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Spontaneous improvement of cutis verticis gyrata secondary to acromegaly following surgical treatment of pituitary adenoma

Cutis verticis gyrata (CVG) is a term fi rst coined in 1907 to describe a thickening of the scalp that causes the scalp to resemble the cerebral gyri due to the formation of ridges and furrows [ 1 ] . Cutis verticis gyrata is classifi ed into primary (idiopathic) and secondary forms; the latter are caused by tumors, nevi, chronic infl ammatory disorders of the scalp or endocrinopathies such as acromegaly, thyroid myxedema, and autosomal dominant insulin resistance syndromes [ 2, 3 ] . A 36-year-old male with no clinically relevant personal or family history presented to the dermatology department with progressive thickening of the scalp during the previous four years. Examination revealed folds and furrows in the parietal area and especially in the occipital area (Figure 1 a). The folds were 5 to 7 mm wide and the furrows measured 2 to 5 mm in depth. Prognathism, macroglossia, nose enlargement, dental malocclusion and frontal bone prominence were not found. When the patient was asked directly about morphological changes in his face, hands or feet he said that his appearance was now rougher. Skin biopsy ruled out the presence of infl ammatory or neoplastic skin diseases (Figure 2 ). Ultrasound fi ndings suggested epidermal and dermal enlargement, causing these structures to have almost three times the normal thickness for his age and gender. Blood levels of Insulin-like growth factor 1 (IGF-1; 1,431 ng/ml) and basal growth hormone (GH; 4.09 ng/ml) were three times the upper limit of normal (IGF-1 360 ng/ml; GH 2.0 ng/ml). The patient was referred for assessment to the endocrinology department, where acromegaly due to a 1-cm pituitary adenoma was diagnosed. No pituitary hormone defi ciencies were detected, and there were no signs of damage to the optic chiasm or cavernous sinus. After the diagnosis of CVG secondary to acromegaly was established, a trans-sphenoidal resection of the pituitary adenoma was performed by a neurosurgeon who specializes in such procedures. Histopathology showed that the pituitary adenoma had two different cell populations, with positive immunohistochemistry for GH and prolactin and a Ki67 proliferation index of 1 %. Three months after treatment, the patient’s facial appearance was less rough with partial resolution of the CVG (Figure 1 b). At six months after diagnosis, biochemical and radiological tests showed that the acromegaly had been completely cured. At a four-year follow-up, there was no indication of recurrence and the CVG had resolved completely.