A case of cutis verticis gyrata associated with epilepsy.
Abstract Cutis vertices gyrata (CVG) is characterized by thickening of the scalp which becomes raised to form ridges and furrows resembling cerebral gyri that cannot be flattened by traction or pressure. The etiology is diverse since different collections of cell types may be responsible for outward convoluted appearance. Herein a case of CVG associated with epilepsy is reported in a young girl in view of the clinical rarity of this association.
Key wordsCutis vertices gyrata epilepsy scalp cerebral gyri.
Cutis verticis gyrata (CVG) is a rare and slowly progressive deformity of the scalp with thick gyrated skin folds and ridges resembling gyri of the brain cortex. Those folds can lead to local skin infections or a social and cosmetic complaint. CVG can be classified into two forms: primary (essential and non-essential) and secondary. The anatomy of the scalp and its attachment are such that folds and gyri are produced when cells infiltrate beneath the epidermis. The etiology is diverse since different collections of cell types may be responsible for outward convoluted appearance and range from inflammatory or hamartomatous infiltrations to neoplastic proliferations.1
A 12-year-old girl presented with a history of deep furrows and convolutions over scalp. These scalp corrugation had been there since birth and were progressively increasing in size. There was no history of any other skin mucosal soft tissue or bone abnormality. She had no history of any inflammatory condition of skin or scalp. There was a history of epileptic seizures since childhood. Apart from seizures her medical history was insignificant. She had no personal history of mental retardation cerebral palsy psychiatric disorder or ocular disorder. There was no family history of consanguinity similar scalp condition or any bony deformity.
Cutaneous examination revealed multiple furrows and folds of 0.5-0.7 cm in depth and 4-5 cm in length arranged anteroposteriorly along the scalp (Figure 1). There was no palmer hyperhidrosis coarsening of facial features or clubbing. Systemic examination including ophthalmological examination was normal. Psychometry suggested a normal mental age and IQ. Routine hematological biochemical and radiological investigations including MRI brain were normal. EEG showed generalized paroxysmal epileptiform activity. Scalp biopsy was not done because her parents were unwilling for the same. Based on these clinical findings a diagnosis of cutis verticis gyrata associated with epilepsy was entertained. The patient was referred to a neurologist for antiseizure medication and advised plastic surgery for her scalp.
Cutis verticis gyrata is a descriptive term for a condition of the scalp in which deep furrows and convolutions are seen that resembles the outer surface of the cerebrum. Though cutis verticis gyrata was coined by Unna in 1907 for cerebriform appearance of the skin Fisher (1922) provided etiopathological details of the disorder. In general there are two main variables of this disease: primary and secondary. Primary type is further divided into essential and non- essential forms. The former may exist as a solitary finding with no association with neurological and ophthalmological disease and the latter may be associated with mental retardation epilepsy and other brain or ophthalmologic abnormalities.23 In primary CVG onset is after puberty with a normal histology or revealing increased connective tissue or epidermal appendages. Secondary CVG may appear at any age
skin folds are usually asymmetric and histology reveals various alterations depending on underlying causes such as tumors neurofibromas cerebriform intradermal nevi and inflammatory conditions. Systemic disorders associated with secondary CVG include acromegaly myxedema or amyloidosis.4
The underlying pathology responsible for thickening of the scalp lies in abnormal proliferation of collagen and reticular fibres deposition of mucopolysaccharides in corium and epithelial hyperplasia.
CVG can also occur as a part of pachydermoperiostosis.5 This syndrome includes clubbing of the digits periosteal new bone formation coarsening of facial features oiliness and furrowing of the skin of face as well as that of scalp seborrheic hyperplasia and hyperhidrosis of hands and feet.
A distinct Lennox-Gastaut syndrome has been described to show a symmetrical spike wave discharges at less than 3Hz even in sleep.6 Rotational traction over scalp hair can produce CVG in normal person.7
Treatment of CVG depends upon the etiology.8 Scalp reductive procedures have been performed for cosmetic reasons.
1. Diven DG Tanus T Raimer SS. Cutis verticis gyrata. Int J Dermatol. 1991;31:710- 2.
2. Dayna GD Tonny T Sharon SR. Cutis verticis gyrata. Int J Dermatol. 1991;30:710- 2.
3. Leticia KS Tantiane G Lauro AC et al. Essential primary cutis verticis gyrata. J Pediatr. 2002;78:75-80.
4. Chang GY. Cutis verticis gyrata underrecognized neurocutaneous syndrome. Neurology. 1996;47:573-5.
5. Rimon DL. Pachydermoperiostosis (idiopathic clubbing and periostosis) (and genetic and physiologic considerations). N Eng J Med. 1965;272:923-31.
