Interrater agreement of classification of photoparoxysmal electroencephalographic response.

Our goal was to assess the interrater agreement (IRA) of photoparoxysmal response (PPR) using the classification proposed by a task force of the International League Against Epilepsy (ILAE), and a simplified classification system proposed by our group. In addition, we evaluated IRA of epileptiform discharges (EDs) and the diagnostic significance of the electroencephalographic (EEG) abnormalities. We used EEG recordings from the European Reference Network (EpiCARE) and Standardized Computer-based Organized Reporting of EEG (SCORE). Six raters independently scored EEG recordings from 30 patients. We calculated the agreement coefficient (AC) for each feature. IRA of PPR using the classification proposed by the ILAE task force was only fair (AC = 0.38). This improved to a moderate agreement by using the simplified classification (AC = 0.56; P = .004). IRA of EDs was almost perfect (AC = 0.98), and IRA of scoring the diagnostic significance was moderate (AC = 0.51). Our results suggest that the simplified classification of the PPR is suitable for implementation in clinical practice.

Assessment of Predictors of Sun Sensitivity as Defined by Fitzpatrick Skin Phototype in an Ecuadorian Population and Its Correlation with Skin Damage.

BACKGROUND: The Fitzpatrick skin phototype scale (FSPTS) is a widely used instrument to assess skin type. METHODS: A cross-sectional survey collected responses from 254 subjects from Quito regarding self-reported FSPTS, gender, age, education, and tobacco and alcohol consumption. Univariate and multivariate logistic regression analyses were performed to determine if ethnicity, hair color, and eye color significantly predict FSPTS. In addition, we studied the correlation between FSPTS and the SCINEXA scale with Pearson's correlation coefficient. RESULTS: Ethnicity, eye color, and hair color are significant independent predictors of FSPTS (p < 0.0001). CONCLUSIONS: Patient self-reported race and pigmentary phenotypes are inaccurate predictors of sun sensitivity as defined by Fitzpatrick skin phototype. Our study does not fully represent the population of the country. There are limitations to using patient-reported race and appearance in predicting individual sunburn risk.

Update on treatment of photodermatosis.

Photodermatoses are a group of skin conditions associated with an abnormal reaction to ultraviolet (UV) radiation. There are several of the photosensitive rashes which mainly affect the UV exposed areas of the skin. It can be classified into four groups: immunology mediated photodermatoses, chemical and drug induced photosensitivity, photoaggravated dermatoses, and genetic disorders. A systematic approach including history, physical examination, phototesting, photopatch testing, and laboratory tests are important in diagnosis of a photodermatosis patient. In order to optimally treat a disease of photodermatoses, we need to consider which treatment offers the most appropriate result in each disease, such as sunscreens, systemic medication, topical medication, phototherapy, and others. For all groups of photodermatoses, photoprotection is one of the essential parts of management. Photoprotection, which includes sunscreening and wearing photoprotective clothing, a wide brimmed hat, and sunglasses, is important. There are also promising emerging photoprotective agents.

A review of UVA-mediated photosensitivity disorders.

A number of skin conditions are characterised by photosensitivity to UVA. Some of these are exclusively UVA-mediated conditions, while others include UVA in the action spectrum which also include UVB and/or visible light. This review aims to describe this diverse range of conditions for non-dermatologist scientists with an interest in this topic. As such, clinical details, including treatments, are brief and succinct. Recent advances in understanding the pathogenesis of these conditions is highlighted.

Photoparoxysmal response and its characteristics in a large EEG database using the SCORE system.

