S13C6bP4b-Letterer-Siwe



S13C6bP4b-1: The biopsy is from an infant with the clinical presentation of acute disseminated histiocytosis X (Letterer-Siwe’s disease). The infant presented with disseminated disease, including hepatosplenomegaly. The infiltrates are focally band-like in the papillary dermis; they are perivascular and peri-appendageal in the reticular dermis and subcutaneous fat. The epidermis shows acanthosis and effacement of the rete ridges.

S13C6bP4b-2: Mononuclear cells form monotonous infiltrates at the dermal-epidermal interface, in the adventitia of skin appendages, and in the perivascular spaces (in their distribution, the infiltrates mainly involve the adventitial dermis). There is little in the way of non-specific inflammatory cells.

S13C6bP4b-3: Band-like infiltrates of atypical histiocytes hug the epidermis. A few, similar cells have migrated into the epidermis.

S13C6bP4b-4: The cells of the infiltrates have pale, amphophilic cytoplasm and eccentric nuclei. The nuclei vary in size and outline. Some of the pale cells individually have migrated into the overlying epidermis. A blue arrow points to a mitotic figure.

S13C6bP4b-5: Many of the nuclei are deeply grooved (blue arrows). Some of the nuclei show a central nucleolus. The distinctive nuclear grooves are a characteristic of the nuclei of some of the cells in the infiltrates of histiocytosis X.

S13C6bP4b-6: There is some degree of cytologic atypia with variations in nuclear size and staining. A mitotic figure is represented near the center of the field. Nuclei are irregular in outline and some are deeply grooved or notched.

S13C6bP4b-7: For this example, the site in the dermis, that is selected for examination, seems to have a correlation with both cytologic expressions, and the degree of atypia. In this field, showing an infiltrate in the lower portion of the dermis, the atypia is less pronounced than in the infiltrates at the dermal-epidermal interface. Atypia is most pronounced near the dermal-epidermal interface (the adventitial dermis is mainly involved - the stroma for S100+ dendritic histiocytes of the dermis is mainly involved).

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