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Trichothiodystrophy–BIDS, IBIDS AND PIBIDS?

SIR, The occurrence of ichthyosis in the trichothiodystrophy or BIDS syndrome recently led Dr J.L.Jorizzo and colleagues {British Journal of Dermatology 1982,106, 705) to propose a possible subset in the form of the IBIDS syndrome. We have observed a patient with the IBIDS syndrome who also had photosensitivity. A io-year-old girl presented with short stature and the typical facial features, with micrognathia, prominent ears, bilateral congenital cataracts, impaired intelligence, extensive dental caries, ichthyosis, brittle hair and nails. The hair showed trichoschisis, with alternate banding on polarizing microscopy and cystine deficiency. In addition, 5-min sun exposure was sufficient to induce erythema, oedema and bullae on the exposed areas. Photosensitivity is not a recognized feature of the BIDS syndrome, but it has recently been reported in a patient with trichothiodystrophy who apparently lacked ichthyosis (Van Neste, 1982). Do these two cases with photosensitivity allow us to extend the acronym to PIBIDS?