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Cutis Verticis Gyrata (CVG) is an uncommon condition, often classified as primary (idiopathic) or secondary to other diseases or syndromes. Its pathogenesis remains poorly understood, and its association with genetic syndromes is particularly rare. Noonan and Turner syndromes are distinct genetic disorders with characteristic phenotypes and multiple systemic involvements. This report aims to highlight the diagnostic complexities when CVG presents in the backdrop of these syndromes. A 38 years old patient was presented with chief complaints of receding hairline, dropping eyelids, cerebral deformations with deep furrows and thickened dermis. On the basis of patient's complaints, Noonan or turner syndrome was considered as possible diagnosis. This particular report presents a case of patient suffering from CVG having history of noonan and turner syndrome. With the detailed MRI, histology etc. CVG was finally confirmed. The novelty of this case lies in its rarity, diagnostic complexity, and the need for a multidisciplinary approach to unravel and manage the intersecting conditions. It contributes valuable insights to the existing medical literature, enhancing our understanding of the interplay between dermatological and genetic conditions. Patients with Noonan and turner syndrome exhibit clinical signs and symptoms that are strikingly similar to those of CVG, suggesting that this presents a significant diagnostic problem. An unfavorable outcome could arise from delayed or incorrect diagnosis. Because of this, it is recommended that healthcare fraternities should include uncommon illnesses like CVG as differential diagnosis. Considering CVG in differential diagnosis is crucial for early identification, accurate diagnosis, and comprehensive management. It ensures that associated systemic and genetic conditions are not overlooked and that patients receive holistic and personalized care.

Cutis verticis gyrata (CVG) is a rare skin condition characterized by ridges and furrows resembling the brain. CVG falls under three categories: primary essential, primary nonessential, and secondary. This case report focuses on primary essential CVG, where approximately a fourth of the scalp and a significant portion of the forehead and eyelid were involved.

Introduction. A differential diagnostic search is a crucial method for making a final clinical diagnosis. Lack of clinical thinking leads to tactical errors, both in making a diagnosis and in choosing a therapy algorithm. The problem of iatrogeneses especially often arises at the initial stages of patient management.Case descriptions. The paper demonstrates the difficulties of verifying the diagnosis of patients with dermatological pathology on the example of clinical cases. The stages of differential diagnostic search are covered in detail. Practicing physicians stress the importance of timely correct diagnosis and, consequently, the choice of therapy. The study involved a detailed analysis of clinical cases and mistakes causing a delay in diagnosis and wrong choice of therapeutic tactics at the outpatient stage. A clinical case with designated diagnosis is presented and analyzed as an illustrative example. Special attention was given to a complex evaluation of subjective, objective and diagnostic data, the significance of analyzing the development of dermatoses.Conclusion. Careful adherence to diagnostic algorithms will avoid iatrogenic errors and thereby improve the quality of medical care and the quality of life of dermatological patients.

Bulldog scalp syndrome or cutis verticis gyrata (CVG) is a rare cutaneous disorder with an incidence of just 0.026 to 1 per 100,000 population and cosmetic problems should not be ignored as they can affect the quality of life of patients in social and psychological aspects. In CVG the scalp thickens to form folds resembling sulci and gyri just as the skin fold of bulldog. It is a clinical diagnosis with various etiologies. It is classified as primary essential or nonessential and secondary CVG. It can manifest with symptoms ranging from mild to severe intensity. Cosmetic problems are the major concern that can affect patients' social and psychological health. If the folds are heavy, they can cause mass symptoms. Thus, surgery remains the definitive treatment option for improving the cosmetic appearance. Both our cases have different etiologies, however, were managed surgically with removal of skin folds (gyrae) and scoring of aponeuroses of the scalp followed by stretching of the scalp and closure to improve appearance. The surgical team as well as patients were satisfied with the appearance of the scalp after healing. CVG though a rare disease with various etiologies is a benign condition with good prognosis with no reports of malignant transformation so far.

