S7C9P6-Neutrophilic Pemphigus

S7C9P6-1: Some examples of pemphigus are characterized by neutrophilic infiltrates; in some of these examples, the infiltrates tend to concentrate in areas of impending acantholysis. On the left in this example, there is a well-developed bulla at the interface between the superficial and basal units of the epidermis. The keratin layer is normal. The basal unit of the epidermis is hyperplastic in psoriasiform patterns. To the right of this defect, in an area identified by red arrows, there are two rounded defects which contain neutrophils.

S7C9P6-2:  In this example, the defect is somewhat rounded and acantholysis is not a prominent feature. The defect contains proteinaceous material. The separation in the epidermis is between the basal unit and the superficial unit (neutrophilic pemphigus). The concave configuration of the floor of the bulla is evidence of a tense vesicle.

S7C9P6-3: At a margin of the same lesion (as seen in S7C9P6-2), there is some evidence of cellular disarray at the interface between the basal and the superficial units of the epidermis (red arrows); neutrophils are distributed along the interface between the superficial unit and the basal unit. There are also cytopathic changes with some of the cells at the interface showing increased cytoplasmic acidophilia. The dermis is relatively free of inflammatory cells. There are “apoptotic” bodies (nuclear and cytoplasmic debris).

S7C9P6-4: Neutrophilic infiltrates define the boundary between the basal and the superficial units (neutrophilic pemphigus). There are scattered fragments of nuclear debris.

S7C9P6-5: This example of neutrophilic pemphigus might be dismissed as pemphigus foliaceus. If, however, attention is given to the nature and distribution of the intra-epidermal infiltrate adjacent to the zone of acantholysis, neutrophils are the reacting cells; they are distributed in a plane which correponds to that of the zone of acantholysis. The separation seems to begin at the interface between the basal and the superficial units of the epidermis (intermediate-zone, “neutrophilic” or “intermediate” pemphigus).

 

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