N.H. Cox

An acantholytic dyskeratotic epidermal naevus with other features of Darier's disease on the same side of the body.

Summary Many epidermal naevi with the histology of Dariers disease have been reported. In the absence of associated features of Dariers disease, they cannot be assumed to have a common pathogenesis with it, and it has been suggested that they are better classified as acantholytic dyskeratotic epidermal naevi rather than naevoid Dariers disease. We describe a patient with such a naevus who had typical nail and palmar changes of Dariers disease on the same side of the body. We suggest that in at least some cases the naevus has the same genetic defect(s) as generalized Dariers disease, and discuss the possibility that a patient with such a naevus could occasionally transmit Dariers disease to an offspring.

The relationship between chronological age and the erythemal response to ultraviolet B radiation

To investigate whether erythemal responses to ultraviolet radiation alter with age, we have reviewed the results of monochromator phototesting in adults and children, and have measured the dose–response curves for UVB erythema in a further 38 subjects. There was no significant difference between adults and children in minimal erythema dose (MED) at 300 nm; the median MED in 254 adults was 34 mJ/cm2 (range 14–80 mJ/cm2) and in 24 children aged less than 15 years was 30 mJ/cm2 (range 10–80 mJ/cm2). Objective measurements of UVB‐induced erythema were performed in 15 subjects aged below 25 years and 23 subjects aged above 60 years. A dose–response curve for UVB erythema was constructed for each subject and the slope of the steepest part of each curve calculated by logit regression; the values in the young subjects were greater than in the older group (P<0.02). However, there was no difference between the two groups in either the visually assessed MED or the calculated UVB dose required to produce a constant degree of mild erythema.

The relationship between plasma psoralen concentration and psoralen-UVA erythema

The plasma 8‐methoxypsoralen (8‐MOP) concentration was measured in 60 patients commencing psoralen photochemotherapy (PUVA). At the time of blood sampling each patient was phototested using a series of 10 exposures to UVA. The resulting erythema was measured objectively 72 h after irradiation and dose‐response curves for psoralen‐UVA erythema were constructed. Although the dose of 8‐MOP was calculated according to body weight, patients receiving 30 mg of 8‐MOP had a significantly lower mean plasma concentration than those receiving higher doses. There was no significant correlation between plasma 8‐MOP concentration and minimal phototoxic dose, either estimated visually or calculated from the dose‐response curves. However the slope of the dose‐response curve showed significant correlation with plasma 8‐MOP concentration. The variation between patients in the rate of increase of the erythemal response, but not the variation in threshold sensitivity, can be explained by differences in plasma psoralen concentration.

Woody hands in a patient with pancreatic carcinoma : a variant of cancer-associated fasciitis-panniculitis syndrome

We report an elderly woman with rapidly progressive painless, woody induration ofthe hands. Mild diabetes mellitus was demonstrated. Skin biopsy features included broad fibrous bands extending deeply into subcutaneous fat, a mild mononuclear cell infiltrate, and post‐thrombolic recanalization of a deep vessel in one specimen. The patietU developed uncontrolled haematemcsis and was demonstrated at laparotomy to have disseminated pancreatic carcinoma. The unusual clinical features and lemporal relationship between the skin changes and the tumour suggesi a paraneo‐plastic eruption, whieh appears besl classified as an example of cancer‐associated fasciitis—panniculitis syndrome.

Induction of lesions of dermatitis herpetiformis by autologous serum

Summary In the present study various factors which contribute to the initiation of lesions in dermatitis herpetiformis (DH) were examined. Thirty‐one patients with DH. seven with bullous pemphigoid. two with linear IgA disease and two healthy subjects were studied either before starting treatment or after stopping dapsone for up to 5 days. Intradermal inoculation of freshly prepared autologous serum was followed after 18–24 h by the formation of DH‐like lesions in 24/31 DH patients. The lesions were erythematous papules, often with vesicles and microscopically showed papillary tip microabscesses. Serum‐induced formation of lesions only occurred in patients with active DH with some spontaneous lesion formation; it did not occur in any of the non‐DH controls. The formation of lesions was dose‐related, declining proportionately with dilution of the serum down to 1/16. Plasma prepared by various methods of anticoagulation (heparin. citrate. EDTA) caused lesser reactions, while addition of heparin or ε‐amino caproic acid (EACA), but not citrate, to serum substantially inhibited the formation of lesions. This suggested the responsible factor might be a protease. Other vasoactive agents including histamine (1–4 μg) and compound 48/80 (1–5 μg) caused normal immediate wealing. DH‐like lesions occurred in only one of 13 subjects challenged with histamine and two of nine challenged with 48/80. In all these, autologous serum elicited large vesicular responses. There is a factor(s) in serum in DH which can initiate the formation of lesions. This factor appears to be activated by clotting and can be inhibited by heparin and EACA, suggesting it may be a protease.

Amenorrhoea during treatment with isotretinoin.

neoplasia, and invasion could not be excluded. Marked koilocytosis was present, but techniques of in situ hybridization to detect HPV DNA were not available. On clinical and histological grounds the diagnosis of penile intra-epithelial neoplasia perhaps with a squamous cell carcinoma, rather than of a verrucous carcinoma, was made and the lesion was, therefore, treated with radiotherapy on the advice of the urologist. Two years later the patient remained well with a soundly healed penis showing only signs of LSA. Perhaps one might add other questions to that of Beljaards et al. Could penile horns and PMKB be related to each other and to LSA, could they all indeed be aspects of LSA, and is HPV concerned in the development of carcinoma in these patients, whether of verrucous, intraepithelial or invasive squamous type?

Effect of isotretinoin on eccrine gland function

Sweat secretion rate, stimulated by iontophoresis of pilocarpine, was measured before and during treatment with isotretinoin in 20 patients. Sweat secretion was increased during treatment in 15 patients (75%), but the mean increase of 15% was not statistically significant (P = 0.085, paired t‐test; 95% confidence limits—0.2% to +30%) and no patients had a clinically apparent alteration of sweating. We conclude that there is no important change in stimulated sweating associated with isotretinoin therapy.