Clinical features

•Rare, generally presenting as a solitary, flesh-colored papule or nodule on the central face with a predilection for the nose

•Often presents in children

•Less commonly affected sites include the scalp, nipples, limbs & external genitalia

•0.5-2.5 cm diameter

•Giant variant

•Non-syndromic

•Not associated with Muir-Torre syndrome

Histological features

•Circumscribed dermal nodule with a characteristic silhouette

•An infundibular cyst is surrounded by laminated dermal collagen, elastic fibers, blood vessels, adipocytes & spindle cells separated from the adjacent dermis by a retraction artifact

•The cyst contains keratinous debris, and its wall shows conspicuous  sebaceous & sometimes hair follicular elements are also evident 

•Significant association with Miescher nevus (as high as 20% for nasal lesions)

•Exceptionally may show spindle cell lipoma-like features

•Exceptionally includes a neural component

•Some authors believe that this entity overlaps sebaceous trichofolliculoma

Differential diagnosis

Folliculosebaceous cystic hamartoma can be distinguished from trichofolliculoma/sebaceous trichofolliculoma by the striking connective component and retraction artifact.