| • | AKA keratosis follicularis (but not follicular) |
| • | autosomal dominant; presents in second decade |
| • | think of this disease and Hailey-Hailey together (Hailey-Hailey was once considered to be the vesicular variant of Darier’s: but Darier’s is chromosome 12q vs. H-H is 3q) |
clinical:
| • | hyperkeratotic papules coalescing to warty plaques in seborrheic distribution (esp. axillae, groin, inframammary folds) |
| • | worsened by humidity and UV light |
| • | clinical variants include hypertrophic, vesicobullous, and linear types |
| • | verrucous papules on dorsum of hands (i.e. acrokeratosis verruciformis of Hopf) |
| • | palmoplantar punctate keratosis/ pits |
| • | (highly characteristic) V-shaped notches at free edge; nail fragility |
| • | red and white alternating longitudinal bands |
| • | subungual hyperkeratosis |
| • | oral: (15-50%) white papules on hard palate; cobblestoning on buccal mucosa |
| • | treatment: oral retinoids |
pathology:
| • | focal acantholytic dyskeratosis, in which suprabasal clefts lie beneath columns of acantholytic and dyskeratotic cells |
| • | corps ronds = acantholytic, dyskeratotic cells with prominent perinuclear vacuoles |
| • | grains = their parakeratotic counterparts |
ddx:
| • | Hailey-Hailey; seborrheic dermatitis; Grover’s disease; PRP; psoriasis |
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