Cutis verticis gyrata (CVG) is a rare skin condition characterized by ridges and furrows resembling the brain. CVG falls under three categories: primary essential, primary nonessential, and secondary. This case report focuses on primary essential CVG, where approximately a fourth of the scalp and a significant portion of the forehead and eyelid were involved. Flap advancement after skin expansion was performed to rectify the disorder. This technique adequately covers the residual defect postexcision and preserves hair growth in affected regions. It is a successful skin expansion technique to cover the exposed scalp, preserve hair growth, and achieve excellent cosmetic results. Our approach demonstrates a promising solution for severe cosmetic disfigurement in primary essential CVG, positively impacting both the physical appearance and psychosocial well-being of the patient. [ABSTRACT FROM AUTHOR]

Pachydermoperiostosis (PDP), also known as Touraine-Solente-Gole syndrome or primary hypertrophic osteoarthropathy, is a very rare disease mainly characterized by pachyderma, finger clubbing, hyperhidrosis, and periostosis. We reported a case of a patient who suffered from gradual pain and enlargement of both hands and feet for 25 years despite receiving treatment from multiple clinics. He also experienced gradual abnormal changes in his face and scalp. Radiographic images of the lower limbs revealed the presence of subperiosteal bone growth and periosteal hypertrophy. The diagnosis of complete PDP was made late due to its rarity and the unfamiliarity of medical practitioners with the diagnosis. This case highlighted the need for medical practitioners to be aware of rare diseases so that patients may be diagnosed and treated earlier and thereby relieving their anxiety and improve their quality of life. [ABSTRACT FROM AUTHOR]

Cutis verticis gyrata (CVG) is a cutaneous benign condition that usually presents symmetric or asymmetric convoluted folds and deep furrows in the scalp, which mimic the disposition of the cerebral sulci and gyri. This scalp deformation may be a worrying situation for the patients and their families. Clinically, its diagnosis may be challenging because it can mimic other cutaneous conditions. So far, the ultrasonographic pattern of CVG has not been reported. Thus, we aim to review the ultrasound findings of this entity. All patients (n = 14) presented zones with dermal and hypodermal thickening that corresponded with the elevated clinical zones, followed by folds with normal cutaneous thicknesses. There was undulation of the cutaneous layers in all cases. The scalp areas involved the frontal, parietal, and occipital regions bilaterally. The mean maximum dermal and hypodermal thicknesses and echogenicities and the color Doppler characteristics are provided. No significant dilation of the hair follicles was detected. In conclusion, CVG presents an ultrasonographic pattern that can support its diagnosis and follow‐up. This can help its differential diagnosis with other scalp dermatologic conditions. [ABSTRACT FROM AUTHOR]

Bulldog scalp syndrome or cutis verticis gyrata (CVG) is a rare cutaneous disorder with an incidence of just 0.026 to 1 per 100,000 population and cosmetic problems should not be ignored as they can affect the quality of life of patients in social and psychological aspects. In CVG the scalp thickens to form folds resembling sulci and gyri just as the skin fold of bulldog. It is a clinical diagnosis with various etiologies. It is classified as primary essential or nonessential and secondary CVG. It can manifest with symptoms ranging from mild to severe intensity. Cosmetic problems are the major concern that can affect patients' social and psychological health. If the folds are heavy, they can cause mass symptoms. Thus, surgery remains the definitive treatment option for improving the cosmetic appearance. Both our cases have different etiologies, however, were managed surgically with removal of skin folds (gyrae) and scoring of aponeuroses of the scalp followed by stretching of the scalp and closure to improve appearance. The surgical team as well as patients were satisfied with the appearance of the scalp after healing. CVG though a rare disease with various etiologies is a benign condition with good prognosis with no reports of malignant transformation so far. [ABSTRACT FROM AUTHOR]

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.

Background: Cutis verticis gyrata (CVG) is a condition of excessive skin growth and excessive laxity of the scalp, leading to deep furrows and folds that resemble the gyri and outer surface of the brain. Approaches for the treatment of CVG range from conservative to surgical, the last one being the predominant way of treating the condition, however, the surgery proposed in the recent literature may not be suitable for patients who desire a less invasive approach. Aim: To report the first case of autologous fat injection as a novel treatment option for primary essential CVG. Methods: A 51 -year-old Colombian man, with no medical records was treated with a scalp injection of autologous fat, with previous failed treatment with pressure therapy apply directly on skin is described. Results: A successful cosmetic improvement and a less invasive approach was reached after two sessions of autologous fat grafting. Conclusion: This method allowed excellent cosmetic outcomes while preserving the option of subsequent surgical repair in refractory cases.

