Follicular atrophoderma and basal cell epitheliomata
Most BCCs manifest during the second and third decade of life, but onset in the first decade has been reported. Lesions are typically localized to sun-exposed areas, particularly the face, and follow an aggressive course. Relapses are common.
Follicular atrophoderma occurs due to poor follicle unit development with subsequent dilatation and deepening of the follicular ostia. It may either be present at birth or it may develop early in life and typically manifests as follicularly based dells devoid of hair.
Hypotrichosis is typically congenital, occurs in 85% of cases, and improves with age. Involvement is usually widespread, but it can also be limited to the scalp, eyebrows, and eyelashes. Hair shaft abnormalities, including pili bifurcati, pili torti, and trichorrhexis nodosa, have also been reported.
Other less common manifestations of BDCS include milia, hypohidrosis, facial hyperpigmentation, and trichoepitheliomas.
For more information, see OMIM.
L73.8 – Other specified follicular disorders
254820002 – Follicular atrophoderma and basal cell epitheliomata
- Rombo syndrome
- Basal cell nevus syndrome (Gorlin syndrome)
- Schöpf-Schulz-Passarge syndrome
- Generalized basaloid follicular hamartoma
- Oley syndrome
- X-linked dominant chondrodysplasia punctata
Last Updated: 10/31/2018