PMC9871614_01
Mounded and refractory keratoses, an atypical presentation of pemphigus vulgaris affecting the scalp. Presentations can include brown-gray mounded scales with or without the crust.
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F1
A physical exam revealed dry, matted mounded plaques with powdery scales, some with yellow-green crust, present throughout his entire scalp (Figure 1).
(A-D) The initial biopsy taken from the scalp in Case 1 was interpreted on H&E (40x) to be consistent with Darier disease since the biopsy demonstrated acantholysis with prominent superficial dyskeratosis. Subsequent biopsy with direct immunofluorescence testing confirmed pemphigus vulgaris, without histologic evidence of pemphigus vegetans.
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F2
A scalp biopsy showed focal acantholytic dyskeratosis, favoring Darier disease (Figure 2).
PMC9871614_02
(A) Mounded and refractory keratoses presenting as exuberant scale on the scalp (Case 2). (B) Close-up image of a plaque.
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F3
Multiple plaques with mounded scale and a verrucous appearance were noted on her scalp (Figure 3), while her trunk and extremities showed predominately post-inflammatory hyperpigmentation.
PMC9871614_03
Mounded and refractory keratoses as an early presentation of pemphigus vulgaris recurrence. In this patient (Case 3), there were no blisters present on the body or scalp.
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F4
A physical exam revealed circular gray, mounded plaques affecting his vertex scalp (Figure 4).
PMC9871614_04