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 Ã--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. [ABSTRACT FROM AUTHOR]

Cutis verticis gyrata is a rare pathology of the scalp characterized by the proliferation of subcutaneous cellular tissue and hypertrophy that produces folds and furrows that give it a cerebriform appearance. Although its etiology is still unknown, the condition is not exclusively congenital, the presence of autosomal dominant mutations in the fibroblast growth factor receptor 2 has been proposed as a theory. This pathology is extremely rare in pediatrics. It is divided into primary and secondary. The case of a 15-year-old male adolescent with the presence of essential primary cutis verticis gyrata, where the main concern was pruritus and the presence of fetidity is presented. Taking into account the benign nature of this condition, only symptomatic management was decided. The case presented is the first reported in Cienfuegos province in pediatric age, which reaffirms the importance of recognizing this entity and its adequate approach to distinguish the clinical forms of presentation for its best therapeutic treatment. Once the oddity of the entity in pediatric age was given this case, in addition to a revision of literature decides to show up.

Abstract Background Noonan and Noonan-like syndromes are multisystem genetic disorders, mainly with autosomal dominant trasmission, caused by mutations in several genes. Missense pathogenetic variants of SOS1 gene are the second most common cause of Noonan syndrome (NS) and account approximately for 13% to 17% of cases. Subjects carrying a pathogenetic variant in SOS1 gene tend to exhibit a distinctive phenotype that is characterized by ectodermal abnormalities. Cutis verticis gyrata (CVG) is a rare disease, congenital or acquired, characterized by the redundancy of skin on scalp, forming thick skin folds and grooves of similar aspect to cerebral cortex gyri. Several references in the literature have reported association between nonessential primary form of CVG and NS. Case presentation we report two cases of newborns with CVG and phenotype suggestive for NS who have been diagnosed to harbour the same pathogenetic variant in SOS1 gene. Conclusions previously described patients with NS presenting CVG had received only clinical diagnosis. Therefore we report the first patients with CVG in which the clinical suspicion of NS is confirmed by molecolar analysis.

Yifan Wang,1– 3 Shan Wang,1– 3 Liyun Zheng,1– 3 Xiuli Wang,1– 3 Hui Wang,1– 3 Zhenyu Zhong,1– 3 Siqi Liu,1– 3 Xiaodong Zheng,1– 3 Min Gao1– 3 1Department of Dermatology, the First Affiliated Hospital of Anhui Medical University, Hefei, Anhui, People’s Republic of China; 2Key Laboratory of Dermatology (Anhui Medical University), Ministry of Education, Hefei, Anhui, People’s Republic of China; 3Inflammation and Immune Mediated Diseases Laboratory of Anhui Province, Hefei, People’s Republic of ChinaCorrespondence: Min Gao; Xiaodong Zheng, Department of Dermatology, the First Affiliated Hospital of Anhui Medical University, No. 81 Meishan Road, Hefei, Anhui, 230032, People’s Republic of China, Tel/Fax +86-0551-62923042, Email ahhngm@126.com; zhengxiaodong.1234@163.comAbstract: SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare disease clinically characterized by a wide range of cutaneous and osteoarticular manifestations. Here, we report a case of SAPHO syndrome with cutis verticis gyrata (CVG) and investigated the genetic causes in the four members of this pedigree. After failure of conventional treatments, a recombinant human TNF-α receptor II:IgG Fc fusion protein (rhTNFR:Fc, YISAIPU®) achieved good control of the disease at the 2-year follow-up. We did not identify any pathogenic mutation in this pedigree. We also summarized the clinical and therapeutic characteristics of 83 patients with SAPHO syndrome through the China National Knowledge Infrastructure (CKNI) database from 2016 to 2021. Patients with acne were young and predominantly male. About 45.8% patients were treated with biological therapies or traditional Chinese medicine (TCM), 84.2% of which showed positive effects against cutaneous and osteoarticular manifestations. We report a case of SAPHO syndrome with CVG that was successfully treated with rhTNFR:Fc. Our results reveal the genetic heterogeneity involved. Biologics and TCM are likely alternative options for the treatment of SAPHO syndrome.Keywords: cutis verticis gyrata, gene mutation, SAPHO syndrome, treatment, whole-exome sequencing

