PALEFACE L98.8

PALEFACE is the acronym for

• Papular
• Angiolymphoid
• Inflammatory
• Focal
• Atypical
• Cellular
• Entity

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:

• Topical or intralesional corticosteroids
• Laser treatment
• Observation possible in asymptomatic cases; recurrences are also possible; malignant progression is not known.

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".

1. Alfarhan A et al. (2023) Angiolymphoid hyperplasia with eosinophilia: a case series and literature review. Int Ophthalmol 43:2457-2467.
2. Fernández-Figueras MT et al et al.(1995) Of APACHE and PALEFACEs. Am J Dermatopathol17:2009
3. 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.
4. 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
5. Lessa PP et al. (2013) Acral pseudolymphomatous angiokeratoma: case report and literature review. An Bras Dermatol 88(6 Suppl 1):39-43.