Congenital self-healing histiocytosis (Hashimoto-Pritzker). An ultrastructural and immunohistochemical study.

Congenital self-healing histiocytosis (CSHH) is a rare primary histiocytic skin disorder. Only a few cases have been studied by ultrastructure and immunohistochemistry. Here we report a new case that was investigated using an electron microscope and a panel of monoclonal (MCA) and polyclonal (PCA) antibodies.

[Hashimoto-Pritzker congenital self-healing histiocytosis].

A full-term female infant was born with numerous purple firm indolent papulonodular lesions, occasionally ulcerated, ranging in size from 1 to 8 mm, scattered all over the body. The liver, spleen, lymph nodes were normal, as were the blood count, skull and chest X-ray. Biopsy of a skin lesion showed a deep dermal infiltrate consisting of histiocytic cells which were OKT6 +, HLA-DR + and S 100 +.

[Renal manifestations of severe human aphthous diseases (author's transl)].

Renal manifestations of severe human aphthous diseases are seldom mentionned in the literature. The authors report on a case of Behçet disease associated with microscopic haematuria and intermittent proteinuria. Renal biopsy showed circumscribed "necrotizing" vascular lesions.