6. Paulson GW. Cutis verticis gyrata and the Lennox syndrome. Develop Med Child Neurol. 1974;16:196-200.
7. Khare AK Singh G. Acquired cutis verticis gyrata due to rotational traction. Br J Dermatol. 1984;110:125-6.8. Abu-Jamra F Dimick DF. Cutis verticis gyrata: A report of a case secondary to acromegaly treated with plastic surgery and X-ray therapy. Am J Surg. 1966;111:274-7.
PhotoDermDiagnosis A 12-year-old girl with scarring alopecia
By: Zahra Rahnama Fahameh Fadaei Siavash Mohammadzadeh Shanehsaz and Anwar Dandashli
_: A 12-year-old girl was referred to the dermatology clinic of Aleppo University Hospital Syria with the chief complaint of alopecia centred on the crown. Alopecia started on the apex a year ago and gradually there was obvious decrease in the density of hair which expanded in a centrifugal fashion (Figure 1). Physical examination of involved area of scalp revealed smooth and shiny surface with massive follicular dropout and a few short brittle hair detectable within the scarred expanse. The patient had no other complaint such as dysesthesia or burning sensation on affected area. There was no evidence of systemic diseases and the patient was not taking any medicine.
Central centrifugal cicatricial alopecia
Histological examination of affected area showed premature desquamation of inner root sheath (Figure 2) and concentric lamellar fibroplasia of affected follicle (Figure 3) lymphocytic perifollicular inflammation.
DiscussionCentral centrifugal cicatricial alopecia (CCCA) is the term adopted by the North American hair research society (NAHRS) to encompass the previous terms of hot comb alopecia" follicular degeneration syndrome" pseudopelade" in African Americans and central elliptical pseudopelade in Caucasians.1
CCCA is a subcategory of primary inflammatory cicatricial alopecia and is the most common form of scarring alopecia in any population that includes a significant number of black patients. The majority of black patients with CCCA are women with female: male ratio of about 3:1.34 The hypothesis of pathogenesis is that patients are predisposed to follicular damage because of an anatomic abnormality namely premature desquamation of the inner root sheath.345 All conditions that result in a progressive pattern of alopecia affecting the crown can mimic CCCA these include male and female pattern alopecia lichen planopilaris and even unusual cases of chronic cutaneous lupus erythematosus. A biopsy is often necessary to confirm the diagnosis.6 The disease begins and remains most severe on the crown or vertex of the scalp gradually expanding in a centrifugal fashion. Symptoms may be mild or absent. Most patients note only mild episodic pruritus or tenderness of involved areas.
Pustules and crusting may also be found in the minority of patients who suffer from rapidly progressive disease or bacterial superinfection (follicular decalvans).78 For relatively non-inflammatory disease a combination of a long-acting oral tetracycline (e.g. doxycycline or minocycline) plus a potent topical corticosteroid (e.g. clobetasol or fluocinonide) is usually sufficient to halt progression. Prolonged treatment for years is usually required for this chronic disease. For highly inflammatory cases ("folliculitis decalvans") an initial 10-week regimen of oral rifampin and clindamycin (both at 300 mg twice daily) was very effective in a non-blinded non-controlled 8 study.
Maintenance therapy (such as oral doxycycline plus topical clobetasol) should follow.
ConclusionWhenever we come across patient presenting with progressive pattern of alopecia affecting the crown we should consider all conditions that can mimic CCCA. Although most often cases of CCCA found among black women of African descent this case was not in this category. The patient was treated by oral tetracycline plus topical clobetasol and there was a noticeable response to treatment.
1.Olsen EA Callender V McMichael A et al. Central hair loss in African American women: Incidence and potential risk factors. J Am Acad Dermatol. 2011;64:245-52.
2. Blattner C Polly DC Ferrito F Elson DM. Central centrifugal cicatricial alopecia. Indian Dermatol Online J. 2013;4:50-1.
3. Sperling L Sau P. The follicular degeneration syndrome in black patients: hot comb alopecia" revisited and revised. Arch Dermatol. 1992;128:68-74.
4. Sperling L Skelton H Smith K et al. The follicular degeneration syndrome in men. Arch Dermatol. 1994;130:763-9.5. Sperling L Solomon A Whiting D. A new look at scarring alopecia. Arch Dermatol. 2000;136:235-42. 6. Callender VD McMichael AJ Cohen GF. Medical and surgical therapies for alopecias in black women. Dermatol Ther. 2004;17:164-76.7. Brooke RC Griffiths CE. Folliculitis decalvans. Clin Exp Dermatol. 2001;26:120-2. 8. Powell J Dawber R Gatter K. Folliculitis decalvans including tufted folliculitis: clinical histological therapeutic findings. Br J Dermatol. 1999;140:328-33.
|Printer friendly Cite/link Email Feedback|
|Author:||Keen, Mohammad Abid; Hassan, Iffat|
|Publication:||Journal of Pakistan Association of Dermatologists|
|Date:||Jun 30, 2014|
|Previous Article:||Sunitinib-induced hand-foot syndrome in a Kashmiri male.|
|Next Article:||Profile of dermatological diseases in Pondicherry University health centre.|