Excess Growth hormone leads to rare case of Cutis Verticis Gyrata

Dr Matteo Parolin and Dr Francesca Dassie at Università degli Studi di Padova, Padua, Italy have reported a rare case of Cutis Verticis Gyrata associated with excessive growth hormone and Acromegaly. The case has appeared in the New England Journal of Medicine.

Acromegaly is a hormonal disorder that results from too much growth hormone (GH) in the body. The pituitary, a small gland in the brain, makes Growth Hormone. In acromegaly, the pituitary produces excessive amounts of Growth Hormone.Usually the excess Growth Hormone comes from benign, or noncancerous, tumors on the pituitary which are called adenomas.

Cutis verticis gyrata (CVG) is a descriptive term for a condition of the scalp manifesting as convoluted folds and furrows formed from thickened skin of the scalp resembling cerebriform pattern. The morphologic changes in the scalp which characterize cutis verticis gyrata were first described by Robert in 1848 and by McDowell and Cowan in 1893 in microcephalic idiots. It was not until 1906 that Jadassohn called to the attention of dermatologists the condition which in the following year was given its present name of cutis verticis gyrata by Unna.Cutis verticis gyrata is long lasting and progressive. It is often found to be unacceptable because of cosmetic reasons.

A 37-year-old man presented to the endocrinology clinic with a 4-year history of excessive sweating, headaches, and joint pain. His wife had also noticed increasing skin folds on his scalp. Physical examination showed thickening of the skin on his scalp with ridges and furrows (Panel A, back of head, and Panel B, top of head). He had enlarged feet and hands and a protruding lower jaw.

Findings from laboratory evaluation were notable for an insulin-like growth factor I level of 907 μg per liter (reference range, 82 to 237) and a random measurement of the growth hormone level of 7.3 μg per liter (reference range, 0 to 0.8). A 75-g oral glucose load did not suppress the growth hormone level and confirmed a diagnosis of acromegaly. Magnetic resonance imaging of the head revealed a pituitary adenoma measuring 27 mm by 22 mm by 25 mm.

The thickening and furrowing of skin on the scalp, called cutis verticis gyrata, can occur as an isolated finding or may be related to a number of conditions, such as acromegaly, as in this case. The patient underwent transsphenoidal resection of the pituitary adenoma. He had residual tumor and was treated with a somatostatin analogue and a growth hormone receptor antagonist. He received injections of soft-tissue fillers in an attempt to create a smoother appearance of the scalp, but the injections had an only partial effect.

The patients with cutis verticis gyrata need to be educated that proper hygiene of the scalp is essential to avoid the accumulation of secretion in the furrows. Lack of hygiene may be the cause of an unpleasant smell and secondary infection.

Improvement of cutis verticis gyrata with topical treatment of causal dermatitis has been reported. Primary essential cutis verticis gyrata is a cosmetic problem, but psychological repercussions are important.Hygiene for folds and furrows is very important. In some patients, using medicated shampoos may be beneficial.