Acantholytic bullous disease: Dariers disease (Keratosis folliculiris)

Deba P Sarma, MD Omaha

M 15 yrs, forehead

Comment

Clinical:
Site: Back, chest, axilla, neck. Oral mucosa may be involved.

Pathology:
Irregular acanthosis, papillomatosis, hyperkeratosis. Suprabasal clefts and lacunae containing dyskeratotic cells. Irregular upward proliferation of one layer of basal keratinocytes (villi) into the lacunae. Basophilic elogated cells with large nuclei and paranuclear halo (grains) in corneal layer. Corps ronds (basophilic cells with dark pyknotic nuclei and perinuclear halo) in the granular layer. Dermal chronic inflammation.