Anonychia with bizarre flexural pigmentation-an autosomal dominal dermatosis

A nulliparous female aged 26 years, who presented with long-standing sore and cracked soles, showed congenital anonychia, long-standing hyper-and-hypo-pigmentation in axillae and groins, dry palmar and plantar skin as well as other skin and hair abnormalities. She gave a family history of nail, hair and pigmentary abnormalities and examination of living affected relatives confirmed this. This appears to be a hitherto undescribed autosomal dominant syndrome.