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C5

 

 CASE #3 (presented by Wesley King Galen and Anupama Kewalramani)

 This 81/2 year old  girl has had a tan maculopapular, generalized eruption since birth. The lesions began as a few pruritic spots on the abdomen and then progressively spread to involve most of the skin surface.  Currently, the lesions consist of tan-pink macules, patches, papules, and plaques.
 
 


Fig. 9 (first biopsy): An interstitial infiltrate of uniform, mononuclear cells in the reticular dermis is most prominent in the upper ˝ of the dermis but extends close to the deep margin of the dermis. Focally, dermal papillae are bulbous and edematous. There are mild perivascular infiltrates in the altered papillary dermis.


 

feb20case3-4(91)

Fig. 10 (first biopsy): Small, mononuclear cells loosely infiltrate the interstitium of the reticular dermis. Similar cells are loosely clustered in a perivascular space in the center of the field.


 

Fig. 11 (first biopsy): The infiltrating cells are cytologically uniform. They have a thin rim of cytoplasm and round or oval nuclei with uniformly distributed chromatin. The infiltrates extend along vessels into the overlying papillary dermis.


 

Fig. 12 (second biopsy):  On this specimen, taken after an interval of 1 year, the infiltrates are dense in the reticular dermis. The intense eosinophilia of the infiltrates in part represents the presence of a high component of eosinophils. The overlying epidermis is hyperplastic.


 

Fig. 13 (second biopsy): Focally, tumor cells are loosely clustered among the collagen bundles. They have “histiocytoid” qualities. Some of the nuclei are indented. The nuclei are enlarged and have more open chromatin patterns. The cells show a thin rim of cytoplasm. Some of the nuclei are rounded with marginated chromatin and prominent nucleoli.


 

Fig. 14 (second biopsy):  In this portion of the specimen, the collagen bundles of the reticular dermis do not form a background. Instead, there is a distinctive fibrosing pattern with fascicles of fibers and cells that intersect to produce ill-defined starburst patterns. On the basis of this pattern, the lesion might be characterized as having fibrogenic properties. 


 
 

Fig. 15 (second biopsy): At higher magnification, the tumor cells are spindle shaped among the delicate fibers. There is a loose infiltrate of eosinophils. Small vessels with thick walls are also a feature.


 

Fig. 16 (second biopsy): Some of the tumor cells are rounded; most are spindle shaped. They have delicate dendrites and are associated with a loose infiltrate of eosinophils. Nuclei are enlarged and have open chromatin patterns. Some show central nucleoli.

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