConclusionsCIN must be differentiated from other conditions that manifest as CVG, including primary essential or non-essential CVG and secondary CVG caused by other reasons. Each CIN patient requires a specific decision of whether to excise the lesion surgically or follow a wait-and-see policy, depending on the patient's will and specific condition. Surgical treatment may be performed when there is an aesthetic demand. However, clinical observation and close follow-up is also a good treatment choice for patients with stable disease or mild symptoms.

Cerebriform intradermal nevus presenting as cutis verticis gyrata: A rarity

Cutis verticis gyrata (CVG) is a rare skin disorder characterized by cerebriform appearance of the scalp with convoluted folds and deep furrows. Cerebriform intradermal nevus (CIDN) is one of the rare causes of CVG. We report a case of CIDN presenting as CVG on the left parietal area of scalp since birth in a 15-year-old girl. She had no neurological or ophthalmologic manifestations or any other cutaneous lesions.

Cutis verticis gyrata secondary to giant cerebriform intradermal nevus in a pediatric patient

Cutis verticis gyrata (CVG) is a long-lasting and progressive condition in which there is hypertrophy and folding of the scalp skin. Cerebriform intradermal nevus (CIDN) is a rare cause of CVG. Here, we report a case with CIDN of the scalp in a 12-year-old girl. Dermatological examination showed a cerebriform appearance with accentuating folds and deep furrows, thickening in the temporal regions. We want to emphasize that CIDN should not be forgotten in the differential diagnosis of the causes of CVG.

Scalp Reconstruction With Free Latissimus Dorsi Flap in a Patient With Giant Cutis Verticis Gyrata.

Cutis verticis gyrata is a rare skin disorder characterized by formation of thick skin folds and grooves. Because of the risk of malignant transformation, these lesions must be surgically excised. In this study, the authors aimed to present a successful reconstruction of scalp with free latissimus dorsi musculocutaneous flap after total excision of giant cerebriform intradermal nevus that causes cutis verticis gyrata. An 18-year-old male patient attended to our clinic with a large tumor that covers almost 80% of his scalp. Lesion is diagnosed as cerebriform intradermal nevus. Excision of the tumor and scalp reconstruction with free latissimus dorsi musculocutaneous flap is planned and the patient is operated. Free tissue transfer is one of the best choices for full thickness defects that covers 80% of scalp with its excellent reconstructive and aesthetical results. Reconstruction with free flaps reduces the number of operations necessary to cover the defect unlike serial tissue expansion or staged excision methods. Free flaps are also a good alternative when the patient has not enough hair-bearing tissue to expand. Low donor site morbidity also makes free latissimus dorsi musculocutaneous flaps excellent choice. Cerebriform intradermal nevus is one of the most common causes of cutis verticis gyrata and has an importance due to the current risk of malign transformation. Latissimus dorsi musculocutaneous free flap is a reliable and aesthetically acceptable reconstruction option in the patients of giant cutis verticis gyrata due to sufficient pedicle length, reliable vascularized tissue volume, and large surface area.

Giant cerebriform intradermal nevus in a young girl.

Giant cerebriform intradermal nevus in a young girl.

Cerebriform intradermal nevus presenting as cutis verticis gyrata with multiple cellular blue nevus over the body: A rare occurrence

Cutis verticis gyrata is a rare skin condition characterized by swelling of scalp resembling the surface of the brain. Various conditions, like cerebriform intradermal nevus (CIN), may give rise to this clinical entity. Moreover, its association with cellular blue nevus is extremely rare and has not been reported so far. Here, we report a 28-year-old male with a huge cerebriform swelling covering the occipital lobe along with multiple nodules all over the body. Histology of the scalp swelling showed solitary or clusters of nevus cells in the dermis and from the body lesions showed features of cellular blue nevus. The diagnosis of CIN with cellular blue nevus was confirmed

Cerebriform intradermal nevus: A rare entity and its associations

Cerebriform intradermal nevus: A rare entity and its associations

English

Cerebriform intradermal nevus is a rare form of cutis verticis gyrata. Clinically it manifests as a scalp deformity resembling the surface of the brain, with cerebriform morphologic characteristics these lesions may become malignant or infected. Here we report a 20 year old boy, with cerebriform and soft fold on right temporo-parietal area present since birth. The clinical and histopathological (solitary or clusters of nevus cells) presentations of cerebriform intradermal nevus are described and the therapeutic possibilities discussed. INTRODUCTION: Cutis verticis gyrata (CVG) is a skin deformity characterized by excessive growth of the skin of the scalp, resulting in furrows and folds which are similar to gyri of the brain cortex. CVG can be classified into two forms: 'primary' (essential and non-essential) and 'secondary'. The primary non-essential type is associated with the neurological and ophthalmological changes, while the primary essential form does not present any other disorder except for the cutaneous alterations. Secondary forms of CVG are much more frequent and accompany other pathologies. The treatment consists of local hygiene and surgical resection of skin excess. Intradermal cerebriform nevus is a rare entity, predominantly affecting females at birth or during the first years of life. The condition usually begins with a macular lesion that develops into parietal and occipital symmetric convolutions, occupying one half to three quarters of the scalp6, 7. It has a growth peak at puberty. The mechanism for the cerebriform pattern on the surface of the intradermal cerebriform nevus is unknown. 12

