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Durairaj Arjunan1, Liza Das2, Sanjay K Bhadada1, Manjul Tripathi3, Tarun Narang4, Pinaki Dutta1
1 From the Department of Endocrinology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
2 From the Department of Endocrinology, Post Graduate Institute of Medical Education and Research (PGIMER); Department of Telemedicine, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
3 Department of Neurosurgery, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
4 Department of Dermatology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
Correspondence Address:
Pinaki Dutta
From the Department of Endocrinology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh
India
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/ijd.ijd_691_23
How to cite this article:Sir,
Cutis vertices gyrata (CVG) is a rare cutaneous manifestation characterised by deep furrows and convoluted folds on the scalp that resemble the sulci and gyri in the cerebral cortex. It is a reasonably well-known association with acromegaly; however, CVG as a presenting manifestation of acromegaly is very rare. Herein, we present two cases of acromegaly with CVG as a presenting manifestation.
Case 1: A 41-year-old gentleman presented to a dermatologist with complaints of unusual skin folds over the scalp for the past 3 years. He was then referred to endocrinology in view of acral enlargement, ring size change, excessive sweating and arthralgia. On examination, the patient had coarsening of the face [Figure 1]a, acral enlargement [Figure 1]b and [Figure 1]c, and dental malocclusion. Skin examination revealed skin tags, acanthosis nigricans, and CVG [Figure 2]a, [Figure 2]b, [Figure 2]c. Laboratory evaluation revealed a plasma Insulin-like Growth Factor 1 (IGF-1) level of 1385 ng/mL (reference range: 94–242 ng/mL) with non-suppressible Growth Hormone (GH)after glucose load (nadir GH: 13.1 ng/mL, normal <1 ng/mL). He also had impaired fasting glucose (111.1 mg/dL, normal <100 mg/dL) and secondary hypogonadism, and all other pituitary hormones were within normal limits. Contrast-enhanced MRI (CEMRI) sella revealed a macroadenoma of size 24 × 22 × 25 mm. CVG was also appreciable on the MRI [Figure 2]d, [Figure 2]e, [Figure 2]f. He successfully underwent transsphenoidal resection of the microadenoma and, on follow-up after 6 months, reported subjective improvement in the severity of CVG. Whole-exome sequencing was performed, which did not reveal any pathogenic mutations, including FGFR2 mutation.
Figure 1: (a) Image showing coarse facial features and skin folds over the forehead, glabella, and cheeks. (b and c) Image showing acral enlargement
Figure 2: (a-c) Image showing multiple cutis vertices gyrata involving the entire scalp region (d-f) MRI showing macroadenoma (arrow) and the CVG (arrowheads)Case 2: A 55-year-old gentleman presented to dermatology with complaints of skin folds over the scalp for the past 3 years, and he was referred to an endocrinology clinic in view of acral enlargement, excessive sweating, and seborrhoea. Hormonal investigations revealed an elevated IGF-1 level with non-suppressible GH after 75-g oral anhydrous glucose. CEMRI revealed pituitary microadenoma (9 × 9 × 9 mm). He underwent trans-sphenoidal resection of the adenoma.
Both patients initially visited dermatology clinics and were later referred to endocrinology, considering the possibility of acromegaly-related CVG. The typical patterns of skin involvement in acromegaly include acanthosis nigricans, skin tags (acrochordons), hyperhidrosis, hypertrichosis or hirsutism in sporadic or syndromic cases, and lipomas, collagenomas, angiofibromas, lentigines, cutaneous myxomas, café-au-lait macules in syndromic cases.
Cutis vertices gyrata (CVG) is one of the least common cutaneous manifestations of acromegaly, with prevalence ranging from 10% to 15% of patients.[1] It is classified as primary essential CVG, primary non-essential CVG, and secondary CVG. Although primary essential CVG is not associated with any other abnormalities, primary non-essential CVG is associated with neuropsychiatric with or without ophthalmologic abnormalities referred to as CVG–intellectual disability syndrome. Mutations in the fibroblast growth factor receptor 2 have been reported in primary CVG.[2] Secondary CVG occurs in relation to underlying disease processes such as acromegaly, pachydermoperiostosis, and cerebriform intradermal nevus[3]. The skin folds in primary CVG are symmetrical and run in an anteroposterior direction in contrast to secondary CVG, which is often asymmetrical.[4]
The pathogenesis of CVG in acromegaly is attributed to the elevated GH, which acts directly or indirectly via IGF-1 acting on the dermal fibroblasts or indirectly via increasing the deposition of glycosaminoglycans (GAGs)[5] Histopathology in CVG secondary to acromegaly reveals GAG deposition with dilated lymphatic vessels and prominent fibroblasts.
The mainstay of treatment of CVG in acromegaly is tumour resection, causing reduced IGF-1, which may cause partial or complete regression of CVG. Hyaluronidase injections have also been tried to treat this condition.[6] Surgical options include simple excision to complete resection of skin folds with myocutaneous flaps or free flaps.[7]
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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