Cutis verticis gyrata (CVG) is classified as primary or secondary according to the absence or presence of underlying soft tissue abnormalities. We report an infant with Turner syndrome (TS) who in addition presented with CVG on the scalp. The skin biopsy revealed a hamartoma‐like lesion. We reviewed the clinical and histopathological findings of the 13 reported cases of congenital CVG in patients with TS, including ours. In 11 of them, CVG was localized on the skin of the scalp, mainly on the parietal region, and in two, on the forehead. Clinically, CVG had a flesh‐colored aspect, with absent or sparse hair, and was not progressive. CVG was classified as primary in four patients who had skin biopsy and it was attributed to the intrauterine lymphedema of TS. However, histopathology in two of these patients identified dermal hamartoma as a secondary cause of CVG, and in three others, including ours, there were hamartomatous changes. Although further studies are required, previous findings support the proposal that some CVG may instead be dermal hamartomas. This report alerts clinicians to recognize CVG as a low‐frequency manifestation of TS, but also to consider the possible co‐occurrence of TS in all female infants with CVG. [ABSTRACT FROM AUTHOR]

T‐cell lymphoblastic lymphoma (T‐LBL) is frequently revealed by amediastinal mass or peripheral lymphadenopathy. Skin lesions in T‐LBLusually present as multiple nodules associated with multiple peripherallymphadenopathy and bone marrow invasion. Our patient is particular bythe revealing presentation of the lesions as Cutis verticis gyrate. T‐cell lymphoblastic lymphoma (T‐LBL) is frequently revealed by a mediastinal mass or peripheral lymphadenopathy. Skin lesions in T‐LBL usually present as multiple nodules associated with multiple peripheral lymphadenopathy and bone marrow invasion. [ABSTRACT FROM AUTHOR]

Pachydermoperiostosis is a rare syndrome that affects the skin and skeletal system. Mutations in the gene encoding hydroxyprostaglandin dehydrogenase (HPGD) are thought to play a role in disease etiopathogenesis. The disease is typically seen in males and is characterized by skin thickening (pachydermia), clubbing, and subperiosteal new bone formation. A 57-year-old male patient was admitted to our clinic with growth complaints of growth his hands, feet, and face. He had a prominence of forehead lines and fist-sized round bulges on his torso and face. The patient’s history also indicated that he was clinically followed up for neurofibromatosis 1 (NF1). No mutations in the HPGD and SLCO2A1 genes were identified. This is the first case in the literature that reviews pachydermoperiostosis and NF together. The coexistence of skin findings of two different diseases in the same patient indicates that both diseases may be related to different genetic pathways.

Abstract T‐cell lymphoblastic lymphoma (T‐LBL) is frequently revealed by amediastinal mass or peripheral lymphadenopathy. Skin lesions in T‐LBLusually present as multiple nodules associated with multiple peripherallymphadenopathy and bone marrow invasion. Our patient is particular bythe revealing presentation of the lesions as Cutis verticis gyrate.

Background: Noonan and Noonan-like syndromes are multisystem genetic disorders, mainly with autosomal dominant trasmission, caused by mutations in several genes. Missense pathogenetic variants of SOS1 gene are the second most common cause of Noonan syndrome (NS) and account approximately for 13% to 17% of cases. Subjects carrying a pathogenetic variant in SOS1 gene tend to exhibit a distinctive phenotype that is characterized by ectodermal abnormalities. Cutis verticis gyrata (CVG) is a rare disease, congenital or acquired, characterized by the redundancy of skin on scalp, forming thick skin folds and grooves of similar aspect to cerebral cortex gyri. Several references in the literature have reported association between nonessential primary form of CVG and NS. Case presentation: we report two cases of newborns with CVG and phenotype suggestive for NS who have been diagnosed to harbour the same pathogenetic variant in SOS1 gene. Conclusions: previously described patients with NS presenting CVG had received only clinical diagnosis. Therefore we report the first patients with CVG in which the clinical suspicion of NS is confirmed by molecolar analysis. [ABSTRACT FROM AUTHOR]

Abstract Background Cutis 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 presentation A 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. Conclusions CIN 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.

Cutis Vertcis gyrata is an uncommon neurocutaneous syndrome characterized by excessive growth of the skin of the scalp or the face, forming folds of similar aspect to cerebral cortex gyri. Three categories have been individualized: the primary form, essential or non‐essential, and the secondary form. [ABSTRACT FROM AUTHOR]

