A 3-month-old male infant was referred to our department with a generalized
brown, thickened leathery skin and blisters which had been present since b
irth; the blisters developed recurrently at sites of minor trauma, healing
without scar formation, although Darier's sign was positive. Microscopicall
y, biopsy specimens showed a dense dermal band-like infiltrate with mast ce
lls, whereas a biopsy from a vesicle showed subepidermal bulla formation. P
hysical examination did not reveal any systemic involvement with mastocytos
is and the patient was treated with cyproheptadine, which has considerable
anti-serotonin activity and, interestingly, reduced the degree of blisterin
g; this treatment might therefore be tried in other cases.