Continuing Education Activity

Cutis verticis gyrata (CVG), also known by the name paquidermia verticis gyrata, cutis verticis plicata, and “bulldog” scalp syndrome, is a rare benign cutaneous disorder that is characterized by convoluted folds and deep furrows of the scalp that mimic cerebral sulci and gyri. Primary non-essential cutis verticis gyrata can be associated with neuropsychiatric and ophthalmological abnormalities. This form now has the name cutis verticis gyrata-intellectual disability (CVG-ID). Secondary and primary non-essential cutis verticis gyrata have associated abnormalities, while primary essential CVG has no associations. This activity reviews the etiology, presentation, evaluation, and management of cutis verticis gyrata and reviews the role of the interprofessional team in evaluating, diagnosing, and managing the condition.

Objectives:

• Describe the presentation of a patient with cutis verticis gyrata.
• Review the three categories of cutis verticis gyrata.
• Summarize the treatment and management strategy for cases of cutis verticis gyrata.
• Review the importance of improving care coordination among the interprofessional team to enhance the delivery of care for patients with cutis verticis gyrata.

Introduction

Cutis verticis gyrata (CVG), also known by the name paquidermia verticis gyrata, cutis verticis plicata, and “bulldog” scalp syndrome, is a rare benign cutaneous disorder that is characterized by convoluted folds and deep furrows of the scalp that mimic cerebral sulci and gyri.[1][2] It was initially reported in the medical literature in 1837 by Alibert.[2] In 1907, Unna devised the term 'cutis verticis gyrata,' which is the accepted nomenclature ever since.[3] In 1953, Butterworth classified CVG into two forms: primary and secondary.[4] In 1984, the classification system was further broken down into primary essential, primary non-essential, and secondary CVG. Primary non-essential cutis verticis gyrata can be associated with neuropsychiatric and ophthalmological abnormalities.  This form now has the name cutis verticis gyrata-intellectual disability (CVG-ID).[5][6] Secondary and primary non-essential cutis verticis gyrata have associated abnormalities, while primary essential CVG has no associations.[7][1]

Etiology

Cutis verticis gyrata has been given a classification system as CVG is a manifestation of a variety of diverse causes rather than an individual disease entity.[6] An understanding of the etiology of CVG helps classify this disease and is essential in its management.[6] A hormonal cause of cutis verticis gyrata has been postulated due to the male predominance and postpubertal onset of this disorder.[5] However, a study of plasma levels of thyroid hormone, cortisol, sex hormone, and prolactin levels in 15 patients with cutis verticis gyrata did not show any abnormal hormone levels.[6] The possible role of genetic transmission of CVG is uncertain.[3][6]

Epidemiology

Cutis verticis gyrata is a rare disorder, predominately seen in men, with a reported prevalence of about 1 in 100000 males and 0.026 in 100000 females.[1][2] Primary CVG usually occurs in postpubertal men before the age of 30, with an increased incidence seen in patients with mental disorders.[6] Secondary CVG, which is slightly more common than primary and can occur at any age with no gender predilection.[1][2] CVG rarely presents in young children.[2]

Pathophysiology

The pathogenesis of primary cutis verticis gyrata remains unclear. A hormonal influence has been postulated as this disorder usually manifests in postpubertal men.[5][3] Secondary cutis verticis gyrata is considered to be a manifestation of a variety of underlying causes, and the pathophysiology, in this case, can be correlated to the specific underlying condition.

Histopathology

The histological appearance of primary cutis verticis gyrata can show significant variation from case to case. Histological features range from essentially normal skin architecture to thickened connective tissue with hypertrophy or hyperplasia of adnexal structures with or without an associated increase in collagen fibers and trapping of the apocrine and eccrine glands.[3][7][8] The histology of the gyrated skin folds in secondary cutis verticis gyrata is that of the underlying disease process.[6]

History and Physical

Cutis verticis gyrata presents with symmetric, redundant scalp folds that exhibit deep furrows and convolutions.[1][2] The hypertrophy and folding of the skin produce an appearance mimicking that of cerebral gyri.[6][3] The multiple-thickened skin folds in primary CVG usually present in a symmetric fashion running anterior to posterior, typically involving the vertex and the occiput.[6][2] Occasionally primary CVG can be seen running in a horizontal direction and affects the entire scalp.[5] In secondary CVG, the redundant skin folds may be more asymmetrical and can even be seen on the forehead.[6] In cutis verticis gyrata secondary to inflammatory and systemic disorders, the folds will typically present in a mild corrugated pattern involving the entire scalp.[6][1] When CVG arises secondary to a neoplasm, it tends to present in the form a  localized area of scalp furrowing, that can then progress to encompass the entire scalp.[6]