OBJECTIVE: To characterize photoparoxysmal EEG response (PPR) using a standardized protocol of intermittent photic stimulation (IPS) and standardized definitions for PPR, classified into six types. METHODS: Using the SCORE system (Standardized Computer-Based Organized Reporting of EEG) we prospectively built a large database of standardized EEG annotations. In this study, we extracted the features related to PPR from the structured dataset consisting of 10,671 EEG recordings with IPS, from 7,188 patients. RESULTS: The standardized IPS protocol elicited PPR in 375 recordings (3.5%), in 288 patients (4%), with a preponderance among young (11-20 years) and female patients (67%). PPR was persistent in patients with multiple recordings. The most frequent type of PPR was activation of preexisting epileptogenic area (58%), followed by generalized-PPR limited to the stimulus train (22%). We could not find any recording with self-sustained posterior response. Seizures were elicited in 27% of patients with PPR, most often myoclonic seizures and absences, in patients with self-sustained generalized PPR. CONCLUSIONS: The most common type of PPR was accentuation of preexisting epileptogenic area. Self-sustained posterior response could not be documented. Self-sustained generalized-PPR had the highest association with seizures. SIGNIFICANCE: Using standardized stimulation protocol and definitions for PPR types, IPS provides high diagnostic yield.

Photosensitivity skin disorders in childhood.

Photosensitivity in childhood is caused by a diverse group of diseases. A specific sensitivity of a child's skin to ultraviolet light is often the first manifestation or a clinical symptom of photodermatosis. It might indicate a serious underlying systemic disease such as lupus erythematosus or dermatomyositis, or a rare group of genetic skin disorders like Xeroderma pigmentosum, Cockayne syndrome, Trichothyodystrophy, Bloom syndrome, Rothmund-Thomson and Kindler syndrome as well as metabolic disorders and cutaneous porphyria. Photosensitivity secondary to topical or systemic agents may also cause photosensitivity in children. Early recognition and prompt diagnosis may prevent complications associated with unprotected exposure to sunlight and avoid actinic injuries that can lead to malignant skin changes.

Xeroderma pigmentosum group E and DDB2, a smaller subunit of damage-specific DNA binding protein: proposed classification of xeroderma pigmentosum, Cockayne syndrome, and ultraviolet-sensitive syndrome.

Xeroderma pigmentosum is a rare photosensitive syndrome that comprises eight different genetic diseases (A to G; variant (V)). Although genotype-phenotype correlations have been evaluated in most XP groups, the relationship between the E subgroup of xeroderma pigmentosum (XP-E) and damage-specific DNA binding protein (DDB) still remained a mystery. Recent studies have provided new insight for XP-E and the role(s) of DDB2, a smaller subunit of DDB. Reclassification studies have confirmed that mutations in DDB2 give rise to XP-E. The mouse model of XP-E demonstrated that DDB2 was well conserved between mouse and human and was critical in controlling proper cell-survival through regulating the tumor suppressor p53-mediated responses after ultraviolet (UV)-irradiation: i.e. defective DDB2 causes the resistance to cell-killing by UV-irradiation due to decreased p53-mediated apoptosis. These phenotypes are unique to XP-E because other XP groups show normal (XP-V) or hypersensitivity (XP-A, B, C, D, F, and G) to UV-irradiation. Thus XP-E is defined as a skin cancer prone disease with unique resistance to UV-irradiation.

[Photosensitization due to drugs: adverse reaction and beneficial effect]. / Photosensibilisierung durch Arzneistoffe. Unerwünschte Wirkung und therapeutischer Effekt.

In all areas of everyday life men get in touch with photosensitizers which increase the susceptibility to solar light. Dermal reactions arise after exposure to a photosensitizer and light. Quality and intensity depend on the compound and the radiation dose. Many drugs provoke these responses appearing as an adverse reaction during conventional treatment or as beneficial effect in the context of photodynamic therapy (PDT) or photochemotherapy (PUVA, ECP). Basic mechanisms and clinical aspects are reviewed.

The development of actinic keratosis into invasive squamous cell carcinoma: evidence and evolving classification schemes.

