A 45-year-old patient presents for a routine health maintenance evaluation. You note furrowing in his scalp. He states that he is unaware of these changes. The patient has no other medical problems.
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Cutis verticis gyrata (CVG) presents as deep linear skin furrows in the scalp. The face and/or neck are rarely affected. As the furrows become more prominent, they begin to resemble cerebral sulci and gyri. There may be alopecia noted on the scalp with sparing of the furrows. On physical exam, the folds are soft and spongy. The furrows cannot be molded with pressure.
CVG is classified as primary or secondary. Primary CVG is further subdivided into nonessential and essential. Primary nonessential CVG is associated with intellectual disability, cerebral palsy, epilepsy, ophthalmologic abnormalities (ie cataracts, blindness), and/or deafness. Primary essential CVG is not associated with any other abnormalities.
Secondary CVG is caused by various disorders such as acromegaly, myxedema and pachydermoperiostosis, and is due to the underlying processes these disorders.
The pathophysiology of CVG is largely unknown. Primary nonessential CVG is thought to be due to genetic and endocrine abnormalities. Primary essential CVG is only due to genetic alterations. There is not yet an agreement on the mode of inheritance. Sporadic, autosomal dominant, and autosomal recessive inheritances have been postulated. Due to the increased prevalence in men, researchers have considered an X-linked inheritance.
Histopathologically, CVG is often normal appearing. If changes are present, they usually include hypertrophy of pilosebaceous structures, and the presence of multiple sweat glands and ducts.
Treatment is often not needed. Surgery is an option for aesthetic reasons. Regression without surgery is rare.
Aplasia cutis congenital is a birth defect that presents with a small, well-circumscribed absence of the skin of the scalp in newborns. The area is made up of a thin, hairless membrane, which is the result of healing that occurs in-utero. Rarely, the defect includes the cranium.
The lesions are often well circumscribed, small and stable. Larger lesions should be given time to heal. Surgery is rarely needed but may be performed later. Grafting is an option for larger lesions.
Cutis rhomboidalis nuchae is a pattern of photoaging seen at the nape of the neck that is particularly prevalent in older men who work outside. It is characterized by furrowing of skin that leads to skin creases that are rhomboid in shape.
Erythema gyratum repens is a paraneoplastic lesion that often presents as concentric rings of erythema and scaling and expands out further and further each day. It is associated with various carcinomas, and may also be associated with connective tissue diseases.
Abiara Vanessa Agwu is a medical student at the University of Texas Medical School at Houston.
Adam Rees, MD, is a graduate of the University of California Los Angeles School of Medicine and a resident in the Department of Dermatology at Baylor College of Medicine also in Houston.
- Freedberg IM et al. Fitzpatrick’s Dermatology In General Medicine. 6th ed. New York: McGraw-Hill; 2003. Print.
- Walia R, Bhansali A. “Cutis Verticis Gyrata.” BMJ Case Reports. 2011;doi:10.1136/bcr.01.2011.3763.
- White GM, Cox NH. Diseases of the Skin: A Color Atlas and Text. London: Hancourt Publishers, 2000. Print.