PALEFACE is the acronym for
This is a very rare, benign, reactive-inflammatory, vasculoproliferative cutaneous disease that is still controversial in terms of its independence and can be classified histologically and clinically between ALHE (angiolymphomatoid hyperplasia) and a pseudolymphoma of the skin.
Children and adults are affected.
Face, neck, upper torso
Solitary or multiple, painless, flat, portentous, skin-colored or reddish small papules.
Papular architecture of the lesion
Prominent, epithelioid endothelial cells (similar to angiolymphoid hyperplasia of the skin with highly endothelial venules - ALHE)
Angioid vascular formations
Lymphocytic infiltrate, T-cell dominant
No true atypia or destruction
No invasive growth
No or little eosinophilia (unlike ALHE or angiolymphoid hyperplasia with eosinophilia (epithelioid angioma).
Immunohistochemistry
CD31+, CD34+ (endothelial cells)
CD3+ T cells (predominantly CD4+)
CD20+ B cells sporadically
Polyclonal TCR and IgH gene rearrangements (i.e. non-malignant)
Angiolymphomatoid hyperplasia with high endothelial venules (ALH-HEV)
Cutaneous pseudolymphoma (TRAPP)
Angiolymphomatoid hyperplasia with eosicnophilia
Kaposi's sarcoma (in atypical cases)
Papular form of mycosis fungoides (early stage)
Surgical excision is usually sufficient
For multiple lesions, if necessary:
This entity was propagated as a new clinical picture by Kempf et al. in 2022, as certain papular skin changes in children and adults could not be clearly assigned to "angiolymphoid hyperplasia of the skin with high endothelial venules/ALHE or angiolymphoid hyperplasia with eosinophilia or a classic pseudolymphoma".