Actinic keratosis is an incipient form of cutaneous squamous cell carcinoma. Consequently, actinic keratoses must be treated expeditiously to forestall their downward growth. Several classification schemes have been proposed to better categorize actinic keratoses, and to guide their diagnosis and treatment. Among these approaches is the "keratinocyte intraepithelial neoplasia" (KIN) system developed by Cockerell; Goldberg's concept of the "proliferative actinic keratosis" (PAK), and Berhane's emphasis on the "inflamed actinic keratosis" (IAK). In the future, disparate classification schemes may be unified into a single pragmatic approach which accurately reflects the biological process whereby actinic keratoses devolve into invasive squamous cell carcinoma.

Photosensitivity. Classification.

Light is a heterogeneous moiety that is vital for the perception and maintenance of all life on earth. Specific advantages as well as adverse effects are associated with particular wavelengths of light. An etiologic classification of the adverse responses or "photosensitivity disease" is presented as an introduction to this issue.

Photobiology. Basic science.

Photobiology is the study of the effects of nonionizing radiation on biologic systems. The biologic effect varies with the wavelength region of the radiation. The radiation is absorbed by molecules in skin such as DNA, protein or certain drugs. The molecules are changed chemically into products that initiate biochemical responses in the cells.

Photosensitivity in childhood.

Out of a total of 370 patients with photosensitivity disorders, the light sensitivity had started at an age of 15 years or earlier in 95 cases (26%). Seventy-eight of these suffered from polymorphous light eruptions (PMLE). In half of the PMLE cases the morphology of the light-induced rash was classified as prurigo-like, and in one-fourth as vesicopapular. Other registered photodermatoses with childhood onset were xeroderma pigmentosum, erythropoietic protoporphyria, systemic lupus erythematosus and pellagra. No childhood cases of photosensitivity from external chemicals or internal medication were encountered.

The morphologic features of polymorphous light eruptions.

When the rashes of one hundred and thirty-eight patients with chronic polymorphous light eruption (PMLE) were carefully analyzed, ten different types of primary lesions, in descending order of frequency were recorded: vesiculopapules, erythema and edema, prurigo-lesions, soft maculondoules, eczema, plaques, urticas, blisters, target lesions, and solid nodules. Scars were noted in a third of the patients. Any type of lesion could be found in combination with any other type of lesion at a rate roughly proportionate to their general prevalence. One type of lesion, however, usually dominated the clinical picture of each patient, and served as a basis for the ordering of the patients into different subgroups. The four most common subgroups, ie, the vesiculopapular, pururigo-like, eczematous, and maculonodular variety accounted for 88 percent of the cases. There were marked intersexual differences in the relative frequency of the different PMLE subtypes. Familiarity with these considerable variationsin the clinical presentation of PMLE will help the clinician in making accurate diagnoses.

Photosensitization problems in livestock.

Photosensitization is a sunburn-like condition caused by the presence of certain photoactive compounds in the skin when it is exposed to the appropriate wavelength of light. Most photoactive compounds (phototoxic agents) that cause PS in livestock are of plant origin, others are drugs, chemicals, or endogenous porphyrins. Photosensitization is a disease caused by phototoxic xenobiotics, or by acquired or hereditary dysfunction of (1) heme synthesis or (2) PE excretion by the liver. Hepatotoxins, especially those of plant origin, most frequently are the cause of the condition. Photosensitization primarily is a disorder of sheep and cattle, but all classes of livestock are susceptible. Clinical recognition of the syndrome usually presents no difficulty because of the restriction of lesions to areas of skin unprotected from sunlight. Prognosis generally depends on the extent of hepatic injury. The most important elements of treatment are termination of exposure to the photo- or hepatotoxin, protection from light, treatment and prevention of infection and fly strike, and provision of nutritious feed.

[Value of the light-test histological study in photodermatoses]. / Intérêt de l'étude histologique du phototest dans les photodermatoses.

