Search

Human piebaldism is an expression of neural crest component abnormality that is classically inherited dominantly and is probably heterogeneous genetically. Though usually a stable cutaneous condition, spontaneous pigmentation in hypomelanotic tracts is known. Here, pigmented skin around the axilla was transplanted to hypomelanotic areas in two patients with piebaldism. The cutaneous autografts, whether full-thickness punch grafts, split-thickness shave grafts, or epidermal suction grafts, retained their capacity of pigmentation. Moreover, there was spread of pigmentation around the grafts. Strategic placement of cutaneous autografts in the most conspicuous hypomelanotic areas of piebald persons can improve their appearance.

Full-thickness and epidermal autografts of normally pigmented skin were transplanted within hypopigmented areas in each of two patients with piebaldism. Full-thickness punch grafts 2 to 4 mm in size retained their pigmentation (donor dominance) and within 4 months pigment was found to have spread around them. Within a year, melanization covered roughly triple the diameter of the grafts (or 9 times their areas) and then came to a standstill. Epidermal grafts (about 12 mm in diameter) healed with no visible scars at either donor or recipient sites. These grafts, too, retained their pigmentation, but no pigment was seen to spread from them in 8 months of observation. Controls of hypopigmented, full-thickness autografts placed into comparable hypopigmented areas showed either no change or but slight pigmentation along the rim of the graft and no spread of pigment.

Lentiginosis profusa syndrome and progressive cardiomyopathic lentiginosis are preferable terms to "leopard syndrome". Considerable cosmetic improvement followed facial dermabrasion and light electrodesiccation of lentigines on the exposed surfaces. Hematologic evaluation did not reveal hematopoietic defect in the patient studied.

Black-hair follicular dysplasia in dogs of mixed breeding was delineated by hypotrichosis and dullness of most black regions of the coat. White regions remained full and lustrous. The abnormality was not grossly visible at birth but became evident after the 1st month of life. Among 15 offspring from 2 matings of an affected pair of dogs, each of 12 spotted pups developed the disorder in black regions; the remaining 3 were all white and appeared normal.

The natural pigmentary demarcation boundaries of the skin can be classified as: Group A--lines along the upper limb with variable trans-pectoral extension; Group B--lines along the lower limb; Group C--paired lines in median or paramedian course on the chest with midline abdominal extension; Group D--posteromedian demarcation; and Group E--bilaterally symmetrical, obliquely oriented hypopigmented macules on the chest. Groups A and C are the most distinct and occur in both Negroes and Japanese.

Ionically separated epidermal sheets from pigmentations of two patients with lentiginosis profusa were incubated in dopa (3,4 dihydroxyphenylalanine) and slide-mounted. Light microscopy revealed intracellular giant pigment granules which appeared like those seen with von Recklinghausen's disease.