Search

Recently, sunlight-induced damage of healthy human skin, including skin malignancies and a large scale of photodermatoses representing a diverse group of diseases, have increased in childhood as a result of unfavorable environmental changes. This article yields an overview of the diagnosis, the clinical features and the treatment of these conditions and disorders and also reveals perspectives. Some diseases are more frequent in the pediatric population than in adulthood, whereas others heal spontaneously during adolescence and vice versa. The majority of cases are idiopathic photodermatoses, mainly polymorphic light eruption. Photosensitivity may be an early symptom of genetic disorders, such as porphyria, or very rare genophotodermatoses. Photosensitivity, secondary to topical or systemic external agents as well as photoexacerbated dermatoses, is not so frequent in childhood. Effective photoprotection is crucial.

A simple disposable chemical UVB radiation dosimeter with silver-mercury-oxalate suspension, SUNTEST, was developed for use by the general public. Its spectral sensitivity curve closely matches the erythemal action spectrum. SUNTEST takes account of the variables influencing the length of sunbathing. The dose of solar UV radiation can be evaluated from the darkening of the dosimeter, from white to dark brown. On the basis of the results of a 5 min exposure, recommendations are given for safe exposure times (which do not produce sunburn) for different skin types. Regular use of this simple UVB dosimeter will contribute to the prevention of acute adverse effects of solar exposure; like any limitation of UV exposures, it may be expected to reduce also the late adverse effects, including skin malignancies.

Photosensitivity in childhood is caused by a diverse group of diseases. It usually indicates idiopathic photodermatoses, first of all polymorphic light eruption. It may be an early symptom of genetic disorders such as porphyria or very rare genophotodermatoses. Photosensitivity secondary to topical or systemic external agents as well as photoexacerbated dermatoses is not so frequent in childhood. Here we present our experience with childhood photosensitivity skin diseases collected over a 40-year period.

Long-term occupational exposure to formaldehyde (FA) increases the risk for nasopharyngeal squamous cell carcinoma. As the skin is also in contact with FA by environmental exposure, we tested the genotoxic properties of appropriate low concentrations (

Background: A failure in the apoptotic response after severe genomic damage could facilitate cell transformation and tumor development, and a constitutive overexpression of either p53 or bcl-2 protein in nonapoptotic tumor cells could signify a defective bax-mediated apoptosis. Objectives: To investigate whether a negative correlation occurs between these 2 proteins in nonmelanoma skin cancer and whether overexpression of either protein is associated with a low rate of spontaneous apoptosis. Design: Immunohistochemical study of nonmelanoma skin cancer archive material. Setting: University referral center. Patients: White patients with tumors on sun-exposed skin areas (ie, 17 basal cell carcinomas and 22 squamous cell carcinomas). Main Outcome Measures: Positivity for p53 and bcl-2 were scored semiquantitatively on 4 levels, and the percentages of apoptotic cells were determined. Results: A significant negative correlation between p53 and bcl-2 expression was found in the basal cell carcinomas, but not in the squamous cell carcinomas, largely attributable to the low level of bcl-2 staining in the squamous cell carcinomas. Squamous cell carcinomas have a significantly higher number of apoptotic cells than basal cell carcinomas: 1.1% vs 0.6%, respectively. This spontaneous apoptosis decreases with increasing bcl-2 (in basal cell carcinoma), whereas it does not appear to be related to p53 level expression. Conclusions: These results indicate that a disturbance in either p53 or bcl-2 suffices to enhance skin tumor formation by suppressing apoptosis; bcl-2 appears to reduce the rate of spontaneous apoptosis, but an aberrant p53 expression does not, and this factor may solely affect the apoptosis from exogenous genotoxicity. Arch Dermatol. 1997;133:599-602

It is known that some trace elements have an influence on the repair of UV light induced DNA damage. We have detected certain alterations in the excision repair of patients with photodermatoses. In these investigations of screening character the levels of zinc, copper, manganese, and iron were measured by means of atomic absorption spectrophotometry in the whole blood of 31 patients with polymorphic light eruption and 27 patients with cutaneous porphyrias. In active stage of polymorphic light eruption decreased zinc, copper, and iron concentrations and an increased manganese content were found. In remission only the zinc level was lower. In the active stage of cutaneous porphyrias a decreased zinc and iron content as well as an increased manganese level could be detected. A presumable connection between the findings and the rate of the excision repair is discussed.

The pathogenetic role of liver damage in photodermatoses induced by oral contraceptives was investigated. From among 121 cases with photodermatosis, it was in 4 cases of polymorphic light eruption-like dermatosis and in 2 cases of porphyria cutanea tarda that a longterm use of antibaby pills preceded the development of skin disease. Physical and biochemical studies of the liver of the patients suggested that the hepatotoxicity of the estrogen component of oral contraceptives can play a role not only in the pathomechanism of porphyric cases, but also in that of cases occurring with the clinical picture of polymorphic light eruption.

