Cutis verticis gyrata is a disease of the scalp characterized by hypertrophy of the dermis with the formation of folds and furrows. The skin of the CVG is sharply thickened and compacted, visually resembles the convolutions of the brain or the scalp of a bulldog. Cracks, lichenization, pigmentation may occur in the area of altered skin. The diagnosis is made according to clinical data, confirmed by histological examination of skin biopsies. For the treatment of pathology, pharmacotherapy of the underlying disease is carried out, surgical techniques are proposed (needle fasciotomy, scalp tightening).
L91.9 Hypertrophic skin change, unspecified
Cutis verticis gyrata (CVG) is a progressive skin disease that occurs with the formation of a folded deformation of the scalp. The pathology was first described in 1837 by the French dermatologist J. Aliberom called “furrowed skin”. The disease is also known as Audrey’s syndrome, bulldog head syndrome. The modern name – cutis verticis gyrata – belongs to the German dermatologist P. Unna (1907). Idiopathic cutis verticis gyrata affects men 5 times more often (1:100,000) than women (0.026:100,000).
Cutis verticis gyrata in modern dermatology is attributed to polyethological pathologies caused by genetic factors acquired by common and dermatological diseases. The main groups of causes include:
- Genetic disorders. Mutations of the FGFR2 gene that cause hypertrophy of the dermis are called as possible etiofactors of PCG. Given the higher incidence rate among men, X-linked inheritance is not excluded. Pachyderma correlates with the syndromes of Klippel-Trenone, Cornelia de Lange, Turner, Noonan, Martin-Bell, Kleinfelter, Ehlers-Danlos, pachydermoperiostosis, etc.
- Chronic diseases. Endocrinopathies (acromegaly, diabetes mellitus, myxedema), systemic (amyloidosis), oncological (leukemia, lymphoma, paraneoplastic syndrome), neurological pathologies (tuberous sclerosis) can contribute to secondary changes in the skin. Connective tissue hyperplasia can be initiated by the abuse of anabolic steroid hormones.
- Dermatoses. Local trophic disorders with subsequent hyperplasia of the dermis can be caused by prolonged and recurrent infectious processes: cutaneous tuberculosis, erysipelas, dermatomycosis, leprosy, syphilis, conglobate acne. Folded thickening of the skin is accompanied by cerebriform nevus of the sebaceous glands, lipomatous nevus, cylindroma, scleromyxedema, psoriasis of the scalp.
- Local violations of lymph flow. In a number of patients, cutis verticis gyrata is provoked by local disorders of lymph circulation due to trophedema, lymphostasis, lymphadenectomy, injuries of the integumentary tissues of the head and face.
To date, three theories are considered as the main pathogenesis of cutis verticis gyrata: genetic, lymphopathological and endocrine. The first suggests that hyperplasia of all layers of the dermis is caused by mutations of the protein-fibroblast growth factor receptor-2. The second believes that PCG is caused by congenital /acquired lymphangiectasias, leading to stagnation of lymph and excessive folding of the skin. The third connects the predominance of men and the post-pubertal onset of the disease with androgen regulation.
During the pathomorphological examination of the skin from the foci of cutis verticis gyrata, a typical histological triad is determined, characteristic already for the early stages of the disease: edema of the dermis, accumulation of mucin, degenerative changes (splitting, fragmentation) of elastic fibers. With a severe neglected form of PCG, hyperplasia of the sebaceous glands and hair follicles, hypertrophy of muscle fibers, fibrosis of connective tissue is detected. Ectasia and thrombosis of the blood and lymphatic vessels of the dermis, pronounced hyperkeratosis and acanthosis are detected.
Taking into account ethological factors , primary and secondary cutis verticis gyrata are distinguished:
1. Primary. It is genetically determined. It has two varieties:
- idiopathic ‒ develops at the post-puberty age (up to 30 years) as an isolated pathology, it is more common in men;
- symptomatic – occurs in a number of congenital neuropsychiatric, ophthalmological, chromosomal diseases.
2. Secondary. Etiologically associated with diseases of internal organs, injuries and infectious lesions of the scalp. Has no gender predisposition.
Clinical signs of the primary variant of cutis verticis gyrata become noticeable during puberty or shortly after it, secondary forms can manifest at any age. In its development, the pathological process goes through several stages.
