Acromegaly-related cutis verticis gyrata

Abstract

Objective

Acromegaly is a disorder caused by hypersecretion of growth hormone (GH), resulting in excessive levels of insulin-like growth factor 1 (IGF-1), and almost always due to a pituitary tumor. It is classically associated with acral enlargement, prominent facial features and soft tissue overgrowth. Skin manifestations include hirsutism, acne, skin tags, oily skin and acanthosis nigricans. However, other uncommon dermatological features, such as cutis verticis gyrata (CVG), may also occur. Here, we review acromegaly-related CVG aiming to raise awareness for its possible occurrence in this setting, and we discuss its pathophysiology, presentation, management and differential diagnosis.

Design

A comprehensive literature search regarding CVG, particularly CVG related to acromegaly, has been carried out. Case reports, original studies and review papers, were considered.

Results

CVG is a rare benign skin lesion characterized by thickened and folded scalp, resembling the brain gyri and sulci. The diagnosis of CVG mainly relies on clinical examination, although tissue biopsy may be necessary in case of uncertain etiology. In acromegaly, CVG appears to be driven by the trophic effects of GH and IGF-1 on skin and soft tissues. While CVG is uncommon in acromegaly, it seems to occur more frequently in male patients. The management of acromegaly-related CVG essentially relies on controlling the serum levels of GH and IGF-1. Surgical skin procedures should be reserved for patients with severe aesthetic distress, after achieving the best possible control of acromegaly.

Conclusions

CVG is a rare manifestation of acromegaly that may allow an earlier diagnosis and a swifter treatment of these patients, which in turn may improve or entirely reverse such remarkable skin lesions.