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Biomedical subjects

D Kemmett

Publications and source records attributed to D Kemmett.

At least 19 recordsLinked to original sources

Subacute cutaneous lupus erythematosus and life-threatening hypokalaemic tetraparesis: a rare complication.

We describe a patient with lesions of subacute cutaneous lupus erythematosus associated with high titres of anti-Ro and anti-La antibodies who developed a rapidly progressive flaccid tetraparesis due to profound hypokalaemia. After investigation she was found to have distal renal tubular acidosis (dRTA) without pathological evidence of lupus nephritis. It is likely that her dRTA was a manifestation of associated Sjögren's syndrome, which had been otherwise asymptomatic. This is the first report of such a complication in the dermatological literature.

Acidosis, Renal Tubular↗

Hydroa vacciniforme: A clinical and follow-up study of 17 cases.

BACKGROUND: Hydroa vacciniforme (HV) is a rare, sporadic, idiopathic photodermatosis characterized by vesicles and crust formation after sunlight exposure. The lesions typically heal with vacciniform scarring. OBJECTIVE: We identify and review the clinical features and follow-up data of Scottish patients with HV and report on the prevalence of this condition. This is the largest recent study of HV patients from a single center. METHODS: In this retrospective study, patients with HV were identified by means of the diagnostic database from the Photobiology Unit, Dundee. Patients were contacted and details of clinical features, duration of disease, results of investigations, and treatment were recorded. At review, disease progress was assessed. RESULTS: Between 1973 and 1997, 17 patients (9 males and 8 females) with a diagnosis of HV were investigated. Data from 15 patients showed a mean age at onset of 7.9 years (range, 1 to 16 years), with females (mean, 6.7 years; range, 2 to 12 years) having an earlier onset than males (mean, 8.7 years; range, 1 to 16 years). A bimodal age distribution was also identified with onsets between the ages of 1 and 7 years and 12 and 16 years. At review, spontaneous clearing had occurred in 9 patients (60%) with mean duration of disease being 9 years (range, 4 to 17 years). Males had longer disease duration (mean, 11 years; range, 5 to 17 years) than females (mean, 5 years; range, 4 to 7 years). Eight patients (53%) were sensitive in the UVA wave-band on monochromator phototesting, and 6 (40%) experienced papulovesicular lesions on repetitive broad-spectrum UVA irradiation. All patients received broad-spectrum sunscreens with variable results. Of the 5 patients treated with narrow-band UVB (TL-01) phototherapy, 3 reported beneficial results with an increase in tolerance to sunlight exposure and associated reduction in disease severity. CONCLUSION: The estimated prevalence of HV was at least 0.34 cases per 100,000 with an approximately equal sex ratio. Males had a later onset and longer duration of disease than females. Phototesting showed abnormal responses in the UVA wavebands in 53% of cases, whereas 60% of patients treated with prophylactic TL-01 phototherapy found it beneficial.

Adolescent↗

Familial hydroa vacciniforme.

Hydroa vacciniforme is a rare, idiopathic photodermatosis with an onset in childhood and characterized by acute vesiculation, crusting and scarring following sun exposure. Familial cases are extremely rare with only one previous report. We report a brother and sister, both of whom have developed hydroa vacciniforme.

Child↗

Sweet's syndrome and malignancy in the U.K.

Acute febrile neutrophilic dermatosis (Sweet's syndrome) is reported to be a marker for underlying malignancy. Much of the evidence for this is based on case reports, small series of cases and reviews of the literature. In order to clarify the association with malignancy and determine the common clinical features of Sweet's syndrome, we reviewed the case notes of patients presenting to six dermatology units in the U.K. Eighty-seven cases of histologically proven Sweet's syndrome were reviewed. Fourteen patients (16%) developed associated malignancy, predominantly haematological, two patients (2%) had a history of previous malignancy and four patients (5%) had premalignant conditions (monoclonal gammopathy, two: myelodysplasia, two). Malignancy developed up to a year after presentation with Sweet's syndrome. Patients with associated malignancy were more likely to be anaemic (P < 0.01) at presentation, had a lower mean platelet count (207 x 10(9)/L vs. 332 x 10(9)/L; P < 0.003) and were, on average, older (59 years vs. 49 years; P = 0.002). Contrary to previous reports, a greater percentage of females developed malignancy than males.

Adolescent↗

Patch testing in discoid eczema.

We report a retrospective study of patch testing in patients with discoid eczema. 48 patients with persistent or severe discoid eczema were patch tested. The mean age of patients was 45 years and the median duration of symptoms was 6 months. 24 patients (50%) had positive patch tests, and 16 of these (33%) were considered to be clinically relevant. The most common allergens implicated were rubber chemicals, formaldehyde, neomycin, chrome, nickel (5, 2, 2, 2, 2, 2 reactions, respectively). 13 of 16 patients were followed up by telephone in 1996, and 8/13 (61%) stated they had benefited from patch testing. This study suggests allergic contact dermatitis is relatively common in persistent discoid eczema, and allergen avoidance may be of benefit. We recommend patch testing should be considered for all patients with severe or persistent discoid eczema.

