In a series of fibrotic and sclerotic diseases including scars keloids fibromas scleroderma and lichen sclerosus et atrophicus, we found modifications in the dendrocyte population. Dendrocytes were numerous in fibrotic diseases associated with little deposits of collagen. Conversely, they were almost absent in sclerotic diseases when the lay-down of collagen was prominent. We hypothesize that dendrocytes, by their content in factor XIIIa, may limit the accumulation of collagen in the skin.

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We report a clinicopathological observation concerning a man and his son affected by the same disease. The main cutaneous lesions included poikiloderma congenitale and generalized alopecia respecting the pubic area. These anomalies were associated with cleft palate and Robin sequence in the boy. The main histological changes consisted in a marked atrophy of hairs with persistence of sebaceous glands. In sun-exposed areas we found colloid bodies melanophages and hyperplasia of the elastic tissue network. Those features resembled those of the Rothmund-Thomson syndrome, but the phenotype and the apparent dominant mode of inheritance exclude this possibility. A new genodermatosis, related with, but distinct from the Rothmund-Thomson syndrome is suspected, for which the acronym PARC syndrome is proposed.

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