S13C5P3- Congenital Histiocytosis

S13C5P3-1: The lesions of regressing congenital histiocytosis share many features with those of classic histiocytosis X. As in classic histiocytosis X, the infiltrates may be focally, or diffusely, band-like in the upper portion of the dermis, particularly the papillary dermis. A distinction between the regressing disorder and classic histiocytosis X may be difficult to define histologically. Inflammatory crusts, as seen in this example of congenital regressing histiocytosis, seem to be a common feature of the regressing variant. The infiltrates often are cytologically bland, but this feature is of too subjective a nature to be of use in making a distinction. The distinctions are primarily based on clinical findings and observations. Age at the onset of the disorder is important; a history of regressing lesions is important. Blue arrows identify loose infiltrates of distinctive histiocytes in a widened papillary dermis. The eroded area to the right is seen at higher magnification in the next photo.

S13C5P3-2: Lesions of congenital regressing histiocytosis often have an inflamed quality; in this field, the epidermis to the left of the center of the field is eroded. The erosion somewhat resembles an early “neurotic excoriation.” Infiltrates of histiocytes are not a feature of the reaction in the dermis beneath the erosion (pressure from infiltrates of histiocytes cannot be cited as an explanation for the erosion).

S13C5P3-3: The cells of the infiltrates in the papillary dermis are loosely arranged in a delicate, edematous fibrous matrix. The nuclei of the histiocytes are irregular in outline.

S13C5P3-4: Some of the nuclei are notched and some have central nucleoli (blue arrows). The infiltrates press upon the basement membrane zone.

S13C5P3-5: The histiocytes of the infiltrates are immunoreactive for S-100 protein. Some of the positive cells appear to be reactive dendritic cells among the tumor cells. The observation, that some of the cells resemble reactive dendritic histiocytes, might be offered in support of a dual population of dendritic histiocytes, one of which has neoplastic qualities.

 

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