Evaluation

Due to the many disorders that can cause secondary cutis verticis gyrata, it is only possible to render the diagnosis of primary CVG after complementary investigations have ruled out local or systemic diseases that are secondarily causing cutis verticis gyrata.[5][4] Corresponding investigations such as skin biopsies, blood tests, and radiological examinations are recommended based on the patient’s clinical presentation to distinguish between primary and secondary forms of CVG.[7][5]

Treatment / Management

 In secondary cutis verticis gyrata, treating the underlying disorder usually causes regression of CVG; however, sometimes further intervention may be required[6] After secondary involvement has been ruled out, primary CVG is treatable in a variety of ways. Local scalp hygiene is important to avoid secretion accumulation, maceration, unpleasant odor, and also to prevent secondary infections[3][4][5] Primary cutis verticis gyrata is a benign cutaneous disorder. Still, because of the associated psychological and cosmetic concerns, surgical intervention remains the most definitive treatment option as of now[6][6]The type of surgical repair will depend on the size, location of the folds, and the expectation of the patient.[9][6]

Surgical modalities range from simple excisions to tissue expansion and skin grafts for patients with more extensive scalp involvement[1][2][6]. Treatment of larger folds can take place in a staged approach with the use of tissue expansion techniques and local flap reconstruction. Alternatively, the surgeon may choose for partial resection of the most abundant segment of the lesion.[2][1] Myocutaneous flaps or free flaps, such as the latissimus dorsi muscle have also been suggestions for complete resection of excess skin folds.[9][1][2] Smaller localized folds can be excised and closed primarily with care to avoid distortion of the brow, eyelid, and hairline depending on the location of the folds.[1][2] Studies have shown that partial resection with immediate closure is the safest and most effective procedure to correct CVG[1][2][3][4] Patients can safely undergo further resection after a one-year postoperative period.[1][3]

Differential Diagnosis

The appearance of CVG is quite distinctive, and making a clinical diagnosis is usually easy. The challenge is to diagnose underlying or associated conditions accurately. The physician must be aware of the common associations and corresponding differential diagnosis. Cutis verticis gyrata now falls under three categories: primary essential, primary nonessential, and secondary.[6][1] Primary essential CVG has no other associated abnormalities.[6][2] Primary nonessential CVG is associated with intellectual disability, neuropsychiatric disorders, seizures, schizophrenia, cerebral palsy, ophthalmologic abnormalities such as cataracts, strabismus, retinitis pigmentosa, and blindness.[2][6][1][3] The IQ of these patients is rarely over 35, and they may present with microcephaly, static encephalopathy, and convulsions.

Secondary cutis verticis gyrata has been reportedly associated with pachydermoperiostosis (idiopathic hypertrophic osteoarthropathy), cerebriform intradermal nevus, acromegaly, and other less frequent cause of CVG such as inflammatory diseases of the scalp, scleromyxedema, leukemia, lymphoma, syphilis, acanthosis nigricans, neurofibromas, nevus lipomatosus,  tuberous sclerosis, cylindroma, Ehlers-Danlos syndrome, amyloidosis, diabetes mellitus, cutis laxa, intracerebral aneurysm, cutaneous focal mucinosis and the misuse of anabolic steroids.[1][3][4][7][5] Chromosomal abnormalities such as Turner syndrome, Klinefelter syndrome, Noonan syndrome, Beare-Stevenson syndrome, and fragile X syndrome also have correlations with CVG.[4][6][1]

Prognosis

Cutis verticis gyrata may be disfiguring, but the process is essentially benign, and the prognosis is good[1]. No reports exist in the literature of malignant transformation of the skin.[3]

Complications

Cutis verticis gyrata is essentially a benign process; however, secondary causes associated with CVG and their complications require management.[1][4]

Deterrence and Patient Education

The patient should be aware of cutis verticis gyrata classifications and most importantly the associations relating to CVG. The benign nature must be made clear to the patient.

Enhancing Healthcare Team Outcomes

Cutis verticis gyrata is essentially a benign process. However, an interprofessional team that involves shared decision making is vital. The handling of primary CVG and its secondary associations involves a interprofessional approach to improve outcomes and decrease morbidity. The work-up should include dermatologists, pediatricians, internists, primary care providers, nurse practitioners, and geneticists to ensure consideration of all possible underlying conditions. For surgical correction, a combined approach involving dermatologists and plastic surgeons is often necessary. an interprofessional team approach, including the physician specialties cited along with nursing personnel, is a prudent approach to care in this condition, providing the best chance for an optimal outcome. Dermatology nurses and nurse practitioners participate in patient and family education, arranging follow-up, and coordinating information exchange between the team members. [Level 5]