[Congenital atrophoderma in multiple plaques. A new clinical form of mastocytosis?].

The authors report the case of an eight-day-old male child with multiple yellowish atrophic plaques, approximately 2 to 10 cm in diameter, on the scalp, neck and abdomen. Darier's sign was negative. No visceral involvement was detected. Histological examination showed numerous histiocytic-like cells with a foamy cytoplasm throughout the dermis. Metachromasy with toluidine blue stain and naphtol AS-D chloracetate esterase were negative. Mastocytes were identified upon ultrastructural examination which demonstrated long interdigitated villi. Unusual features were the absence of typical cytoplasmic granules and the presence of multiple large electron-lucent vacuoles which were assumed to be stored lipids. The authors hypothesized that chronic stimulation of mastocytes in utero may have led to cell degeneration through an immunologic or pharmacologic mechanism. This could explain the negativity of specific stains for mastocytes and the collagen and elastic damage responsible for the atrophy of the lesions.