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1. Histopathology of Inflammation

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 CASE #2 (presented by Wesley King Galen and Venetia Patout)

  This eight year old girl’s disorder was first diagnosed at the age of 9 months. She presented with hyperpigmented macules of the trunk. At 19 months of age, she began to develop periorbital hyperpigmented macules that spread to the perioral and submental areas over the following years. At the time of the meeting, hyperpigmented macules involved the periorbital, perioral and submental areas, and the back, chest, arms, and legs. Over the years many of the lesions have cleared.

Fig. 4 (first biopsy): Infiltrates of small cells are perivascular in the reticular dermis. They are prominent in perifollicular connective tissue sheaths but this reflects the rich vascularity of this anatomic compartment. The epidermis shows slight acanthosis and papillomatosis with scattered accentuations of the keratin layer.


Fig. 5 (first biopsy): The cells in the perivascular spaces are small and cytologically uniform. They have a thin rim of lavender cytoplasm and some have a perinuclear halo (a feature providing a plasmocytoid quality). Some of the nuclei are elongated or oval in outline. Mononuclear cells also form loose, non-specific interstitial infiltrates in the neighboring reticular dermis.


Fig. 6 (second biopsy): This biopsy taken after an interval of several years shows a lesion mostly confined to a widened papillary dermis. There are loose micronodular infiltrates of mononuclear cells in the papillary dermis. The epidermis shows partial erosion of the rete ridges.


Fig. 7 (second biopsy): At higher magnification, the nodularity in large part represents a contribution of tortuous, thick-walled vessels of the papillary dermis. The perivascular infiltrates are sparse and loose. On the left, fibrous lamellae partially encircle a lobular aggregate of vessels.


Fig. 8 (second biopsy): In the micronodular, fibrovascular components, it is difficult to identify specific, infiltrating cells. Some of the vessels in cross-section show duplication of basement membranes.

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