Cutis verticis gyrata - A rare presentation of Primary systemic Amyloidosis.

Sir,

A 35-year-old female presented in the Department of Dermatology with asymptomatic skin-colored swellings and loss of hair over the entire scalp since 3–4 years. On clinical examination, multiple, skin colored, waxy cerebriform nodules and plaques that were coalescing in nature and diffusely involve the whole scalp with alopecia. Similar lesions were present in periorbital region and nipple-areola complex [Figure 1]. Macroglossia and periorbital purpura were also present. Nails and mucosae were normal. Based on history and clinical examination, clinical diagnosis of secondary cutis verticis gyrata (CVG) was made. Macroglossia and periorbital waxy skin-colored nodules with purpura pointed toward amyloidosis. Further, the patient was evaluated thoroughly to rule out other causes of secondary CVG. On investigation, her complete blood count showed low hemoglobin and rest was normal. Renal functions test was also deranged with raised blood urea and serum creatinine. Electrocardiography, chest X-ray, X-ray skull, thyroid function tests, venereal disease research laboratory, and ultrasound abdomen were normal. Magnetic resonance imaging head showed diffuse irregular nodular thickening of scalp layers without any intracranial lesions and bony abnormalities. On histopathological examination, accumulation of homogeneous eosinophilic material in the dermis was seen in Figure 2. Congo red stain was positive and apple-green birefringence was seen on polarizing microscopy. Thus, features were suggestive of amyloidosis. Bone marrow examination, serum and urine electrophoresis were found to be normal. The patient was diagnosed as a case primary systemic amyloidosis (PSA) and was referred to medicine for further management.

Figure 1

(a) Scalp skin thrown into longitudinal folds forming cutis verticis gyrata; (b) cerebriform nodules on scalp with alopecia; (c) photograph showing the left and right side of nipple-areola complex with multiple nodules; (d) red arrow showing periorbital purpura and black arrow showing macroglossia

Figure 2

(a) Photomicrograph showing eosinophilic amorphous material in dermis (H and E, ×10); (b) Section showing staining with Congo red showing brick-red deposits in dermis

CVG is a rare congenital or acquired condition of scalp, characterized by convoluted folds and deep furrows that resemble the surface of cerebral cortex. It may be primary or secondary. Secondary CVG are associated with other diseases such as acromegaly, myxedema, hamartomatous lesions, tumors, idiopathic hypertrophic osteoarthropathy, syphilis, leukemia, cretinism, tuberous sclerosis, neurofibromatosis, acanthosis nigricans, Ehlers–Danlos syndrome, and trauma. Primary forms are those which are not associated with any underlying cause.[1]

Amyloidosis is one of the causes of secondary CVG. Amyloidosis can be classified as localized or systemic. Localized amyloidosis is the deposition of amyloid in only one organ. Systemic amyloidosis can be primary or secondary. PSA can be idiopathic or myeloma associated. Secondary systemic amyloidosis is associated with chronic inflammatory disorders.[2] Ours is a case of PSA as it was not associated with any chronic inflammatory disease.

Systemic amyloidosis shows cutaneous involvement is about 50% of patients.[3] The manifestations of cutaneous amyloidosis depend on the site of amyloid deposition. Deposition around pilosebaceous glands leads to the destruction of hair and alopecia. Amyloid deposition in scalp skin results in enlargement of skin which gets thrown into convoluted folds and furrows resembling CVG.[2] CVG may be a rare presenting feature for PSA as in our case. Thus, dermatologist facing this condition must have high index of suspicion for the diagnosis of such cases, as primary systemic amyloidosis can seldom present as cutis verticis gyrata at the initial stages.

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