Susana Giraldi

Cutis verticis gyrata in a child with Turner syndrome.

To the Editors: Cutis verticis gyrata (CVG) is a rare disorder characterized by elevations and depressions of the skin that form cerebriform folds. Data on the association of CVG with Turner syndrome (TS) are scarce (1,2). This report emphasizes the association of CVG with chromosomal abnormalities and proposes inclusion of these syndromes as secondary causes of CVG. We saw a Caucasian female infant 18 days old, the product of a full-term, uncomplicated pregnancy who presented with abnormal scalp folds at birth (Fig. 1). Her parents were nonconsanguenous. The infant’s weight and height were in the 5th percentile. She had surplus skin over the nape of the neck, edema of the feet, epicanthi, ogival palate, and furrows resembling gyri on the anterior biparietal portion of the head measuring 3 cm × 2 cm, with absence of hair. The neurologic and cardiac evaluations were normal. Laboratory tests found negative chromatin in buccal smears, with a 45-XO karyotype. A skin biopsy specimen from a lesion revealed a normal epidermis and thick fibrous bands of collagen in the superficial and reticular dermis. At 5 months of age, the edema of the feet and folds on the scalp persisted and a winged neck was also noted. At 10 months her height was in the 3rd percentile and weight in the 10th percentile, with normal growth velocity and normal mental status. In 1953, Polan and Butterworth (3) classified CVG as primary (or idiopathic) and secondary. As a primary illness, it is usually associated with neurologic abnormalities. When secondary, it can be due to inflammatory changes; tumors, nevi, neurofibroma and fibroma; trauma; and miscellaneous disorders such as acromegaly, myxedema, cretinism, leukemia, syphilis, acanthosis nigricans, and tuberous sclerosis. The presence of CVG without other manifestations is termed primary essential CVG (4). TS manifests clinically at birth with edema over the dorsum of the hands and feet and accentuation of the nuchal folds. Marini et al. (1) reported a case of CVG associated with TS and suggested that the congenital lymphedema presented in this syndrome might be the cause of the CVG. Lymphedema in TS is thought to originate from a congenital abnormality of the lymphatic vessels, which can be caused by hypoplasia or by obstruction of their drainage (5). Embryologically the lymphatic vessels of the scalp drain to the jugular lateral sacs and these normally communicate with the final portion of the right lymphatic duct and the thoracic ducts. In the absence of a connection between the venous and lymphatic systems, as occurs in some chromosomal abnormalities, there is lymph accumulation in the jugular sacs and in the tissues, causing cystic hygroma and hydrops. If there is intrauterine correction or if an alternative route of lymphatic drainage develops, there will be reabsorption of the edema and regression of the cystic hygroma. In TS and other aneuploid syndromes this results in congenital winged neck and puffiness of the dorsum of the hands and feet (6–8). Approximately 80% of children with TS are born with lymphedema, mainly of the dorsum of the hands and feet. Cystic hygroma occurs in 50% of patients. Usually the lymphedema resolves by recanalization of the lymphatic vessels. The resolution of both edema and cystic hygroma could explain the dermatoglyphic abnormalities, the pterygium coli, and the low hairline that occurs in TS (5,9–11). It is postulated that CVG might also result from resolved intrauterine scalp lymphedema (1,2). The echographic detection of lymphedema in a fetus with CVG and TS has been reported (12). Lymphedema and cystic hygroma may also occur in Down and Edwards syndromes (13). Newborns with these chromosomal abnormalities, characterized by “pterygium coli” at birth, were noted by ultrasound between the 14th and 16th weeks of gestation (13) to have cystic hygroma and generalized edema (including the head). At present, in Polan’s classification, chromosomal alterations are not listed as a cause of CVG. Some authors have shown the association of CVG with TS (1,2) and we Figure 1. Furrows on the anterior biparietal portion of the head.

Tinea nigra: relato de seis casos no Estado do Paraná

Six cases of Tinea nigra attended at our Pediatric Dermatology Clinic in Hospital de Clinicas de Curitiba - UFPR from 1978 to 2001 are presented. Four cases are schoolchildren and had contact with sand on a beach and two are adolescents. One with bilateral plantar lesions represents the first case reported in national literature.

Paraqueratose granular: relato de seis casos em crianças

Granular parakeratosis is an alteration of keratinization that was first described in adults. It is characterized by hiperkeratotic plaques and papules in intertriginous areas. The authors describe six cases of granular parakeratosis in children. One patient had lesions on the buttocks; two children presented papules in both axillae and cervical region (presentations never described before in the literature). The remaining three patients presented with lesions in the inguinal folds. Review of the literature and discussion on the pathogenesis of this rare dermatosis are presented.