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Biomedical subjects

J L Hawk

Publications and source records attributed to J L Hawk.

At least 127 records · Page 7Linked to original sources

Polymorphic light eruption sine eruption.

We describe seven patients, four female and three male, who developed intense pruritus on sun-exposed skin without visible change. The clinical features resembled those of polymorphic light eruption (PLE) without rash. Four patients also occasionally developed typical PLE upon sun exposure, but sun-induced pruritus alone occurred most frequently. No patient was taking any drug therapy. One patient developed similar pruritus following solar simulated irradiation, and one following PUVA therapy. All other laboratory investigations were negative. Treatment with low dose UVB phototherapy or PUVA therapy was effective. The condition, which we have called polymorphic light eruption sine eruptione (PLESE), appears to be a variant of PLE not previously reported.

Adolescent↗

Hydroa vacciniforme: a review of ten cases.

Ten patients with hydroa vacciniforme are reported. The condition appears to be a distinct clinical and histological entity. Haematological, biochemical, immunological, bacteriological and viral investigations were negative. Three patients demonstrated low minimal erythema doses (MEDs) following monochromatic ultraviolet (UVA) irradiation of back skin; such UVA sensitivity may be a feature of hydroa vacciniforme. Treatment was unsatisfactory, although spontaneous improvement in the condition tended to occur and regular application of sunscreens with high protection factors against both UVA and UVB was helpful.

Adolescent↗

The role of psoralen photochemotherapy (PUVA) in the treatment of severe atopic eczema in adolescents.

Fifteen adolescent children with severe, persistent atopic eczema were treated with oral psoralen photochemotherapy (PUVA). This resulted in initial clearance of eczema in 14 of the 15 children, nine of whom achieved a remission. Apart from its effectiveness, a major benefit of this therapeutic approach was that it was associated with resumption of normal growth in children who were previously growing poorly, either as a direct result of severe eczema or its treatment. Nevertheless, against the considerable advantages of PUVA for this group of patients have to be balanced the possible hazards, because relatively high exposures are required in some individuals, both initially to induce clearance and subsequently to maintain it.

Adolescent↗

A histological study of the evolution of solar urticaria.

The histological evolution of solar simulator-induced lesions of solar urticaria was investigated in four severely affected white patients. A series of two to 32 minimal whealing doses of radiation, each much lower than the 24-hour minimal erythema dose, was administered to separate buttock sites. Biopsy specimens were obtained from the exposed areas at five minutes and two and 24 hours later, as well as from adjacent nonexposed skin. Lesions showed a statistically significant dose-dependent increase, predominantly perivascular, in upper dermal neutrophil and eosinophil numbers at five minutes and two hours, but not at 24 hours, and at higher radiation doses in mononuclear cell numbers by 24 hours. Nonirradiated patient skin and irradiated control subject skin was not similarly affected. These changes may be associated with the pathogenesis of solar urticaria.

Adult↗

Xeroderma pigmentosum complementation group G--report of two cases.

Genetic complementation studies allowed assignment of a brother (XP124LO) and sister (XP 125LO), aged 14 and 12 years respectively, to the rare complementation group of classical xeroderma pigmentosum (XP), XP-G. Both patients manifested only mild cutaneous changes, with no UV-induced skin tumours, although abnormal sensitivity to UVB wavelengths was demonstrated by irradiation monochromator skin testing. Physical and neurological development was normal. Measurement of UV-induced unscheduled DNA synthesis in cultured fibroblasts showed reduction of repair synthesis to 14% and 16% of normal in XP124LO and XP125LO, respectively. This contrasts with a reduction to 5% of normal in previously described group G patients, XP2BI and XP3BR, who had correspondingly severe cutaneous and neurological manifestations.

Adolescent↗

Hydroxychloroquine in polymorphic light eruption: a controlled trial with drug and visual sensitivity monitoring.

A double-blind controlled trial of oral hydroxychloroquine (HC) treatment in polymorphic light eruption (PLE) was completed in 13 patients on active treatment and 15 on placebo during June, July and August 1982. HC dose was 400 mg daily for the first month and 200 mg daily thereafter. Exposure to ambient solar ultraviolet radiation (UVR) was monitored throughout the trial by polysulphone film lapel badges. Patients scored their symptoms on a visual analogue scale. Drug concentration was monitored in plasma and hair, and oculotoxicity was assessed by visual contrast sensitivity. Moderate clinical improvement occurred, associated with a statistically significant improvement in skin rash (P less than 0.01).