Hypertrophic osteoarthropathy (HOA) is a disease characterized by abnormal skin findings and bone deformities related to subperiosteal bone formation. The disease can be associated with major systemic manifestations (secondary form) or present with absent or less prominent systemic signs and symptoms (primary form). The primary form is called pachydermoperiostosis (PDP). Whole body diffusion weighted imaging with background suppression (WB-DWIBS) is a magnetic resonance imaging (MRI) technique that has been used to highlight whole body involvement in various entities by suppressing background body signals, and is commonly used in oncologic work-ups. In this paper, we present the case of a 23-year-old male presenting with normocytic anemia and coarse facial features, as well as biological anomalies, and we report the use of WB-DWIBS in establishing the patient's diagnosis of PDP. • Hypertrophic osteoarthropathy can be primary or secondary. • Pachydermoperiostosis is the primary form of HOA. • Pachydermoperiostosis can be associated with systemic manifestations. • WB-DWIBS can be used in systemic processes to detect extent of disease involvement. • WB-DWIBS may be useful in pachydermoperiostosis to detect systemic manifestations. [ABSTRACT FROM AUTHOR]

Abstract Cutis Vertcis gyrata is an uncommon neurocutaneous syndrome characterized by excessive growth of the skin of the scalp or the face, forming folds of similar aspect to cerebral cortex gyri. Three categories have been individualized: the primary form, essential or non‐essential, and the secondary form.

Objectives: Cerebriform intradermal nevus and giant congenital blue nevi are rarely reported melanocytic nevi with clinical and histopathologic similarities. Both are known to produce cutis verticis gyrata. We report a significantly large occipital scalp congenital blue nevus with secondary cutis verticis gyrata. The aim of this report is to increase clinical awareness of this entity, highlight histopathologic and mutational features of cerebriform intradermal nevi and giant congenital blue nevi, and stress the importance of clinicopathologic correlation for diagnosis. Methods: Case report and review of the literature. Results: A 20-year-old Asian male presented with a long-standing, large (20 cm × 30 cm), exophytic tumor at the occipital scalp and posterior neck. The skin overlying the lesion was arranged in thick folds resembling the surface of the brain, devoid of hair follicles, and discolored by salt-and-pepper pattern hyperpigmentation. After correlating the clinical and histopathologic findings, we diagnosed giant congenital blue nevus with secondary cutis verticis gyrata. Staged surgical excision was performed with subsequent treatment for hypertrophic scarring and occipital alopecia. Conclusions: Cerebriform intradermal nevus and giant congenital blue nevus have overlapping histologic and clinical features. Head and neck surgeons should be aware that nomenclature of these tumors is subjective and often imprecise. Diagnosis requires correlation of clinical findings, patient history, and histopathology. Surgical excision is advised due to rare malignant transformation potential. [ABSTRACT FROM AUTHOR]

Cutis verticis gyrata (CVG) is a rare skin disorder characterised by thick skin folds and grooves, resembling the gyri and sulci of the cerebral cortex. It can be classified as primary essential, primary non-essential and secondary CVG. The rarity of the condition poses a challenge in the diagnosis and ultimately requires good communication between the clinician and the dermatopathologist for diagnosis. Many names have been given to the condition, including Robert-Unna syndrome, cutis verticis plicata, paquidemia verticis gyrata, cutis sulcata, cutis striata or “bulldog” scalp syndrome. To date, the treatment is still limited to plastic surgeons with excision of the folds, scalp reduction and reconstruction. In this article, we are sharing a rare case and the surgical management of a middle age gentleman with cutis verticis gyrata secondary to cerebral intradermal nevus (CIN) over his occipital scalp.

Abstract Cutis verticis gyrata (CVG) is a neurocutaneous syndrome characterized by the formation of folds in the scalp that resembles the cerebral cortex. We present two cases of CVG and intellectual disability with drug‐resistant epilepsy. Recognizing CVG is necessary to provide interdisciplinary support for the treatment of comorbidities associated with this entity.

Background: Cutis verticis gyrata (CVG) is a rare condition characterized by deep skin folds over scalp. The secondary CVG can occur due to conditions including malignancies such as leukemia. However, there are limited reports on lymphoma as a secondary etiology for CVG, to date. Case Report: A 41-year-old man patient came to our dermatology clinic complaining of generalized pruritus, deep skin folds on scalp, and weight loss from 3 years ago, as well as chronic cough from one year ago. On examination, he had left axillary and supraclavicular lymphadenopathy. Since mediastinal lymphadenopathy was reported on his lung high-resolution computed tomography, a lymph node biopsy was done, and a diagnosis of Hodgkin lymphoma was made. Chemotherapy was commenced for the patient, and after two sessions, his pruritus and cough vastly improved. Additionally, at a follow-up session 3 years after admission, his CVG improved. Conclusion: This case report is one of limited available reports about lymphoma as a secondary cause of CVG. Timely diagnosis of secondary causes of CVG, including hematological malignancies, could be of critical importance. In addition, in this case, treatment of the underlying cause of CVG resulted in an improvement in CVG itself.

