Histology and electron microscopy of fucosidosis of the skin. Subtle clues to diagnosis by electron microscopy.

Fucosidosis is an autosomal recessive inborn error of metabolism in which fucose-containing glycolipids, glycoproteins, and oligo- and polysaccharides accumulate in tissues as a consequence of alpha-L-fucosidase deficiency. Since the detection of this entity in 1966 several cases have been described, but until now investigations of clinically uninvolved skin have not been performed. In this study we have investigated clinically normal skin obtained from a patient with fucosidosis and his healthy sister, by light and electron microscopy, to determine whether normal skin in this condition yields clues that may have prognostic relevance.

[Sweet syndrome. Acute neutrophilic dermatosis in multiple myeloma].

A 64-year-old woman complained of reddish plaques that had suddenly appeared on her upper arms and trunk. Histological investigation revealed perivascular distribution of a neutrophilic infiltrate in the upper and mid-dermis; direct immunofluorescence showed deposits of C3 and fibrin in and around the vessels. Laboratory values in the blood, X-ray of the head and fine-needle biopsy of the hip showed the typical pattern of multiple myeloma.

A clinical, histological, and immunohistochemical comparison of acrodermatitis chronica atrophicans and morphea.

We compared 19 patients with acrodermatitis chronica atrophicans (ACA), a dermatosis caused by Borrelia burgdorferi infection, and 40 patients with morphea, a disease of heterogeneous origin where a borrelia etiology has been suggested in some cases, both clinically and histologically to define the differences between these two dermatoses. Clinically, ACA involves acral body sites with lower temperatures, is seen mostly in elderly persons, and presents as a livid discoloration that is not sharply demarcated. Morphea can be localized in embryonal structures, affects any age and body site, and exhibits extension at the periphery of the lesions.

[Serology of HIV infection].

Serological testing aimed at the detection of antibodies against the human immunodeficiency virus (HIV) is now performed throughout the world to screen stored blood for transfusion, for the diagnosis of HIV infection and of AIDS, and to determine the prognosis of AIDS patients. The present report, based on 258,090 serum samples screened for HIV antibodies, gives an overview of the theoretical and practical aspects of both modern screening methods and confirmation tests for HIV1 and HIV2, the demonstration of serological antigen and IgM, and HIV serology of the cerebrospinal fluid. Knowledge of HIV serology appears to be important for any physician, and in particular for the dermatologist, since HIV infection (like syphilis) can cause a variety of dermatological manifestations mimicking other dermatoses.