Partial Spiky Acantholysis due to Loss of Desmosomal Attachments in Erythrokeratodermia Cardiomyopathy Syndrome

Desmosomal‐type acantholysis may be a clue to underlying desmoplakin variants, which can be associated with cardiomyopathy. Recognition of this unique and sometimes subtle pattern of acantholysis can be beneficial for patient management and outcomes. Biopsies from three patients with erythrokeratodermia cardiomyopathy and known variants in desmoplakin were examined for the presence of desmosomal‐type acantholysis. Biopsies from all three patients had findings of increased space between keratinocytes of the basal and sometimes suprabasal layers, sometimes causing the polygonal keratinocyte border to appear spiky. Desmosomal‐type acantholysis may be a clue to an underlying defect in keratinocyte desmosomes.