Round to slightly oval nucleus with pink cytoplasm
•
Grains
Raisinoid nuclei with pink cytoplasm
•
Minimal to no cytologic atypia
Top Differential Diagnoses
•
Darier disease
Histologically similar
Clinically different
–
Multiple lesions in seborrheic distribution
•
Acantholytic dyskeratotic acanthoma
Similar to WD
–
Both have acantholysis and dyskeratosis
–
Both are usually clinically solitary lesions
Unlike WD
–
Not cup-shaped or associated with hair follicles
•
Acantholytic squamous cell carcinoma
Infiltrative
Cytologic atypia
Atypical mitoses
TERMINOLOGY
Abbreviations
Definitions
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Cup-shaped epidermal proliferation
•
Acantholysis is present
•
Dyskeratosis (corp ronds and grains) is present
ETIOLOGY/PATHOGENESIS
Pathophysiology
•
Germline mutations in
ATP2A2 not described (as are seen in Darier disease)
•
Human papilloma virus infection has not been associated with WD
CLINICAL ISSUES
Epidemiology