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Journal of Postgraduate Medicine, Vol. 48, Issue 1, 2002 pp. 41 & 45 Spot the Diagnosis Thami GP, Kaur S Department of Dermatology and Venereology, Government Medical College and Hospital, Sector-32 B, Chandigarh, India Code Number: jp02017 A 65-year-old male had a six-month history of alopecia and thickening of the scalp. He had no history of seizures or any significant past medical history. General physical examination was normal with proportionate length of trunk and limbs. There was no lymphadenopathy or organomegaly and his systemic examination was unremarkable. Scalp revealed bilaterally symmetrical longitudinal gyrate folds of skin involving the entire scalp with loss of scalp hair (Fig 1a and 1b). There was no erythema, papules, pustules or scarring of scalp. A detailed cutaneous examination revealed no café-au-lait spots, ash leaf macules, axillary freckling or neurofibromas. The examination of nails, mucosae and eyes was normal. Investigations revealed normal hemogram, serum biochemistry, urine analysisand a chest radiograph. Psychiatric evaluation revealed an average intelligence (PQ 89, VQ 93) without any other abnormality. A skin biopsy showed hyperkeratosis, acanthosis and a mild lymphomononuclear cell infiltrate with hypertrophy of sebaceous glands. What is your diagnosis for this condition? Diagnosis: Primary Cutis verticis gyrata The term cutis verticis gyrata (CVG) describes the hypertrophy and folding of skin of scalp to present a gyrate or a cerebriform appearance.1 It may be primary (idiopathic) or secondary to various disorders that cause hypertrophy of soft tissues of the scalp.1 The essential abnormality is overgrowth of the scalp in relation the underlying skull. Histopathology reveals hypertrophy of sebaceous glands without increase in the collagen.1 Primary CVG usually affects males and is sporadic in nature although genetically determined (autosomal) cases have also been described.1,2 In one large series of 275 cases of CVG, 48% were primary or idiopathic.2 It has been observed in 0.5 % of mentally retarded individuals in Sweden, Scotland and USA.1,3 The scalp folds develop in late childhood or at puberty and slowly become more accentuated. Cerebral palsy and epilepsy may be also be associated.1,3 CVG may also develop at a later age in patients with Lennox-Gastart syndrome, characterized by retardation and slow irregular space and wave complexes on electroencephalograph.1 The secondary CVG may be observed with tuberous sclerosis, plexiform neurofibromatosis, Darier's disease or internal malignancy.1,4 Congenital melanocytic nevi of scalp with cerebriform appearance may also simulate CVG.1 Patients with endocrine disorders like acromegaly and rarely myxoedema and cretinism may also develop CVG.1 Other acquired causes include prolonged traction to scalp hair.1,5 An important differential diagnosis is pachydermoperi-ostosis1 in which there is thickening of skin of face, hands, feet and long bones of limbs.1 Recent onset CVG in old age, in the absence of predisposing endocrine, tractional and inflammatory factors must lead to diligent search for possible internal malignancy.
Thami GP, Kaur S Department of Dermatology and Venereology,
References
This article is also available in full-text from http://www.jpgmonline.com/ © Copyright 2002 - Journal of Postgraduate Medicine The following images related to this document are available:Photo images[jp02017f1.jpg] [jp02017f2.jpg] |
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