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Incontinentia Pigmenti (Bloch-Sulzberger Syndrome) Manifesting as Painful Periungual and Subungual TumoursFrom the Departments of Plastic Surgery and Dermatology, Frenchay Hospital, Bristol, UK Correspondence: P. L. G. Townsend FRCS, Department of Plastic Surgery, Frenchay Hospital, Bristol, UK. 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.
Journal of Hand Surgery (British and European Volume), Vol. 18, No. 5,
667-669 (1993) |
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