Clinic and histologic analysis of 92 phototests of various photodermatosis (14 benign summer light eruption (BSLE), 47 polymorphous light eruption (PMLE) 20 persistent light reactor photodermatitis (PLRP), 11 drug induced photodermatitis (DIP) has been performed. Clinically, a phototoxic aspect was observed in 30 cases, it was photoallergic in 36 cases and uncertain in 26 cases. Histologically, a phototoxic picture was present in 32 cases, a photoallergic one in 48 cases, and 12 cases exhibited unclassified aspect. Only one case of BSLE was associated with photoallergic aspect, clinically and histologically. Photoallergic type was observed in 70 p. 100 of iterative exposure phototest (IEP) of PMLE; clinical and histological aspects were concordant; in contrast phototoxic aspects were constantly observed when single exposure phototest (SEP) were used. In PLRP histological aspect of IEP was constantly of photoallergic type; whereas clinical aspect was uncertain in 50 p. 100 of the cases; 7 of the 10 biopsies of SEP exhibited a photoallergic picture. In DIP histological aspect of IEP was constantly photoallergic, whereas 5 of the 6 cases exhibited uncertain clinical aspect; only one of the 5 SEP gave a photoallergic reaction both clinically and histologically. Comparison of histological features revealed a more typical photoallergic picture in PLRP than in PMLE. We conclude that: the reproducibility of clinical and histological lesions is better with the iterative exposure phototest than with single exposure phototest; histologic examination is more reliable than clinical findings in persistent light reactor photodermatitis and drug induced photodermatitis.

[Does benign summer light eruption exist?]. / La lucite estivale bénigne existe-t-elle?

BACKGROUND: Contrary to other countries, in which a unique entity is recognized, two sub-groups of light eruption, benign summer light eruption and polymorphous light eruption, are identified in France. Benign summer light eruption was individualized with a few criteria: age at onset between 25-35 years old, female predominance, onset within 12 hours after an intensive exposure to sun, presence of lesions on areas which have not been regularly exposed to sunlight such as the upper chest, absence of lesions on the face, improvement during the summer period and negativity of the polychromatic phototest. In fact patients usually presenting with benign summer light eruption do not have all the criteria and they gradually develop a polymorphous light eruption. The aim of this study was to quantify, among the patients presenting with a light eruption, the population with three main criteria of benign summer light eruption. PATIENTS AND METHOD: Eighty-seven patients presenting with a light eruption, a polymorphous light eruption or a benign summer light eruption, were selected by a dermatologist. For each patient the presence or absence of the 3 main criteria were noted: a) no lesion of the face, b) improvement of the eruption during the summer period, c) negative polychromatic phototest. RESULTS: Among the 87 patients, 9 of them (10 p. 100) had the three main criteria of benign summer light eruption. DISCUSSION: Benign summer light eruption is either rare or defined with wrong criteria. The polychromatic phototest was gradually replaced by the UVA phototest. In fact, provocation UVA phototests do not individualize benign summer light eruption from polymorphous light eruption. Individualization of the benign summer light eruption is not justified because there is a continuous spectrum of light eruptions, ranging from the benign eruption which improves during the summer period and the chronic eruption with a high photosensitivy and lesions of the face.

Phototoxicity and photoallergy.

Phototoxic and photoallergic reactions resulting from a combined exposure to photosensitizing chemicals and light are common. Diagnosis of such reactions requires an understanding of its clinical presentation and recognition of commonly encountered photosensitizers. Phototesting allows identification of the photosensitizer and appropriate management of the patient.

Photosensitivity: classification, diagnosis, and treatment.

Photosensitivity disorders are common. Phototoxicity due to excess ultraviolet exposure while on a photosensitizing drug and polymorphic light eruption are two frequent examples. Patients should be encouraged to consider Taylor's ABCDEs: Avoidance, Barrier creams, Clothing, Dihydroxyacetone, and Education.

A case of type IV solar urticaria identified by reverse in vitro serum test.

We had a 17-year-old male patient with solar urticaria diagnosed as type IV of modified Harber's classification. The action spectrum of this case was estimated to be 433-499 nm and the inhibition spectrum was 533 nm. Both in vitro serum test and tentatively designated "reverse in vitro serum test" were positive. The patient was prescribed 20 mg/day epinastine hydrochloride (Alesion) orally for 49 weeks with improvement of the symptoms.