A Case of Netherton's Syndrome in a Newborn

Netherton's syndrome is an unusual disorder which consists of triad of ichtyosiform dermatosis, multiple defects of hair shaft and an atopic diathesis. The finding of bamboo hair is pathognomic in Netherton's syndrome and the ichthyosiform dermatosis may consist of either ichtyosis linearis circumflexa or congenital ichthyosiform erythroderma. Often, variability in the clinical features leads to a delay in diagnosis in many cases. We report a case of Netherton's syndrome diagnosed in the neonatal period. The patient presented with severe ichthyosis and confirmed microscopically distinctive bamboo hair.

Erythrokeratodermia Variabilis with Alopecia Universalis

We describe a 8-year-old girl with erythrokeratodermia varibilis (EKV). This diagnosis was supported by erythematous, configurate patches over the entire body. Their size, shape and location varyed with enviromental factors and emotional state. In addition, well defined brownish, hyperkeratotic plaques were scattered over the trunk and extremities. Also, this patient had alopecia universalis, which is not typical of EKV. Etretinate therapy resulted in an almost complete clinical clearing of the plaques; however, her alopecia universalis and erythematous lesions were not affected.