Cutaneous manifestations of fucosidosis.

Angiokeratoma corporis diffusum (ACD) is still often thought to be synonymous with Anderson-Fabry disease, a deficiency of alpha-galactosidase. It is important, however, to consider other possible enzyme deficiencies in patients with ACD. We report an 8-year-old boy with neurodevelopmental delay who was diagnosed as having fucosidosis following recognition of ACD in the dermatology department. Other cutaneous features in this patient included distal transverse purple nail bands, acrocyanosis and a naevus anaemicus. Histology and electron microscopy of skin papules was consistent with angiokeratoma. Skeletal survey demonstrated dysostosis multiplex. The diagnosis was confirmed by leucocyte oligosaccharide enzyme analysis. There are only three previous reports of fucosidosis in the U.K.

Assessment of an anti-T-cell receptor variable region antibody panel in cutaneous T-cell lymphoma.

Using a panel of seven commercially available monoclonal antibodies directed against specific sites of the T-cell receptor variable region, we have studied the staining patterns in 15 biopsies from 11 patients with cutaneous T-cell lymphoma (CTCL), and in five patients with benign lymphocytic infiltrates. Eight of the 11 patients with CTCL showed staining of the majority of lymphocytes with either V beta 8 alone (n = 3), V beta 12 alone (n = 2) or with both V beta 8 and V beta 12 (n = 3). None of the biopsies from patients with benign dermatoses showed staining of the majority of lymphocytes with any of the antibodies.

Epidermolysis bullosa acquisita occurring in association with systemic lupus erythematosus.

A 77-year-old retired male physician with a 6-year history of systemic lupus erythematosus (SLE) developed a mechanobullous eruption, the features of which were clinically and immunopathologically consistent with a diagnosis of 'classical' epidermolysis bullosa acquisita (EBA). As EBA shares immunopathological findings with a number of cases reported as the 'bullous eruption of SLE', the clinical findings commonly recognized as 'classical EBA' may, in patients with SLE, represent a specific subset of the bullous eruption of SLE rather than a separate diagnostic entity. There are few reports in the literature describing classical EBA in patients with SLE. Findings in this patient add further support to the suggestion that EBA occurring in association with SLE, represents a subset of the bullous eruption of SLE, the clinical features of which may be modified by genetic susceptibility or disease activity.

Pyoderma faciale in a patient with Crohn's disease.

We wish to report the progress of a patient with pyoderma faciale and Crohn's disease. The patient is interesting in that on two occasions the relapse in her skin condition coincided with the introduction of non-steroidal anti-inflammatory drugs. Therapy with isotretinoin was effective and well tolerated.

Effect of maintenance chemotherapy in childhood on numbers of melanocytic naevi.

OBJECTIVE: (a) To determine whether children given chemotherapy for haematological malignancy have significantly more melanocytic naevi than age matched children in the local population; (b) to establish whether any observed variation in naevus counts from normal is seen at the start of maintenance chemotherapy. DESIGN: Follow up of 29 consecutive children starting maintenance chemotherapy, with parental interview and count of all melanocytic naevi > or = 2 mm on the child's skin. Assessment repeated three years later after completion of maintenance chemotherapy. Other dermatological problems identified at either visit were also recorded. SETTING: Royal Hospital for Sick Children, Glasgow. RESULTS: At the start of maintenance chemotherapy all children had total body counts of melanocytic naevi within the normal range established for age matched children in the local population. Three years later total body naevus counts were significantly increased, the median increase being 66 naevi per child (95% confidence interval 57 to 94). The only other problem noted in these children was relatively poor regrowth of scalp hair. CONCLUSION: Children on maintenance chemotherapy for haematological malignancies develop an excessive number of melanocytic naevi. Excessive numbers of melanocytic naevi are the most important risk factor for melanoma in the general population. These children should have periodic skin examinations at their follow up visits, and both child and parent should be educated about clinical features of early melanoma.

Dynamic hepatic scintigraphy in the screening of psoriatic patients for methotrexate-induced hepatotoxicity.

We report the use of dynamic hepatic scintigraphy in the assessment of the hepatic status of psoriatic patients before and during methotrexate therapy. Eighty-seven paired dynamic scans and percutaneous liver biopsies were performed in 63 patients. The liver biopsies were graded according to Warin et al. with fibrosis of grade 2 or worse being a strong indication for withdrawal of methotrexate. The sensitivity of dynamic hepatic scintigraphy in detecting fibrosis of grade 2 or worse was 83.3% and the specificity was 81.5%. The predictive value of a normal scan for fibrosis of grade 0-1 was high (98.5%) although the predictive value of an abnormal scan for fibrosis of grade 2 or worse was low (25%). Dynamic hepatic scintigraphy may therefore offer a means to reduce the number of liver biopsies necessary in patients receiving methotrexate for psoriasis.

Psoriasis: an audit of patients' views on the disease and its treatment.

This audit aimed to establish the effect of out-patient clinic attendance on patients' attitudes to their psoriasis, its severity and its treatment. A secondary aim was to determine if there was any correlation between the patients subjective concept of disease severity and objective medical assessment. The audit was carried out by means of paired questionnaires completed by patients at their first visit to the out-patient clinic and at a review visit 3 months later. On each of these occasions an objective assessment of psoriasis severity score was made by the dermatologist. We were able to confirm previous reports of major psychosocial effects of psoriasis on the life-style of many patients. At their first visit 67% (30/45) of patients stated that their life-style was affected by psoriasis, while at review 3 months later this figure was 40% (18/45). Overall 76% (34/45) of patients felt their psoriasis had improved following attendance at the clinic, while 11% (5/45) of patients felt it had deteriorated over the 3-month period. However, all patients stated they found attendance at the out-patient clinic to be of benefit. There was a significant correlation between the psoriasis severity score by the patient and by the dermatologist (P less than 0.001).

Melanoma in people aged 65 and over in Scotland, 1979-89.

OBJECTIVE: Detailed analysis of primary cutaneous melanoma first diagnosed in Scotland in patients aged 65 and over. DESIGN: Comparison of changing incidence, sex distribution, site, histogenetic type, tumour thickness, and prognosis of all primary cutaneous melanomas in patients aged 65 and over diagnosed in Scotland in the 11 years 1979-89 with similar data for patients aged under 65. SETTING: Data were obtained from the Scottish Melanoma Group's database, established in 1979, which aims to record detailed clinical, pathological, and surgical follow up details of all primary cutaneous melanomas registered in Scotland. PATIENTS: 1430 patients (954 women, 476 men) aged 65 and over; comprising over a third of the 3903 patients with primary melanoma recorded for all age groups in Scotland during this period. RESULTS: The overall incidence of melanoma in patients aged 65 and over increased from 12.2/100,000 in 1979 to 20.7/100,000 in 1989, with the greatest increase seen in older men, from 7.8/100,000 in 1979 to 18.0/100,000 in 1989. The site most commonly affected was the face in both men and women (33% of all tumours). The most common histogenetic type was superficial spreading melanoma. 526 patients (37%) had melanomas with a tumour thickness of 3.5 mm or greater in the older age group, compared with 453 patients (18%) in those aged under 65. The highest proportion of thick tumours was seen in older men. Five year survival figures for 616 patients diagnosed between 1979 and 1984 were 88%, 66%, and 47% for thin, intermediate, and thick tumours respectively. Overall five year survival for the older age group was 64% compared with 78% for the younger age group. CONCLUSION: The increase in melanoma in the elderly and the high proportion of thick tumours, especially in men, require a specific educational programme for both primary and secondary prevention directed towards the older population.