PMC8055181_01
A, Asymmetrical facial angioedema. Angioedema was more prominent on the cheek and eyelid. B, Presence of hives on the thorax. Pruritic and painful purplish papules were observed on the left anterior thorax (black arrow). C, Urticarial circles that emerged on the lateral neck. D, Painful purplish macules can be seen, suggesting vasculitis.
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The episodic angioedema was added to pruritic and painful skin eruption of erythematous circles, which persisted for more than 24 h, that were palpable, somewhat purplish, and more pronounced on the face, arms, and trunk (Figure 1A-D).
A, Bone marrow biopsy (HE 400x, scale bar 0.1 mum) with an enrichment of eosinophils. B, Bone marrow biopsy (200x, scale bar 0.2 mum) with CD117 immunostaining, suggesting an increase of mast cells. The immunostaining for myeloperoxidase (C, 100x, scale bar 0.4 mum) shows an elevated myeloid to erythroid proportion. Bone marrow presented grade 1 fibrosis, as evidenced by reticulin staining (D, 200x, scale bar 0.2 mum). Panels E (40x, scale bar 1 mum) and F (400x, scale bar 0.1 mum), Skin biopsy showing dermal small capillary vessels with inflammatory infiltrate in the walls, suggesting urticarial vasculitis.
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Histologic sections of bone marrow biopsy (Figure 2A-D) showed a high cellularity specimen (around 70%), with increased myeloid cells, markedly exhibiting eosinophilic differentiation, leading to 8:1 granulocyte to erythroid proportion. Skin biopsies (Figure 2E and F) showed a leukocytoclastic vasculitis, predominantly with intact and degenerated neutrophils and eosinophils.
Serial blood cell counts after the patient began treatment. Dashed lines represent the simple moving average trend. Leukocyte counts, especially neutrophils and eosinophils, present a sinusoidal-like trend, suggesting a cyclic pattern. One could think that the cyclic shape could be due to irregular adherence of the patient to corticoid-based treatment. Another explanation could be that the multilineage cell cycling observed in patients with episodic angioedema with eosinophilia occurs despite corticoid treatment.
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The patient has been followed-up and consecutive blood cell counts have been taken (Figure 3).