LETTER TO THE EDITOR
|Year : 2023 | Volume
| Issue : 1 | Page : 88-90
Cutis Verticis Gyrata Heralding a Diagnosis of Primary Systemic Amyloidosis in a Patient with Cardioembolic Stroke
Ayush Agarwal1, Pinki Chatterpal1, Surya Kant2, Ajay Garg2, Sudheer Arava3, Achal K Srivastava3
1 Department of Neurology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neuroraimaging and Interventional Neuroradiology, All India Institute of Medical Sciences, New Delhi, India
3 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
|Date of Submission||26-Aug-2022|
|Date of Acceptance||18-Nov-2022|
|Date of Web Publication||04-Jan-2023|
Achal K Srivastava
Department of Neurology, AIIMS, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Agarwal A, Chatterpal P, Kant S, Garg A, Arava S, Srivastava AK. Cutis Verticis Gyrata Heralding a Diagnosis of Primary Systemic Amyloidosis in a Patient with Cardioembolic Stroke. Ann Indian Acad Neurol 2023;26:88-90
|How to cite this URL:|
Agarwal A, Chatterpal P, Kant S, Garg A, Arava S, Srivastava AK. Cutis Verticis Gyrata Heralding a Diagnosis of Primary Systemic Amyloidosis in a Patient with Cardioembolic Stroke. Ann Indian Acad Neurol [serial online] 2023 [cited 2023 Feb 1];26:88-90. Available from: https://www.annalsofian.org/text.asp?2023/26/1/88/367044
Cutis verticis gyrata (CVG) or “bulldog scalp” is a rare dermatological condition of the scalp characterized by convoluted vertical folds and deep furrows resembling the cerebral cortex. CVG can be either primary or secondary, with the latter associated with diseases like amyloidosis, myxedema, acromegaly, syphilis, leukaemia, lymphoma, tuberous sclerosis, pachydermoperiostosis, and Ehler Danlos syndrome. It usually occurs late into the disease course, and its presence heralding the primary diagnosis is rare. We present a middle-aged lady who presented with cardio-embolic stroke, where the presence of CVG was the initial manifestation and also led to the eventual diagnosis of systemic amyloidosis.
A 48-year-old lady, without previous medical comorbidities, presented to our emergency with acute left hemiparesis for 2 hours. Upon evaluation, her National Institute of Health Stroke Scale (NIHSS) score was 15 with an Alberta Stroke Program Early Computed Tomography Score (ASPECTS) of 6 [Figure 1]a. Her CT angiography suggested right distal M1 middle cerebral artery (MCA) occlusion [Figure 1]b. She was thrombolyzed with tenecteplase, and endovascular thrombectomy was offered but declined by the caregivers. Post-thrombolysis her NIHSS improved to 11, and anti-edema measures were instituted. Her 2D echocardiography revealed restrictive cardiomyopathy with bi-atrial enlargement and an ejection fraction of 30–35%. Continuous rhythm monitoring did not reveal any arrhythmias.
|Figure 1: Axial NCCT image (a) shows hypodensity involving right insula (arrow head) and right frontal operculum (arrow). Axial CT angiography base image (b) shows abrupt cut-off of superior division of right MCA (arrow) with paucity of distal branches in its distribution|
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Examination revealed a loss of hair with vertical grooves in her scalp (CVG) [Figure 2]a and [Figure 2]b, macroglossia, left upper motor neuron-type facial palsy, and left hemiplegia. These scalp changes had been present for the past 3–4 years and had been ignored as an age-related malady.
|Figure 2: (a and b) Front and lateral views of the scalp revealing alopecia and cutis verticis gyrata|
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Her routine blood investigations were within normal limits. The urine routine revealed the presence of 2+ proteinuria (24-hour urinary protein 233.5 mg/day), and chest X-ray showed bilateral pleural effusion. Pleural fluid (PF) analysis revealed no cells, PF albumin 1gm/dl (serum albumin: 4.3 gm/dl), and PF LDH 116 U/L (normal <166U/L). Serum electrophoresis with immunofixation showed a narrow M band of lambda light-chain origin. Thereafter, serum-free light chains were assayed and revealed a significantly decreased serum-free kappa-to-lambda light-chain ratio of 0.004 ([normal ratio: 0.260–1.650]; kappa: 20.6 mg/L [normal: 3.3–19.4] and lambda: 5075.92mg/L [normal: 5.71–26.30]). Viral markers for HIV, hepatitis B and C, thyroid profile, venereal disease research laboratory (VDRL) for syphilis, and anti-nuclear antibody tests were negative, and ultrasound abdomen and pelvis were normal. In lieu of her age, scalp (CVG), renal, cardiac and hematological findings, systemic amyloidosis was suspected and scalp and tongue biopsy were done. The biopsy was suggestive of diffuse, nodular, extracellular, acellular hyalinized material in the dermis which showed organophilia with Congo red staining and apple green birefringence under polarized light [Figure 3]. Her tongue biopsy also revealed similar findings confirming the diagnosis. She was started on apixaban for secondary stroke prevention and bortezomib, cyclophosphamide, and dexamethasone (VCD regimen) from hematology and is improving. Her modified Rankin scale score was 2 at 3-month follow-up, without any further events.
|Figure 3: Skin biopsy shows: (a) Unremarkable epidermis and the lower dermis show dense accumulation of eosinophilic extracellular hyalized material [Arrow]. (b) The substance is deposited around the adnexal structures. (c) Congo red stain reveals congophilic substance. Immunohistochemical stain is (d) negative for serum amyloid protein [SAA], (e) mild positivity for kappa light chain [star], and (f) strong positivity for lambda chain deposition [star]|
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CVG was coined by Unna in 1907, and its common parlance is the “bull dog scalp.” Primary CVG is more common in males in the third decade, whereas secondary CVG is commoner than the primary form and has no age or gender predilection (prevalence: 0.026–1 in 100,000 individuals)., The thickened scalp folds are usually symmetrical and run antero-posteriorly, involving the entire scalp. However, secondary SVG can rarely be asymmetrical or involve the forehead. Cutis verticis gyrata is disfiguring and leads to cosmetic problems, but primary CVG is essentially benign, with a good prognosis. There have been no published reports of its malignant transformation. Secondary CVG prognosis depends on its cause and usually responds to the management of the primary disorder.
Dermatological involvement in systemic amyloidosis is seen in approximately 50% of cases, the manifestations depending on the site of involvement. Deposition around pilosebaceous glands of the scalp leads to alopecia and rarely enlargement of skin, which may get thrown into deep furrows leading to secondary CVG patterns. Only a single case report in literature has previously described this finding in primary systemic amyloidosis.
Our patient's scalp manifestations preceded her stroke by 3–4 years. Had medical attention been sought, it might have led to an earlier diagnosis and stroke prevention. We seek to highlight this uncommon finding of CVG and its even rarer association with primary systemic amyloidosis.
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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]