P28 HAIR REPIGMENTATION, A NOVEL CLINICAL SIGN IN PORPHYRIA CUTANEA TARDA.

FCG Shaffrali, AJG McDonagh & AG Messenger. Royal Hallamshire Hospital, Sheffield S10 2JF, UK

Porphyria cutanea tarda (PCT) is the commonest of the cutaneous porphyrias.  Increased skin fragility and blistering, hyperpigmentation, premature skin ageing and sclerodermoid changes are often seen but hair changes are less frequent, the commonest being hypertrichosis with alopecia only rarely reported.  We now report two cases with hair repigmentation, a previously unreported feature.

Case 1:  A 71 year old female was seen with a 6 month history of skin fragility with blistering on the dorsa of her hands.  Her previo the right stimulus.usly white hair had darkened during the same period and she had a sclerodermoid appearance of the upper trunk, facial hypertrichosis, blistering on her hands, and strikingly dark hair.  Biochemical studies confirmed PCT.  Treatment consisted of venesection and subsequently low dose chloroquine.  Follow-up over 8 years has seen improvement in the hypertrichosis and blistering, but no change in the sclerodermoid areas, and her hair has remained dark.

Case 2:  A 69 year old female was seen with a one year history of increasing pigmentation on the chest, face and lower abdomen, with darkening of her hair over the same period.  She had sclerodermoid changes on the trunk, with marked hyperpigmentation on the central area of her face.  There was diffuse scarring alopecia, and the remaining hair was dark.  Investigations confirmed PCT, and she remains on treatment with no change in hair pigmentation after 12 months.

Hypertrichosis in PCT is well recognised, and may even be the presenting complaint.  The alopecia seems to be a progressive scarring disorder, starting as diffuse hair loss and spreading widely with sclerosis seen histologically.  Hair darkening has not previously been reported in PCT, although it may occur as a post-inflammatory change, after ionising radiation, certain drugs and in coeliac disease.  The mechanism of hair repigmentation is elusive; stem cells located in the outer root sheath may be triggered to repigment the hair with melanin given