Cutis Verticis Gyrata (CVG) is an uncommon condition, often classified as primary (idiopathic) or secondary to other diseases or syndromes. Its pathogenesis remains poorly understood, and its association with genetic syndromes is particularly rare. Noonan and Turner syndromes are distinct genetic disorders with characteristic phenotypes and multiple systemic involvements. This report aims to highlight the diagnostic complexities when CVG presents in the backdrop of these syndromes. A 38 years old patient was presented with chief complaints of receding hairline, dropping eyelids, cerebral deformations with deep furrows and thickened dermis. On the basis of patient's complaints, Noonan or turner syndrome was considered as possible diagnosis. This particular report presents a case of patient suffering from CVG having history of noonan and turner syndrome. With the detailed MRI, histology etc. CVG was finally confirmed. The novelty of this case lies in its rarity, diagnostic complexity, and the need for a multidisciplinary approach to unravel and manage the intersecting conditions. It contributes valuable insights to the existing medical literature, enhancing our understanding of the interplay between dermatological and genetic conditions. Patients with Noonan and turner syndrome exhibit clinical signs and symptoms that are strikingly similar to those of CVG, suggesting that this presents a significant diagnostic problem. An unfavorable outcome could arise from delayed or incorrect diagnosis. Because of this, it is recommended that healthcare fraternities should include uncommon illnesses like CVG as differential diagnosis. Considering CVG in differential diagnosis is crucial for early identification, accurate diagnosis, and comprehensive management. It ensures that associated systemic and genetic conditions are not overlooked and that patients receive holistic and personalized care., Competing Interests: The authors declare that they have no financial conflict of interest with regard to the content of this report., (© 2024 The Authors.)

Background: The quality of life of patients with Cutis Verticis Gyrata (CVG) has not been previously investigated in the literature. Objective: Description of features and evaluation of quality of life of CVG patients. Materials and methods: We employed a mixed methods study. We retrospectively collected data from the clinical database from 2012 to 2020. We selected 17 clinically diagnosed CVG patients and prospectively evaluated the quality of life of these CVG patients. Results: Out of 13 patients, 11 patients were in between the 14-47 age group. The majority of them were male (77%), Caucasian (54%), had no significant family history (92.3%), and had the age of onset below < 30 years (69.2%). The majority of the patients had a secondary form of CVG (54%) followed by a primary essential form of CVG (46.1%). The CVG had a small effect on the majority of patient's quality of life (77%), and a majority of patients were not taking any treatment (69.2%) Conclusion: In our study, the CVG was shown to have a small effect on the patient's quality of life. [ABSTRACT FROM AUTHOR]

Pachydermoperiostosis is a rare syndrome that affects the skin and skeletal system. Mutations in the gene encoding hydroxyprostaglandin dehydrogenase (HPGD) are thought to play a role in disease etiopathogenesis. The disease is typically seen in males and is characterized by skin thickening (pachydermia), clubbing, and subperiosteal new bone formation. A 57-year-old male patient was admitted to our clinic with growth complaints of growth his hands, feet, and face. He had a prominence of forehead lines and fist-sized round bulges on his torso and face. The patient's history also indicated that he was clinically followed up for neurofibromatosis 1 (NF1). No mutations in the HPGD and SLCO2A1 genes were identified. This is the first case in the literature that reviews pachydermoperiostosis and NF together. The coexistence of skin findings of two different diseases in the same patient indicates that both diseases may be related to different genetic pathways. [ABSTRACT FROM AUTHOR]

Background: Cutis 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 Presentation: A 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.Conclusions: CIN 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. [ABSTRACT FROM AUTHOR]