At the initial (soft) stage, transient swelling of the head tissues appears, the skin acquires a dough-like consistency. Its surface is smooth, shiny, pink with a slight cyanotic tinge. At the next stage – intermediate – edema persists constantly, the skin thickens, its color pales. The final stage (compaction) is characterized by loss of elasticity of the dermis, its growth and thickening. Hyperkeratosis develops in areas of cutis verticis gyrata, skin cracks form, pigmented spots appear.
Skin changes more often affect the parietal-occipital zone, less often the face. The relief of the scalp resembles the surface of the cerebral cortex. The folds (ridges) of the skin are 1-2 cm high, 2-4 cm wide, separated by furrows-depressions. The direction of the folds is usually longitudinal (from the crown to the back of the head), in the occipital region the folds may have a transverse arrangement. With primary CVG, the ridges and furrows run parallel to each other, with secondary, as a rule, they are asymmetric.
Primary symptomatic cutis verticis gyrata is often associated with neurological (oligophrenia, microcephaly, cerebral palsy, epilepsy), psychiatric (schizophrenia), ophthalmological pathologies (cataract, strabismus, retinitis pigmentosa, blindness).
The altered relief of the scalp in the form of ridges and furrows represents a noticeable aesthetic defect, gives the patient psychological experiences, creates difficulties during hygienic procedures and in choosing a hairstyle. Patients with cutis verticis gyrata often complain of itching, painful cracks, wetness of the skin in the interstitial space, an unpleasant smell. Recurrent infections (folliculitis, impetigo), seborrhea easily develop in areas of the altered scalp, papillomas form.
The diagnosis of cutis verticis gyrata is usually made by a dermatologist based on the clinical picture. The scalp is dense and elastic to the touch, when trying to stretch the folds do not lend themselves to straightening. In the area of the skin ridges, the hair is sparse, however, between the folds the hair rods are thickened, growing in the form of thick bundles. The method of confirming diagnosis is a dermal biopsy with histological examination of samples.
To search for concomitant diseases that can provoke hypertrophy of the scalp, an instrumental laboratory examination is performed:
- blood tests: glucose, ELISA (IgE total, antiTP IgG), hormonal studies (thyroid panel, STH, testosterone), cancer markers (REA, alpha-fetoprotein);
- CT scan of the skull to exclude intracranial lesions;
- MRI of the soft tissues of the head before planning surgical treatment;
- Laboratory genodiagnostics with the advice of a geneticist.
With characteristic changes in the skin of the face and head, it is necessary to exclude diseases that can give a similar clinical picture:
- Hoffmann ‘s perifolliculitis;
- limited scleroderma;
- keloid scars;
- nodular dermal allergid Gouzhero;
- flabby skin syndrome (elastolysis).
A single treatment regimen for CVG has not been developed. Therapeutic measures depend on the patient’s complaints and wishes. It is important to observe local hygiene of the scalp to avoid accumulation of secretions, maceration, unpleasant odor, secondary infections. With the development of infectious processes, a course of antibiotic therapy is carried out, retinoic acid preparations are prescribed, therapeutic shampoos with zinc pyrithione are recommended for scalp care.
In secondary pachyderma, the main attention is paid to the therapy of the causal disease. Topical GCS are used for the treatment of dermatitis, vasodilators, antihypoxants, microcirculation correctors are used to improve the trophic tissues. Physiotherapy is carried out: electrophoresis and ultraphonophoresis with lidase, laser therapy.
Plastic surgery is performed in order to improve the appearance. However, they do not prevent the progression of cutis verticis gyrata. The following techniques are used for aesthetic correction:
- Percutaneous needle fasciotomy. Allows you to loosen the tension of the skin folds by applying deep incisions with injection needles.
- Lifting of the scalp. It involves excision of excess dermis forming folds, followed by skin lifting in the longitudinal and transverse direction. With extensive resections, the subsequent closure of the defect may require the installation of tissue expanders, transplantation of free skin flaps.
Prognosis and prevention
Primary cutis verticis gyrata is a progressive disease that cannot be completely cured. With the help of medical procedures and proper care, it is only possible to improve the appearance, eliminate unpleasant symptoms and complications. There were no cases of malignant transformation of the skin.
The prognosis of the secondary form of cutis verticis gyrata is associated with the course of the underlying disease. Prevention is also associated with the exclusion of possible causes (injuries, infectious dermatitis of the scalp, the use of steroids), the prevention of systemic diseases.