Incontinentia pigmenti (Bloch-Sulzberger syndrome) manifesting as painful periungual and subungual tumours.
A 25-year-old lady presented with painful periungual tumours which were associated with lytic changes of underlying terminal phalanges. She had been previously treated as a case of chronic paronychia but the problem recurred. She was subsequently diagnosed as a case of Incontinentia pigmenti which is a rare genodermatosis with mixed ectodermal and mesodermal polydysplasias, onchychodystrophy being a very rare manifestation. She was treated by excision of painful finger lesions, but there is no specific treatment for the underlying condition.
Adeniran
A
A
Department of Plastic Surgery, Frenchay Hospital, Bristol, UK.
Townsend
P L
PL
Peachey
R D
RD
D000328:Adult; D005260:Female; D006801:Humans; D007184:Incontinentia Pigmenti / Q000150:complications*; D007636:Keratoacanthoma / Q000209:etiology* / Q000601:surgery; D009262:Nails / Q000601:surgery*; D012871:Skin Diseases / Q000209:etiology* / Q000601:surgery