Clinical features 

• Solitary or, more commonly, multiple, soft, elevated, and well-circumscribed papules, plaques, or nodules are observed. 
• Lesions are most commonly located over the face. Reported extrafacial locations include the scalp, the trunk, and the upper and lower extremities.
• The size of the lesions varies from a few millimeters to several centimeters in diameter.
• The color varies from shades of dull red to brown, blue, and purple.
• Lesions have a smooth surface with prominent follicular orifices (peau d'orange) and may be covered by telangiectases.

Copyright : Mitsuhiro Sugiura, M.D.

Histological features 

• The epidermis is unaffected. 
• A grenz zone of uninvolved dermis is located beneath the epidermis. Below the grenz zone is a dense, polymorphous inflammatory infiltrate located most often in the papillary and mid dermis. 
• The infiltrate consists of neutrophils, lymphocytes, eosinophils, monocytes, and, occasionally, mast cells. 
• Vasculitic changes, including perivascular inflammation with nuclear dust and vessel wall damage, are often observed. 
• Extravasated RBCs and hemosiderin deposition are found, which may contribute to the color of the lesions. 
• Later, lesions may show considerable fibrosis around venules.
• Direct immunofluorescence reveals immunoglobulin G, fibrin, and, occasionally, immunoglobulin M deposition at the basement membrane zone and perivascularly.
• Electron microscopy reveals more perivascular eosinophils than suggested by light microscopy. Charcot-Leyden crystals, the eosinophil granules, are evident within eosinophils as well as histiocytes, providing evidence of degranulation.

For  additional information about chondrodermatitis nodularis helicis see: eMedicine  and PathConsult 

Cases associated with this book:

Superficial basal cell carcinoma with a bonus diagnosis
Author: Artur Zembowicz M.D. Ph.D.

Conference: Dr. Z's Consultations