Pemphigus foliaceus (PF) is an autoimmune blistering disease characterized by the disruption of the epidermal cell adhesion protein desmoglein 1 (DsG1). PF classically presents with superficial erosions or blisters, but can rarely mimic other dermatological conditions, which makes diagnosis challenging. We report the case of a 57-year-old Sri Lankan man with a one-month history of widespread ichthyosis-like plaques and scales which started on his scalp and progressed in a cranio-caudal fashion and were associated with pruritus and few blisters. Histopathology revealed features consistent with PF, and immunofluorescence studies further confirmed the diagnosis of PF. This case highlights the atypical presentation of PF resembling ichthyosis, emphasizing the need to consider autoimmune blistering diseases for unexplained scaling dermatoses. The unusual nature of PF presenting with ichthyosis-like scaly plaques highlights the need for further research. Accurate early diagnosis and treatment can both significantly improve outcomes.
Keywords: blistering disease; ichthyosis; pemphigus foliaceus; pemphigus vulgaris; plaque; scale.
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