What syndrome is this? Costello syndrome.
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Abnormalities, Multiple↗
Biomedical subjects
Giuseppe Micali
Publications and source records attributed to Giuseppe Micali.
Cutaneous manifestations and massive genital involvement in Hennekam syndrome.
We describe a 16-year-old boy with intestinal lymphangiectasia, lymphedema of the limbs and genitalia, mild mental retardation, and facial anomalies (Hennekam syndrome) and cutaneous lesions. Severe edema in the genital area created a gigantic mass that included the scrotum and penis. Numerous grouped red-violaceous pseudo-vesicular lesions and plaques, as well as verrucous brown papules, were present on the penis and scrotum. The prepuce was hypertrophic, with severe phimosis. Histologic analysis revealed dilated lymphatic vessels lined by a discontinuous layer of flat endothelial cells in the papillary dermis and extending down to the reticular dermis. Dilated blood vessels were also present but no cellular abnormalities were identified. A diagnosis of superficial cutaneous lymphatic malformations was made. To the best of our knowledge, this is the first detailed cutaneous histologic investigation in a patient with Hennekam syndrome. We assume that the onset of cutaneous lesions in our patient was likely triggered by a generalized worsening of his lymphedema.
Adolescent↗
Videodermatoscopy improves the clinical diagnostic accuracy of multiple clear cell acanthoma.
A 69-year-old male presented with about 20 reddish, sharply circumscribed, smooth papules, 5-10 mm in diameter, sometimes with a peripheral scaling collarette, scattered on the legs, which appeared during the last 5 years. Videodermatoscopic examination showed a similar pattern in all lesions, consisting of symmetrical and homogeneous pinpoint-like vascular structures which tended to be arranged in a net-like pattern. At higher magnification each vascular structure appeared to have a bush-like aspect. On the basis of past medical history, clinical and dermatoscopic features, the diagnosis of multiple clear cell acanthoma (CCA) was considered. Histologic examination of a papular lesion confirmed the diagnosis. In conclusion, although the dermatoscopic pattern of CCA is not specific, videodermatoscopy may improve the clinical diagnosis of single or multiple CCA, ruling out clinically similar disorders that do not show the same dermatoscopic vascular pattern of CCA.
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