Dermoscopic features of cerebriform intradermal nevus as secondary cutis verticis gyrata

International Journal of DermatologyVolume 53, Issue 3 p. 346-348 Case Report Dermoscopic features of cerebriform intradermal nevus as secondary cutis verticis gyrata María Huerta-Brogeras MD, PhD, María Huerta-Brogeras MD, PhD Department of DermatologySearch for more papers by this authorLuz Martin Fragueiro MD, Luz Martin Fragueiro MD Department of Pathology, Hospital Universitario de Fuenlabrada, Madrid, SpainSearch for more papers by this authorDiego Martínez-Sánchez MD, Diego Martínez-Sánchez MD Department of DermatologySearch for more papers by this authorEsther Castaño MD, PhD, Esther Castaño MD, PhD Department of DermatologySearch for more papers by this authorOlga Nieto Perea MD, Olga Nieto Perea MD Department of DermatologySearch for more papers by this authorJesús Borbujo MD, PhD, Jesús Borbujo MD, PhD Department of DermatologySearch for more papers by this author María Huerta-Brogeras MD, PhD, María Huerta-Brogeras MD, PhD Department of DermatologySearch for more papers by this authorLuz Martin Fragueiro MD, Luz Martin Fragueiro MD Department of Pathology, Hospital Universitario de Fuenlabrada, Madrid, SpainSearch for more papers by this authorDiego Martínez-Sánchez MD, Diego Martínez-Sánchez MD Department of DermatologySearch for more papers by this authorEsther Castaño MD, PhD, Esther Castaño MD, PhD Department of DermatologySearch for more papers by this authorOlga Nieto Perea MD, Olga Nieto Perea MD Department of DermatologySearch for more papers by this authorJesús Borbujo MD, PhD, Jesús Borbujo MD, PhD Department of DermatologySearch for more papers by this author First published: 22 August 2013 https://doi.org/10.1111/j.1365-4632.2012.05535.xCitations: 5 María Huerta-Brogeras, MD, PhD Calle del Molino 2 CP 28942 Fuenlabrada Madrid Spain E-mail: [email protected] Conflicts of interest: None. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume53, Issue3March 2014Pages 346-348 RelatedInformation

Aesthetic Reconstruction of Congenital Cerebriform Intradermal Nevus by Sequential Tissue Expansion at an Early Age: Ten-year Follow-up

DOI: 10.7247/jiumf.19.3.9 Cerebriform intradermal nevus (CIN) is a congenital tumoral abnormality of the scalp. The incidence of development of malignant melanoma in CIN can rise up to 10%. Surgical excision of the lesion is the sole treatment modality for CIN. In this case, we present the 10year follow-up of a 45-day-old infant with congenital CIN covering 75% of his scalp who had scalp reconstruction by sequential tissue expansion. Sequential tissue expansion processes with rectangular-shaped expanders of 450, 400, and 250 ml in size, respectively, were applied over an eight-month period. The interval between the two procedures was two weeks. The tissue expansion technique is a useful option for hair-bearing scalp reconstruction. This technique can be used sequentially or gradually. In order to obtain homogenous follicle distribution, the rest of the scalp should be expanded as much as possible. Careful planning in large defects helps prevent complications. In this case, we applied a sequential tissue expansion procedure when the patient was 45 days old, and the expansion period was continued for eight months. This very early age is a unique case for tissue expansion applications when compared with examples in the literature. On the other hand, we want to underline the aesthetic result of this case rather than the reconstructive method. Reconstruction of huge scalp defects by re-expansion (sequential expansion) and leaving the expander beneath the expanded flap until the sequential procedure decrease flap contracture reduces the required number of operations, and produces better aesthetic outcomes.

A case of cutis verticis gyrata secondary to giant cerebriform intradermal nevus

A case of cutis verticis gyrata secondary to giant cerebriform intradermal nevus

Giant Ceribriform Intradermal Nevus on the Back of a Newborn

A male newborn had a large cerebriform tumor covering his shoulders and almost the entire surface of his back. After exclusion of further abnormalities, the diagnosis of cerebriform intradermal nevus was made. This particular variant of giant melanocytic nevus should always be differentiated from cutis verticis gyrata, if located on the vertex. The clinical manifestation of cerebriform intradermal nevi as giant melanocytic nevi on the back is extremely rare, with only one instance reported to date. Such nevi are a therapeutic challenge, particularly if the skin lesion covers a large surface of the body, as in the patient presented here.

