No abstract

No abstract

A three-year old patient had ichthyosis and neutral lipid storage disease (Chanarin-Dorfman syndrome), characterized by congenital ichthyosiform erythroderma and leukocyte vacuoles. He did not show any of the internal system involvement that was found in previously described cases. Patients with this syndrome demonstrate a great variability of clinical involvement. The affliction may be very mild as in our case or it may lead to rapid death, as with the patient’s brother born a Harlequin baby, who only survived 5 days. Because of this wide spectrum of clinical variability mild cases might escape diagnosis. It is, therefore, suggested that every case of ichthyosis should have a peripheral blood smear evaluation with special attention to the morphology of the leukocytes. Our case clearly demonstrates the value of such a screening examination. Although the patient had been under treatment for almost 3 years, only screening examination of blood smears of all the patients with ichthyosis finally led to the correct diagnosis.

No abstract

No abstract

No abstract

No abstract

Two biopsies of an erosive lesion of the nipple had an appearance of an acantholytic disease without showing malignant cells. Only a third biopsy through the nipple with removal of a larger portion revealed some nests of atypical, large cells with clear cytoplasm, typical of Paget’s disease. Immunohistochemical findings with carcinoem-bryonic antigen confirmed the diagnosis of Paget’s disease of the nipple. This is the first case of Paget’s disease which shows extensive acantholysis on microscopic examination and which resembles pemphigus vulgaris histologically. Acantholytic diseases are easily distinguished from Paget’s disease and have never been mentioned in the differential diagnosis of this disease. A large biopsy through the nipple with the removal of a liberal portion of the nipple is suggested in every case of a suspected unilateral lesion of the nipple in order to avoid the overlooking of small nests of Paget’s cells, as in our first biopsies, showing a histological picture of an acantholytic disease.