Cluster of Vacuolated Spinous Keratinocytes: A Clue to Cornoid Lamellation and a Case of Porokeratosis Disseminata.

Cornoid lamella is a definitive diagnostic feature of porokeratosis, a disorder of keratinization that has various different clinical presentations. A tiered column of parakeratosis and a diminished granular layer with dyskeratotic keratinocytes at the column base are all useful and known features of cornoid lamellation. We have found a cluster of vacuolated spinous cells to be a reliable fourth feature of cornoid lamellation and a feature when present alone to be a reliable sign of impending cornoid lamellation on deeper sections. Furthermore, we present a novel clinical phenotype of disseminated papular porokeratosis with effective clinical response to combination of phototherapy, oral and topical retinoids, and topical vitamin D derivative, which too contained all 4 features of cornoid lamellation.

Hailey-Hailey-Like Pattern of Acantholysis on the Scalp Should Raise the Possibility of Incipient Pemphigus Vulgaris.

Hailey-Hailey disease (familial benign chronic pemphigus) is a genodermatosis with a typical clinical presentation of macerated plaques involving intertriginous areas of the body and affects multiple family members. Epidermal acanthosis, incipient, and completed acantholysis of many of the spinous keratinocytes of the epidermis and foci of dyskeratosis are well-recognized histologic findings. We have observed solitary lesions on the "scalp" of patients having a Hailey-Hailey-like pattern, and not the suprabasilar clefting of pemphigus represents the first manifestation of pemphigus vulgaris, which was confirmed with direct immunofluorescence in all our patients.