Angiodestructive Type E Lymphomatoid Papulosis in the Setting of Pregnancy

blistering necrotic hemorrhagic lesion

A 29-year-old pregnant woman presented with an abrupt onset of blistering necrotic hemorrhagic lesion on the left hand. It subsequently resolved leaving an eschar. The patient then developed a 1.1-cm demarcated purple nodule located on the dorsal aspect of the right foot (Figure 1). The nodule was tender to palpation. The nodule has since undergone progressive involution. The patient felt otherwise well. A biopsy was performed.

Figure 1. Demarcated purple nodule (1.1 cm) located on the dorsal aspect of the right foot.

Light Microscopy

The biopsy showed a striking zone of epidermal and dermal necrosis (Figure 2). The viable skin showed a striking angiocentric atypical mononuclear cell infiltrate surrounding and permeating blood vessels with attendant injurious vascular alterations as characterized by endothelial cell necrosis with luminal and mural fibrin deposition (Figure 3). The cells in apposition to the vessels had an immunoblastic appearance. The cells were 12 to 15 µm  manifesting moderate amounts of cytoplasm and conspicuous nucleolation. A few other inflammatory cells were noted primarily in the context of neutrophils and eosinophils. 

hematoxylin-eosin statin

Figure 2. Low power hematoxylin-eosin stain (20×) shows a striking zone of epidermal and dermal necrosis. The viable skin showed a striking angiocentric atypical mononuclear cell infiltrate.

Phenotypic Profile

The atypical lymphocytes expressed CD3 (Figure 4), CD8, granzyme, TIA, and CD30 (Figure 5), while the CD20 stain was negative. There was a significant decrement in staining for CD7. 

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