Chromatin-bound metal ions interact with enzymatic reactions involved in DNA repair processes. Content of Cu, Zn and Mn was studied in the chromatin of lymphocytes derived from patients with some light sensitive skin diseases, i.e. 26 with polymorphic light eruption and 10 with porphyria cultanea tarda. After isolating the lymphocytes by ficoll-urographin method and separating the chromatin by lysis of the cells, the trace elements studied were determined by neutron activation analysis. The Zn and Mn content in the chromatin of the patients did not differ from that of the controls. The copper concentration was significantly higher in the chromatin of the cells derived from patients with polymorphic light eruption, but not in cases with porphyria cutanea tarda. The results are discussed in connection with the UV-light induced excision repair.

Repair of UV-light induced DNA damage and changes in the semiconservative DNA synthesis were studied by in vitro autoradiography in the skin of patients with lightdermatoses (polymorphous light eruption, porphyria cutanea tarda, erythropoietic protoporphyria) and xeroderma pigmentosum as well as in that of healthy controls. In polymorphous light eruption the semiconservative DNA replication rate was more intensive in the area of the skin lesions and in the repeated phototest site, the excision repair synthesis appeared to be unaltered. In cutaneous porphyrias a decreased rate of the repair incorporation could be detected. Xeroderma pigmentosum was characterized by a strongly reduced repair synthesis.

Polymorphic light eruption (PLE) can be defined as a delayed abnormal response to sunlight. In this paper some features of the presumable immune response were studied in 55 patients with PLE and the results were compared with the findings available in 58 cases with porphyria cutanea tarda (PCT). The mean intracutaneous reactivity index measured by skin tests of delayed type was normal in both diseases. The number of the active and total E-rosette-forming peripheral lymphocytes was significantly lower in the active stage of PLE whereas in remission normal values were found. In active PCT only the number of the total E-rosette-forming cells was decreased. The percentage of lymphocytes with so-called dot-like α-naphthyl acetate esterase enzyme reaction was reduced significantly in the active stage of PLE whereas in remission their number was normal. Changes observed in PLE seem to be functional and suggest the involvement of T lymphocytes in some phases of the hypersensitive cutaneous reaction induced by sunlight.

Prostaglandin F2α (PGF2α) as a possible mediator was studied. Its plasma content was determined by radioimmunoassay. Changes in the DNA synthesis were followed by autoradiography. In active polymorphous light eruption (PLE) and porphyria cutanea tarda (PCT) a remarkable increase (over 300 pg/ml) in plasma content occurred, especially in cases involving large skin areas. Values returned to normal in remission. PGF2α administered i.d., significantly increased the DNA synthesis of the epidermal cells 48 h after injection similar to the effect of three minimal erythema dosis UV-irradiation. This was more pronounced in PLE patients than in controls. These findings suggest some role of PGF2α in producing the inflammatory and perhaps proliferative components of the skin symptoms in PLE. PGF2α — in parallel to literary data concerning PGE — seems to be a mediator of UV-induced changes in DNA synthesis of the epidermal cells.

The relationship between porphyria and liver damage was studied in 24 cases with porphyria cutanea tarda treated with Ippen's cure. In the history of 22 patients an excessive alcohol ingestion, in that of 2 other patients permanent medication with antibaby pills could be demonstrated. Conclusions for the condition of the liver parenchyma were derived from the serum activities of mitochondrial GLDH and lysosomal arylsulfatase. The degree of the mesenchymal reaction of the liver was estimated by the quantitative assay of serum immunoglobulins. On the basis of the results a liver damage involving both the parenchyma and mesenchyma was proved in each patient. The immunoglobulogram showed a considerable resemblance to that of 16 patients with chronic alcoholic hepatopathy. The IgG and IgA values tendentiously decreased after a successful Ippen's cure. It is probable that the products of pathological porphyrin metabolism contribute to the aggravation of the chronic inflammatory liver process. Presumably the serial phlebotomies influence the activity of the mesenchymal reaction of the liver favourably by means of reducing its porphyrin and iron depot. It appears that valuable information can be obtained about the nature and course of the liver damage also in PCT by the help of immunoglobulin and enzymatic assays.

Es wurden die Lymphozyten-Subpopulationen im Blut und im dermalen Infiltrat der Hautsymptome und des Lichttests bei der polymorphen Lichtdermatose (PLD) mit Hilfe der sauren α-Naphthylazetatesterase-Methode (ANAE) untersucht. Die Verteilung der Subpopulationen der ANAE-positiven T-Lymphozyten zeigt eine wesentliche Verschiebung im Blut in der aktiven Phase der PLD. Die Veranderungen sind wahrscheinlich von funktionellem Charakter. Das Hautinfiltrat beinhaltet uberwiegend T-Lymphozyten. Im Infiltrat des positiven Lichttests wird der T-Zellencharakter mit der Zeit immer deutlicher. Die Ergebnisse weisen auf eine wahrscheinliche Mitbeteiligung der T-Zellen in der durch Sonnenlicht induzierten hypersensitiven Hautreaktion hin.