Fig. 3
From: H syndrome: 5 new cases from the United States with novel features and responses to therapy
Patient 2, skin punch biopsy. a. Low-power view of skin overlying mons pubis shows patchy edema and chronic inflammation of the deep dermis and subcutaneous tissue with focal lymphoplasmacytic aggregates as well as mononuclear inflammatory cells dispersed within the interstitium. b. High-power view of deep dermal inflammatory aggregates shows prominent perivascular lymphoplasmacytic cuffing as has been described in cutaneous Rosai-Dorfman and related diseases. c. Foci of dermal edema contain scattered mononuclear cells including histiocytes, although emperipolesis is not appreciated. d. Immunohistochemistry for CD163 confirms the presence of dermal histiocytes, with occasional larger histiocytes highlighted. e. The histiocytes noted in 3D are not highlighted by immunohistochemistry for S100 protein; a peripheral nerve in the center of the field serves as a positive internal control