1983 Volume 45 Issue 3 Pages 372-376
A 45-year-old man had a generalized pigmentary disorder with the onset at about age 12. In the family history a sister, 50 years old, had a similar skin pigmentary defect with the onset at a similar age. The histological features resembled those of xeroderma pigmentosum with an atrophy in the depigmented area and elongation of the epidermal rete ridges in the pigmented area. Ultrastructurally, ultrathin sections from the pigmented area showed melanin-pigment-accumulated keratinocytes with signs of degeneration. On the contrary, those from the depigmented area showed not such cells but rather vacuolated keratinocytes or keratinocytes with an electron lucent substance in the perinuclear area. In all the ultrathin sections, the melanocytes appeared almost normal. Occasional occurrence of amyloid deposition in close contact with the degenerated basal cells was suggestive of a keratinocyte origin. Therefore this pigmentary disorder may relate to abnormal conditions in pigment transfer, as the result of keratinocyte degeneration, and here hereditary factors may play an important role.