Adolescent↗

The spectrum of nail involvement in palmoplantar pustulosis.

Abnormalities of the nail are not a well recognized feature of palmoplantar pustulosis (PPP). However, in a group of 50 patients with PPP, we found nail dystrophy in 15 (30%). The most frequent pattern was subungual pustulation, present in 10 patients, and progressing to nail destruction in two. Onycholysis and pitting of the nail were present in a few patients. In contrast to psoriasis vulgaris, the rate of linear nail growth in PPP was no greater than in matched normal controls. This is another clinical feature of PPP that separates it from psoriasis vulgaris.

Adult↗

Increased natural autoantibody activity to cytoskeleton proteins in sera from patients with necrobiosis lipoidica, with or without insulin-dependent diabetes mellitus.

Necrobiosis lipoidica (NL), a skin disease, is associated with insulin-dependent diabetes mellitus (IDDM). Natural autoantibody (NAb) activity in sera from 16 patients suffering from NL, with or without IDDM, was compared to that in sera from 41 patients with IDDM and 43 healthy controls. Isotype-specific enzyme-linked immunosorbent assays (ELISAs) were used to detect NAbs against actin, myosin, keratin, desmin, troponin, tropomyosin, thyroglobulin, insulin, single-stranded DNA and the hapten trinitrophenyl. NAb activity was significantly higher in sera from patients with NL (either with or without IDDM), compared with that detected in sera from patients with IDDM which was similar to that of healthy individuals. High proportion of NL sera exhibited increased IgG anti-tropomyosin (69%), anti-troponin, anti-desmin and anti-keratin (50% each), anti-insulin (44%) and anti-trinitrophenyl (31%) activities, as well as increased IgA and IgM anti-keratin activities (26% and 31%, respectively). The great majority (88%) of positive sera were polyreactive and contained NAbs, polyspecific and monospecific (as demonstrated by immunoadsorption studies), belonging to more than one isotype; there was no predominant serological reactivity pattern. In conclusion, increased NAb activity to cytoskeleton proteins is associated with the dermatological disease NL and not to the overlapping autoimmune disease (IDDM). The origin and significance of these NAbs is discussed.

Adult↗

Myxoid cysts treated by infra-red coagulation.

The infra-red contact coagulator was applied to the treatment of myxoid cysts. The area beneath and around each lesion was infiltrated with local anaesthetic before coagulating the cyst with overlapping pulses. Initially four cysts were punctured and emptied of mucin but coagulation was delayed for 7 days. In this group there were two recurrences. The protocol was then changed so that coagulation was performed immediately after emptying the cyst. Fourteen cysts were treated in this way and there were two recurrences. The technique was well tolerated and local complications arose in five individuals.

Adult↗

Antibodies to neutrophil cytoplasmic antigens: serologic marker for Sweet's syndrome.

Seven patients with a clinical and histologic diagnosis of Sweet's syndrome were tested for the presence of circulating antibodies to neutrophil cytoplasmic antibodies. Six of the seven patients had detectable antibodies to neutrophil cytoplasmic antibodies at a serum dilution of at least 1:20. Antibodies to neutrophil cytoplasmic antibodies were not found in serum from patients with a range of cutaneous diseases, some known to cause clinical or histologic confusion with Sweet's syndrome. The detection of circulating antibodies to the neutrophil cytoplasm may be of possible diagnostic value in Sweet's syndrome.

Adult↗

The influence of the menstrual cycle and pregnancy on atopic dermatitis.

The prevalence of a premenstrual deterioration in the symptoms of atopic dermatitis, as determined by a postal questionnaire completed by 150 women, was 33%. There was a significant association between a premenstrual worsening of atopic dermatitis and the presence of the symptoms of the premenstrual syndrome (P less than 0.002). Pregnancy had an adverse effect on atopic dermatitis in the majority of cases (52%), usually starting in the first 20 weeks of gestation, although an appreciable proportion of women (24%) had improved during their pregnancy.

Adolescent↗

Sweet's syndrome: a clinicopathologic review of twenty-nine cases.

Twenty-nine patients with Sweet's syndrome were studied. Not all of Sweet's original criteria were necessarily present and diagnosis was dependent on the recognition of the typical, acute, tender, erythematous plaques and the characteristic histologic features of a neutrophilic infiltrate with leukocytoclasis. Women are affected much more frequently than men. The origin of Sweet's syndrome is still unclear, but an underlying disease was found in more than 50% of our cases. A streptococcal infection was evident in six cases, inflammatory bowel disease in three cases, malignancy in four cases, and pregnancy in two others. Treatment with oral prednisolone for an average of 6 weeks was the usual treatment, although in four patients the disease cleared spontaneously. Resolution of the eruption is occasionally followed by milia and scarring. Recurrences are common and affect up to one third of patients.

Adult↗