PIBIS syndrome is similar to BIDS syndrome

BIDS syndrome

BIDS syndrome, also called nonphotosensitive trichothiodystrophy 1 , Amish brittle hair brain syndrome and hair-brain syndrome, is an autosomal recessive genodermatosis similar to IBIDS syndrome but without the ichthyosiform changes...

, but also associated with photosensitivity

Photosensitivity

Photosensitivity is the amount to which an object reacts upon receiving photons, especially visible light.- Human medicine :Sensitivity of the skin to a light source can take various forms. People with particular skin types are more sensitive to sunburn...

, and in contrast to IBIDS syndrome

IBIDS syndrome

IBIDS syndrome, also known as trichothiodystrophy , photosynthetic trichthiodystrophy , trichothiodystrophy with congenital ichthyosis, Tay syndrome or sulfur-deficient brittle hair syndrome, was first described by Tay in 1971...

, the ichthyosis

Ichthyosis

Ichthyosis is a heterogeneous family of at least 28, generalized, mostly genetic skin disorders. All types of ichthyosis have dry, thickened, scaly or flaky skin...

 is mild and not congential. Additionally, there is a relationship with xeroderma pigmentosum

Xeroderma pigmentosum

Xeroderma pigmentosum, or XP, is an autosomal recessive genetic disorder of DNA repair in which the ability to repair damage caused by ultraviolet light is deficient. In extreme cases, all exposure to sunlight must be forbidden, no matter how small. Multiple basal cell carcinomas and other skin...

 in that virtually all PIBI(D)S patients have a deficiency in the nucleotide excision repair of UV-induced DNA damage that is indistinguishable from that of xeroderma pigmentosum group D.

See also

• BIDS syndrome
  BIDS syndrome
  BIDS syndrome, also called nonphotosensitive trichothiodystrophy 1 , Amish brittle hair brain syndrome and hair-brain syndrome, is an autosomal recessive genodermatosis similar to IBIDS syndrome but without the ichthyosiform changes...
  
  
• IBIDS syndrome
  IBIDS syndrome
  IBIDS syndrome, also known as trichothiodystrophy , photosynthetic trichthiodystrophy , trichothiodystrophy with congenital ichthyosis, Tay syndrome or sulfur-deficient brittle hair syndrome, was first described by Tay in 1971...
  
  
• Skin lesion
• List of cutaneous conditions