Case reports 49

FIGURE 2. Linear atrophic lesions on the thighs.

operatively at theageof 3 years). There is bilateral syndactyly of the fourth and fifth toes. Mostofthe nails are hypoplastic.

The skin shows the characteristic linear atrophic slightly yellowish lesions mainly on thebody, thighs, arms and face with prominent telangiectasia. Oval atrophic areas, especially on theskin of the thighs, with fat herniation are seen. The scalp hair was thin and brittle but hasimproved lately.

Comment. This brave, cheerful girl has coped with life and school very well. She attendednormal school until the age of 15. Since 1984 she has been at the special residential school, ExhallGrange in Coventry. She uses a variety of visual aids, including low visual aid glasses and atelescope. She is active and a keen swimmer. The papillomata on the upper lip were treated withcryosurgery in stages at the age of 7 years with a good cosmetic result.

(4) Incontinentia pigmenti with associated nail changes

D R J.A.COTTERILL

The General Infirmary at Leeds

R.C, female, aged 11 years.History. This patient was born with a bullous eruption which subsequently evolved through a

warty stage into the classical whorls of incontinentia pigmenti. She has other associated features.

Case reports

FIGURE I. Discolouration of the distal two-thirds of the nail plates.

including an area of scarring alopecia on the scalp, and peg teeth. Her mother has similar teeth, asmall area of alopecia and a pigmented whorl in one groin.

Examination. The patient shows a red/brown discolouration under the distal two-thirds ofmost of the nail plates of the fingers. Other classical features of incontinentia pigmenti are alsopresent.

Comment. Although nail changes have been described in incontinentia pigmenti,^ the changesseen in this patient appear unique and in particular there were no bony changes seen on X-ray inthe distal phalanges and no evidence of impaired renal or hepatic function.

REFERENCE

I Simmons DA, Kegel MS, Scher RK,Hines YC. Subungual tumours in incontinentia pigmenti.122: 1431.

1986;

(5) Raindrop pigmentary naevus

DR S.A.KHAN

Pinderfields General Hospital

J.G., female, aged 20 years.History. This patient's mother noticed pigmentation appearing in the groins when the patient

was 2 years old. Subsequently, areas of pigmentation gradually appeared over most of the trunkand patches on the limbs. Her hair was very thin and the finger and toe nails showed thinning,splitting and early pterygium formation. The raindrop pigmentation increased in intensity and