Introduction: Pityriasis amiantacea (PA) is a rare presentation characterized by the presence of extensive adherent scaling that tightly encircles and affixes tufts of hairs secondary to inflammatory or infectious dermatoses. However, the occurrence of PA as a consequence of leukaemia cutis has not been previously reported., Case Report: A 32-year-old man with B-cell acute lymphoblastic leukaemia (B-ALL) presented with severe scalp scaling and hair loss for 2 months. Examination revealed extensive, tightly adherent scales encircling and binding down hairs, along with haemorrhagic crusts. Skin examination showed nontender, partially blanchable papules forming ill-defined plaques, giving a cobblestone appearance. Trichoscopy revealed white crusts, tufting, and micro-haemorrhages. Scalp biopsy confirmed precursor B-ALL infiltration. He was diagnosed with PA with secondary cutis verticis gyrata due to leukaemia cutis and referred to a haemato-oncologist for further management., Discussion: Leukaemia cutis is a rare condition where leukaemia cells infiltrate the skin, often associated with acute myeloid leukaemia and ALL. It can lead to secondary verticis gyrata. The co-occurrence of PA and cutis verticis gyrata is rare and previously unreported, highlighting the need for increased awareness among clinicians., Competing Interests: The authors have no conflicts of interest to declare. None of the authors report any form of support or financial involvement. There are no nonfinancial relationships (personal, political, or professional) that may potentially influence the writing of the manuscript., (© 2023 S. Karger AG, Basel.)

Pachydermoperiostosis (PDP) is a rare disorder characterized by pachydermia, digital clubbing, periostitis, and an excess of affected males. It is the primary form of hypertrophic osteoarthropathy (HOA) and there are some rare associations of PDP with other disorders. Here we describe a patient with Crohn’s disease associated with PDP. A 26-year-old man, who was a known case of Crohn’s disease, referred with diffuse swelling in the upper and lower limbs and cutis verticis gyrata since 7 years ago. PDP was suspected and endocrinological and radiological studies were conducted for the evaluation of underlying disease. He was prescribed celecoxib, low-dose prednisolone, and pamidronate to control the swelling, periostitis, azathiopurine, and mesalazine according to gastrointestinal involvement. In conclusion, it is important to identify this condition since a misdiagnosis might subject the patient to unnecessary investigations.

Cutis verticis gyrata (CVG) is a neurocutaneous syndrome characterized by the formation of folds in the scalp that resembles the cerebral cortex. We present two cases of CVG and intellectual disability with drug‐resistant epilepsy. Recognizing CVG is necessary to provide interdisciplinary support for the treatment of comorbidities associated with this entity. [ABSTRACT FROM AUTHOR]

Insulin resistance syndrome may be associated with acanthosis nigricans and obesity. The authors present the case of a 15 year-old girl with insulin resistance syndrome in which acanthosis nigricans and obesity were preceded by cerebriform hyperplasia of the scalp skin leading to a diagnosis of cutis verticis gyrata. In addition to the clinical manifestations of this noteworthy case, which was also characterized be the early age of onset, the authors put forward some hypotheses on its pathogenesis. [ABSTRACT FROM AUTHOR]

BACKGROUND: The cutis verticis gyrata is a very rare clinical sign, consisting of thickening and folding of the scalp as grooves and convolutions that simulate the surface of the brain. CLINICAL CASE: A 28-year-old male patient, black, originally from The Gambia, subjected to periodic health checks, without any pathological history of interest and without symptoms of any kind at the time of the medical visit. He attended to the office after discovering a cutis verticis gyrata in the crown area just after shaving his hair. The history and initial physical examination data were highly unremarkable and suggestive of essential primary cutis verticis gyrata. CONCLUSION: Any asymptomatic carrier of cutis verticis gyrata on the scalp with a biopsy lacking pathological elements and with normal analytical and hormonal results has a high probability of suffering from pachydermoperiostosis. [ABSTRACT FROM AUTHOR]

We report a 44‐year‐old male with cutis verticis gyrate (CVG)‐mental retardation syndrome. His backhead skin formed several folds, which were diagnosed as CVG. Neurological examinations revealed bilateral marked visual acuity loss, deaf‐mutism, mild mental retardation, bilateral Babinski's sign, severe spastic paraplegia, and cerebellar ataxia. Brain magnetic resonance imaging showed atrophy of the cerebellum and the corpus callosum. The fundus showed bilateral optic nerve atrophy. The clinical features had the primary non‐essential form in the CVG classification. Our patient was important because of the low frequency of this form in mental retardation. [ABSTRACT FROM AUTHOR]

Cutis verticis gyrata is a rare disorder characterized by redundant skin forming deep furrows and convolutions. It has been associated with several systemic and cutaneous disorders. We report a case of primary non-essential cutis verticis gyrata in association with acne keloidalis nuchae in a schizophrenic patient. [ABSTRACT FROM AUTHOR]

Cutis verticis gyrata (CVG) is a rare disease manifesting as convoluted folds and furrows, resembling sulci and gyri formed from thickened skin of the scalp. It can be congenital or acquired and primary or secondary. We report a rare case of 28-year-old male with a localized variant of primary essential CVG.