Cerebriform Intradermal Nevus

Cerebriform intradermal nevus is a rare form of cutis verticis gyrata. Clinically it manifests as a scalp deformity resembling the surface of the brain, with cerebriform morphologic characteristics. Degeneration into malignant melanoma has been reported. Herein, a cerebriform intradermal nevus of the scalp in a 7-year-old girl is reported. The clinical and histopathologic presentations of cerebriform intradermal nevus are described and the therapeutic possibilities discussed.

Elevated Serum Antimelanocyte Antibody Level in Cerebriform Intradermal Nevus with Vitiligo

We describe a rare, but typical case of cerebriform intradermal nevus associated with vitiligo. A 45-year-old man had a patch of alopecia over his vertex scalp for 15 years. The microscopic findings of the biopsy revealed a typical deep-seated intradermal nevus and neuroid differentiation with a few pigments. Three hypopigmented patches developed on the forehead, cheek and index finger five years after the scalp lesion, with loss of both melanocytes and melanins. In addition, no dopa reactions were present. Compared to normal controls, the serum anti-melanocyte antibody level in the patient was elevated as determined by cellular enzyme-linked immunosorbent assay (cellular ELISA). This is the first reported case with elevation of serum antimelanocyte antibody level of cerebriform intradermal nevus with vitiligo. This antibody's presence may be related to the occurrence of the vitiligo in patient with cerebriform intradermal nevus.

Essential primary cutis verticis gyrata

OBJECTIVE: To report a rare case of a child with essential primary Cutis verticis gyrata. REPORT: Nine-year-old boy with extensive hypertrophy of scalp skin, with a cerebriform appearance. No underlying neurologic and ophthalmologic disorders were found, and no other cases were described in his family. COMMENTS: The diagnosis of primary Cutis verticis gyrata was established by thickening of the scalp and absence of neurologic and ophthalmologic abnormalities. Differential diagnosis comprises secondary conditions such as: cerebriform intradermal nevus, pachydermoperiostosis, acromegaly, and inflammatory diseases of the scalp. This is the first report of a child with this form of Cutis verticis gyrata.

Cerebriform intradermal nevus

A case of cerebriform melanocytic intradermal nevus is presented, because of its extreme rarity, familial characteristics, and the good result obtained with total excision and skin grafting. The treatment is of interest because these lesions may become malignant or infected, and there can be aesthetic problems which could result in behavioral difficulties and the patient feeling socially rejected. We insist on the importance of following a systematic study of these patients, and possible extracutaneous lesions must be ruled out. The cerebriform intradermal nevus is a benign tumor, which may appear at birth or at an early age. It is located on the scalp, and its external appearance is similar to that of the brain.

Cutis verticis gyrata associated with chronic schizophrenia in Chinese

Cutis verticis gyrata (CVG) is an unusual conformation of the scalp, characterized by the presence of folds and furrows that impart to the scalp a corrugated or a gyrate appearance, which resembles the human cerebrum. CVG is classified into primary and secondary by etiology (Polan and Butterworth 1953). CVG caused by chronic inflammatory dermatoses , cerebriform intradermal nevus , neurofibroma, dermatofibroma, and miscellaneous systemic diseases (acromegaly, syphilis, leukemia, etc.) is secondary. When there is no identified cause, it is primary (Orkin et al. 1974). Secondary CVG is found in mentally normal persons. However, primary CVG is usually found in mentally abnormal persons, with or without neurological deficits (Orkin et al. 1974). The prevalence of CVG in the Chinese population is unknown. However, primary CVG associated with mental retardation (Tsuang and Lin 1974) and secondary CVG in three patients with acromegaly (Li 1955) have been reported. In this report, the author presents 25 cases of CVG associated with chronic schizophrenia found in a Chinese population. In July 1986, 1244 male patients hospi~i~d in Yuli Long Stay Mental Institute (located in Eastern Taiwan) were inspected by the author to screen out cases of CVG. Patients with CVG were then investigated by a consultant dermatologist to make a differential diagnosis and to establish a final diagnosis. The histories of the patients with CVG were reviewed by the author, including both physical and psychiatric illnesses, and present physical and mental status was evaluated. As disappearance of CVG after castration was reported (Hellerstrom 1933), Akesson attempted ethinyl estradiol treatment with two patients without effect (Akesson 1964). The present author attempted a further trial by administering 1.25 mg conjugated estrogen once daily for 2 months to three patients who agreed to this, but without cessation of their antipsychotic medication. Given the possibility that longterm administration of antipsychotic drugs might induce CVG, antipsychotic medication (including haloperidol, chropromazine, and thioridazine) was suspended for 2 months in 7 patients who agreed to do so, in order to test this possibility,