Adult↗

Prophylactic PUVA and UVB therapy in polymorphic light eruption--a controlled trial.

A double-blind controlled trial of low-dose prophylactic oral psoralen photochemotherapy (PUVA) and ultraviolet-B (UVB) irradiation therapy was undertaken from April to September 1983 in 42 patients with polymorphic light eruption (PLE). Patients were randomly allocated to three groups, PUVA with oral 8-methoxypsoralen (8-MOP), UVB with oral placebo, and control low-dose UVA with oral placebo. The initial dose given to each active treatment group was a third of the predetermined minimal phototoxic or erythema dose, followed three times weekly for 6 weeks by doses incremented by an eighth on each occasion in the PUVA group and by a seventh in the UVB group. Ultraviolet radiation exposure was monitored throughout with polysulphone film lapel badges. Patients recorded their symptoms on a visual analogue scale. Symptoms of rash and itch in patients treated with PUVA and UVB were significantly less affected by increasing exposure to ultraviolet radiation than were these symptoms in control patients.

Clinical Trials as Topic↗

Immunologic differentiation of the Sézary syndrome due to cutaneous T-cell lymphoma and chronic actinic dermatitis.

Peripheral blood mononuclear cells from two well-defined groups of patients with the Sézary syndrome have been studied employing indirect immunofluorescent and indirect immunogold techniques in light and electron microscopy, using monoclonal antibodies against T-cell subpopulations. Four patients had chronic actinic dermatitis (CAD) of the actinic reticuloid variant, with erythroderma. Eight patients had cutaneous T-cell lymphoma. All patients showed the clinical features of the Sézary syndrome, including erythroderma, palmoplantar hyperkeratosis, and peripheral lymphadenopathy, and in all patients significant numbers (0.5-30.5 X 10(9) cells/liter) of circulating mononuclear cells were observed with Sézary cell morphology on light-microscopic examination of blood films. Major differences were observed in the circulating T-cell subpopulations in the two groups. In the erythrodermic CAD patients, there was a moderately elevated T-cell count (1.6 +/- 0.6 X 10(9) cells/liter; normal, 1.0 +/- 0.3 X 10(9) cells/liter) of which the majority of the cells was suppressor T cells (OKT8+) giving a very low helper:suppressor T-cell ratio of 0.1:1-0.36:1 (normal, 1.7:1-3.5:1). In cutaneous T-cell lymphoma, there was also an elevation of the T-cell count (9.5 +/- 12.9 X 10(9) cells/liter), but in these patients the predominant cell was the helper T cell (OKT4+) with a high helper:suppressor T-cell ratio of 3.7:1-98:1.

Antibodies, Monoclonal↗

Sunscreens.

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Humans↗

Spontaneous photo-onycholysis.

Three cases of onycholysis following, and apparently caused by, sun exposure are reported. No case was associated with drug ingestion or demonstrable metabolic abnormality. Cutaneous phototesting of all patients with an irradiation monochromator was within normal limits, although the action spectrum for the defect may lie in the ultraviolet-A (UV-A) region. This appears to be the first report of spontaneous photo-onycholysis.

Adult↗

Late-onset erythropoietic protoporphyria with unusual cutaneous features.

A 68-year-old man with apparently light-exacerbated erythematous cutaneous plaques on his face and on the dorsa of his hands was found to have late-onset erythropoietic protoporphyria, diabetes mellitus, and hyperlipidemia. Extensive deposits of material that stained with periodic acid-Schiff were present in the lesional dermis. Monospecific antibody studies showed this material to be mainly type IV collagen. These findings strongly suggest that the lesions are a manifestation of erythropoietic protoporphyria. The late onset and asymptomatic unusual cutaneous lesions appear to be a new presentation of the disease.

Aged↗

Treatment of chronic actinic dermatitis with azathioprine.

Fourteen patients with severe unremitting chronic actinic dermatitis were treated with oral azathioprine in a dosage of 100-200 mg daily for a mean 11.5 months. Clinical improvement, apparently permanent, occurred after a few weeks to several months and continued for up to 2 years. Nine patients cleared or improved markedly, one patient cleared and then relapsed again on treatment, two patients showed no response and two patients needed to discontinue therapy because of gastrointestinal side-effects. We feel that in many patients azathioprine can be a very effective therapy for this extremely incapacitating disease.

Aged↗