An unusual radiological presentation of pulmonary Langerhans' cell histiocytosis

Abstract

Langerhans' cell histiocytosis is a proliferative disease of the dendritic cell lineage. It usually has a characteristic radiological presentation of apical nodules and small cysts occurring predominantly in young adult smokers. Here we report a case of multisystem Langerhans cell histiocytosis in a 75-year-old woman with unusual chest radiology.

Figure 1

Submammary skin rash.

Figure 2

Chest x-ray on presentation.

Figure 3

CT thorax demonstrating peripheral septal thickening and ground glass opacification.

Figure 4

Skin biopsy: within the skin biopsy from submammary chest wall, there was an upper dermal and epidermotropic infiltrate of relatively uniform large cells disposed loosely and in aggregates. They displayed abundant eosinophilic cytoplasm and infolded or bean-shaped vescicular nuclei sometimes with longitudinal grooves. Positive immunohistochemical staining with S100, CD1a and langerin (CD207) demonstrated these to be differentiated Langerhans cells. The histiocytic marker CD68 was negative. The more specific MelanA and HMB45 melanoma markers were negative, and CK7 was negative for Paget's disease. Admixtures of lymphocytes and scattered eosinophils were also present within the dermis.

Figure 5

Open lung biopsy. 1—high-power H&E showing the septa containing a mixed infiltrate, including histiocytes. 2—high-power of CD1a stain showing the Langerhan cell histiocytes.

Figure 6

CT thorax 6 months following commencement of therapy.