Cutis verticis gyrata (CVG) is a rare condition of the scalp in which thickening of the dermis induces rigid folds and furrows resembling the cerebral cortex. Two forms of primary CVG exist: essential, in which CVG is the only presenting problem, and nonessential, in which the scalp condition occurs along with neuropsychiatric ailments. CVG can also occur secondary to a variety of causes including inflammatory, neoplastic, and metabolic conditions or drug use. A review of the available literature, including description of the epidemiology, pathophysiology, histology, and typical management of CVG, is provided. However, we identified no literature describing the complications of CVG in the setting of a craniotomy. The patient presented here is a 54-year-old man with CVG who presented with occlusion of the M2/M2 branches of the middle cerebral artery, resulting in malignant cerebral edema, requiring emergent management via decompressive craniectomy. Because of the thickening of the scalp, skin incision was complicated by bleeding and difficulty in achieving hemostasis using Raney clips. Plastic surgery was consulted intraoperatively for assistance with complex closure of the wound in a multilayered fashion. Despite this, the patient's postoperative course was complicated by cerebrospinal fluid leakage due to difficulty in approximating the incision during closure. Subsequent cranioplasty was performed jointly between neurosurgery and plastic surgery. Despite its rarity, CVG is an important issue for neurosurgeons to understand as it can present complications in performing craniotomy, most notably during the scalp exposure and closure. CVG may also complicate the postoperative course if adequate approximation of the tissues cannot be achieved, resulting in wound infection and/or cerebrospinal fluid leak. The presented patient benefited from a combined neurosurgical and plastic surgical approach that was implemented intraoperatively and continued through the postoperative stages and the subsequent cranioplasty. [ABSTRACT FROM AUTHOR]

Background. Primary essential cutis verticis gyrata is a condition that usually affects healthy subjects associated to convoluted folds and furrows formed from thickened skin of the scalp resembling cerebriform pattern. Case. we describe a case of association between primary essential cutis verticis gyrata and new daily persistent headache. Discussion/Conclusion. In our knowledge this is the first description of new daily persistent headache associated with primary essential cutis verticis gyrata. We think that primary essential cutis verticis gyrata could be considered as a possible trigger factor, never described before, for the development of new daily persistent headache. [ABSTRACT FROM AUTHOR]

Cutis verticis gyrata (CVG) is a rare condition of the scalp characterized by convoluted folds and furrows produced by the excessive growth of the skin of the scalp and resembling the cerebral gyri. CVG can be identified as primary--essential or nonessential--or secondary. Herein, we report the case of a 20-year-old female with primary essential CVG, who presented herself with thickened and convoluted skin folds over the vertex and parietal region of the scalp persistent for one year prior without other symptoms. CVG is a rare congenital or acquired disease distinguished by redundancy of the scalp skin that resembles the cerebral gyri. The management of primary essential CVG can be symptomatic or surgical depending on the patient's aesthetic expectations. [ABSTRACT FROM AUTHOR]

Cutis verticis gyrata is a rare disease characterized by convoluted folds and deep furrows of the scalp, resembling the gyri and sulci of the cerebral cortex. Basal cell carcinoma is the most frequent cancer in Caucasians, patients frequently presenting multiple tumors. We report the case of a 62 year old male, Caucasian patient, from the urban area, who addressed the dermatology department of our hospital for multiple tumors located on the face and upper trunk. A careful examination revealed cerebriform folding of the skin of the scalp. Neurological, psychological, ophthalmological and endocrine disorders were disproven. The patient was diagnosed with cutis verticis gyrata based on the clinical picture and anamnesis, and basal cell carcinoma based on the histopathological examination. Since cutis verticis gyrata predated the BCCs by four decades, and no other conditions were associated, the patient was diagnosed with primary essential cutis verticis gyrata.

Cutis verticis gyrata is a rare skin condition characterized by convoluted folds and furrows formed from thickened skin resembling a cerebriform pattern. The condition is usually but not exclusively limited to the scalp. It may present in either primary form with or without any associations or in secondary forms with a wide variety of causes. Cerebriform intradermal nevus is one such rare cause of this unusual condition. We present a case of cerebriform intradermal nevus involving scalp and the upper half of left cheek which had both cerebriform appearances as well as the smooth surface.

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.

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. [ABSTRACT FROM AUTHOR]