Cutaneous angiolymphoid hyperplasia with high endothelial venulesT98.8

Fernández-Figueras MT et al. (2001)

Angiolymphoid hyperplasia with high endothelial venules (ALHE) is a rare, benign vasculoproliferative cutaneous disease characterized by a combination of proliferating blood vessels with characteristic high endothelial venules and a strong lymphocytic infiltrate. The autonomy of angiolymphoid hyperplasia with high endothelial venules is still unclear.

The cause is still unknown. ALHE is an idiopathic, benign, lymphoproliferative reaction of the skin. The distinction from Kimura's disease (angiolymphoid hyperplasia with eosinophilia), with which it was often confused in the past, is blurred and can only be made histologically.

Common in young adults (20-40 years), slightly more common in women.

Mostly head and neck area, especially ears and temples.

Solitary or multiple reddish-livid, usually asymptomatic, but sometimes painful papules, nodules or plaques. Bleeding easily.

Proliferation of small to medium-sized blood vessels. The vascular endothelia are often epithelioid (plump, with eosinophilic cytoplasm) with prominent venules and characteristic cytoplasm-rich high endothelial cells (cobblestone pattern). Furthermore, there is a dense lymphocytic infiltrate, often with eosinophilic granulocytes. No evidence of atypia or destructive growth.

Immunohistochemistry: CD31+, CD34+ (endothelial cells); VEGF+ (vascular growth factors); T- and B-cell infiltrate mixed, polyclonal.

Angiolymphoid hyperplasia with eosinophilia

Kimura's disease (more subcutaneous, often with lymph node involvement and IgE elevation)

Kaposi's sarcoma: mostly multiple, HIV positivity

Lymphoma / pseudolymphoma: Histology diagnostic

Surgical excision is the treatment of choice (but recurrence rate possible)

Alternative: Laser therapy

Alternative: Corticosteroids (topical/intralesional)

Alternative: Imiquimod

Slow, progressive course, spontaneous remission possible, but rare.

1. Fernández-Figueras MT et al. (2001) Cutaneous angiolymphoid hyperplasia with high endothelial venules is characterized by endothelial expression of cutaneous lymphocyte antigen. Hum Pathol 32:227-229.
2. Fernandez-Flores A et al. (2015) Expression of WT-1 by the vascular component of acral pseudolymphomatous angiokeratoma of children. J Cutan Pathol 42(1):50-55
3. Lessa PP et al. (2013) Acral pseudolymphomatous angiokeratoma: case report and literature review. An Bras Dermatol 88(6 Suppl 1):39-43.