17 Dr R P Warin: This is a good example of reticulohistiocytosis. In 1957, with Dr C D Evans, Dr M Hewitt and others (British Medical Journal i, 1387) I reported 4 patients with this condition and reviewed the literature which at that time included 12 other cases. Two of our patients died, one with a fibrinous pericarditis, and the other of secondary carcinoma, probably bronchial in origin. The other 2 improved. Histochemical investigations at that time suggested that the granular cytoplasm contained a polypeptide-lipoid complex. Dr R Summerly: I am looking after a woman aged 66 whose lesions consist solely of the sheeted papular elements demonstrated so well in Dr Hall-Smith's patient. My patient has no clinical or radiological evidence of joint involvement and her skin lesions seem to be regressing. Thus she seems to demonstrate a benign end of the spectrum of multicentric histiocytosis, but will a disabling arthropathy yet supervene? Dr P Hall-Smith: Cases of reticulohistiocytosis without arthritis have been reported but it is thought that these may be a forme fruste of the disease. Orkin et al. (1964) reported 100% joint involvement in the 23 cases they reviewed.

Section of Dermatology

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Focal Dermal Hypoplasia (Goltz's Syndrome) R A Marsden MRCP and R H Lindenbaum MRCP (The Slade Hospital, Headington, Oxford, 0X3 7JHand Department of Medical Genetics, Oxford)

Girl aged 10 months Clinical features: Pitted lesions arranged in clusters were noted at birth on the skin of the -face (Fig 1), trunk and limbs. Recently these have become paler on the trunk and hyperpigmented on the legs. A 1.5 x 1 cm area of denuded skin was present on the occiput, and healed with scarring. Two nodules on the left thumb and perianal verrucous lesions have not changed since birth. Telangiectasia is present on the cheeks. The nails are poorly developed and some are absent. Many of the teeth show notching of their free margins. Partial soft tissue syndactyly involves left middle and ring fingers, and left third and fourth toes (Fig 2); the right foot shows clefting between fourth and fifth metatarsals (Fig 3). This unusual combination of syndactyly and clefting is characteristic of Goltz's syndrome. Birth weight was unremarkable (3330 g), but height, weight and head circumference have now fallen to the lower end Qf the normal range for age. No skeletal or eye defects have been detected, and mental development appears normal.

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Family history: No parental consanguinity; no affected relatives are known. The father was aged 29 and mother 25 at the birth of the affected child. The mother has borne one normal son (half-brother to the affected child), and is now pregnant for the third time.

Dr W A D Griffiths: A young man came to have his piles excised at St Thomas' Hospital and the bits were sent for histopathology. The report was of simple papillomas, but on referral Dr H J Wallace made the correct diagnosis of focal dermal hypoplasia. It was interesting to note that this male case was particularly severely affected. He walked with crutches because of the bony defects and had warty polyps in the groins and perineum in addition to the usual stigmata of this disease.

Comment A great variety of mesodermal and ectodermal defects may be found in focal dermal hypoplasia (Goltz et al. 1970). Nearly all reported cases are female and arise sporadically with no history of parental consanguinity. In familial cases all affected relatives are on the maternal side. Of 29 recorded pregnancies in affected females 8 aborted spontaneously; the others produced 7 males (1 affected, 6 normal) and 13 females (8 affected, 5 normal). Another child, whose sex is not specified, was also normal. No unaffected female has had more than one affected child. There are no reports of affected males repro-

ducing. Available data suggest that the syndrome is probably an X-linked dominant trait and that males are more severely affected than females, most male fetuses aborting spontaneously. Sporadic cases would arise by mutation on a parental X chromosome. Other theories suggest the syndrome is an autosomal dominant trait with limitation to females, or that it results from a cytoplasmic factor lethal to males. Alternatively an X-autosome translocation or environmental teratogens might be responsible (Walbaum et.al. 1970). Further information on offspring of affected females (and males) is required before the exact mode of inheritance can be determined. Increased paternal age is associated with an increased frequency of mutation to genes having certain harmful dominant traits (Jones et al. 1975). We found no evidence for this in relation to sporadic female cases of Goltz's syndrome (mean paternal age 25.8 years, mean maternal age 23.9 years), but age data were lacking in most

The following cases were also shown: Reticular Erythematous Mucinosis Dr G Archibald (for Dr H T Calvert)

Poikiloderma Atrophicans Vasculare Dr R A Marsden and Dr H R Vickers Epidermolysis Bullosa Dystrophica et Albopapuloidea Dr R A Marsden and Dr R I Van Hegan Exfoliative Dermatitis in Down's Syndrome Dr R Clayton (for Dr L Fry) Subcorneal Pustulosis and Myeloma Dr J J Cream

Cutaneous Leishmaniasis Dr A du Vivier (for Dr M Feiwel)

Sclerosing Lipogranuloma of Penis Dr D I Vollum Tuberculoid Granuloma of Nose Dr Robin Marks (for Professor M W Greaves)

reports.

Postscript (March 1976): The mother's third pregnancy resulted in the birth of an unaffected boy. REFERENCES

Goltz R W, Henderson R R, Hitch J M & Ott J E (1970) Archives of Dermatology 101, 1 Jones K L, Smith D W, Harvey M A S, Hall B D & Quan L (1975) Journal ofPediatrics 86, 84 Walbaum R, Samaille G & Dehaene Ph (1970) Pddiatrie 25, 911

Vinyl Chloride Disease Dr J M Gartside (for Dr E N M Johnston)

Vinyl Chloride Disease Dr C Harrington (for Dr A E Walker) Familial Self-healing Squamous Cell Carcinoma Dr C J Guerrier