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At least 19 recordsLinked to original sources
PubMed1996

Clinical characteristics of three patients with UVs syndrome, a photosensitive disorder with defective DNA repair.

Recently, we established a new category of photosensitive disorder termed UVs syndrome. Cells from patients with UVs syndrome have a similar UV sensitivity as xeroderma pigmentosum (XP) cells, but have a normal level of unscheduled DNA synthesis (UDS) unlike XP. UVs syndrome is distinct from Cockayne syndrome (CS) or XP including XP variant (XP-V), as determined by studies of genetic factors using cell fusion, microinjection, and postreplication repair assays. In this study, we identified three Japanese patients with UVs syndrome: an 11-year-old girl, a 17-year-old male, and an 8-year-old boy. The first two patients were siblings, while the third was a case from a different family. All of these patients exhibited acute recurrent sunburn. Common clinical manifestations of these patients were slight erythema and dryness, a number of freckles on sun-exposed areas, and slight telangiectasia only seen on the cheek and nose. Patient 3 showed a lowered minimal erythema dose between 280 and 300 nm. The patients' fibroblasts showed similar characteristics to those in CS, such as UV sensitivity, and a failure of RNA synthesis (RRS) after UV irradiation, despite a normal level of UDS. Thus, UVs syndrome is a new hereditary photosensitive disorder with clinical manifestations similar to a mild form of XP but showing the cellular characteristics of CS.

Adolescent↗
PubMed1998

Pediatric photosensitivity disorders.

Physicians who serve the pediatric population are the first line in identification and management of photosensitivity disorders present in childhood. The role of the dermatologist is not only to identify and treat these disorders, but also to counsel patients and their families in specific light avoidance, photoprotection, and in some cases, major lifestyle adjusts. This article reviews photosensitivity disorders, treatment, and prevention.

Child↗
PubMed2002

Increase in sunburns and photosensitivity disorders at the edge of the Antarctic ozone hole, southern Chile, 1986-2000.

BACKGROUND: Over the past 15 years Punta Arenas, Chile, a medium-sized city located on the extreme southern tip of South America, has repeatedly been exposed to acute, sudden episodes of highly increased levels of ultraviolet B (UVB) 280-320 nm radiation because of the passage of the spring Antarctic "Ozone Hole" overhead, or nearby. OBJECTIVE: Our purpose was to observe the relationship between episodes of ozone depletion, increased UVB radiation, and sunburns and photosensitivity disorders in Punta Arenas, Chile, during spring. METHODS: Incidence of photosensitivity disorders and sunburns was registered by dermatologists during each of the past 15 springs. Local data of sudden, severe ozone depletions (<250 Dobson units) and the corresponding increase of UVB radiation were reviewed. RESULTS: Patients with sunburn increased significantly during the austral spring of 1999 (P <.01). This was especially noticeable (29/31 cases) on weekends with ozone depletion, and increased UVB radiation (P <.01) occurred on the Sundays Oct 31, Nov 21, and Dec 5, 1999. The incidence of photosensitivity disorders, although statistically not significant, increased 51% over the past 7 years. CONCLUSIONS: An acute impact on human health (sunburn) occurred because of abrupt ozone depletion and the accompanying increase in UVB during the mid and late austral spring of 1999. Most sunburns (93.5%) occurred on weekends. Ozone levels as well as seasonal and recreational factors played a mayor role in the increase in sunburns. The increase in radiation at 300 nm, the most carcinogenic wavelength, on days under the Antarctic ozone hole is a matter of special concern.

Chile↗
PubMed1999

High sensitivity of the ultraviolet-induced p53 response in ultraviolet-sensitive syndrome, a photosensitive disorder with a putative defect in deoxyribonucleic acid repair of actively transcribed genes.

Previously, we reported a new category of photosensitive disorder named ultraviolet-sensitive syndrome (UVs S) [T. Itoh, T. Fujiwara, T. Ono, M. Yamaizumi, UVs syndrome, a new general category of photosensitive disorder with defective DNA repair, is distinct from xeroderma pigmentosum variant and rodent complementation group 1, Am. J. Hum. Genet. 56 (1995) 1267-1276.]. Cells derived from these patients show impaired recovery of RNA synthesis (RRS) after UV-irradiation irrespective of having a normal level of unscheduled DNA synthesis (UDS). These characteristics are reminiscent of Cockayne syndrome (CS) cells. By comparing sensitivity of the UV-induced p53 response in cells with different types of defects in nucleotide excision repair, we hypothesized that the UV-induced p53 response is triggered by inhibition of RNA synthesis [M. Yamaizumi, T. Sugano, UV-induced nuclear accumulation of p53 is evoked through DNA damage of actively transcribed genes independent of the cell cycle, Oncogene 9 (1994) 2775-2784.]. To test this hypothesis, we determined sensitivity of the p53 response in UVs S cells by immunostaining, Western blotting, and FACScan analysis. Maximal nuclear accumulation of p53 in the UVs S cells was observed with a one-third UV dose required for that in normal cells, while almost identical p53 responses were observed in UVs S and normal cells following treatment with heat or alpha-amanitin. Recovery of RNA synthesis after a low dose of UV-irradiation was impaired in UVs S cells to the same extent as seen in CS cells. These results provide further evidence to support our previous hypothesis regarding the mechanism of the p53 response induced by DNA damage.

Amanitins↗
PubMed1996

The coexistence of psoriasis with lupus erythematosus and other photosensitive disorders.

The coexistence of psoriasis with LE or other photosensitive disorders is rare in our patient population, occurring in 0.69% of patients with psoriasis and 1.1% of those with LE. PMLE was the most common cause of photosensitivity in psoriatic patients without LE, occurring in 32%. Less common causes included drug-related photosensitivity (thiazides and thiazide derivatives in four of the five cases), PUVA reactions, and photocontact reactions. The Goeckerman regimen or UVB applied in a cautious, well-controlled atmosphere was generally well tolerated in this group, including patients with PMLE. Photosensitivity occurred in 50% of our patients with psoriasis and LE, and it was secondary to LE in 70% of cases. Most patients were female and had SLE. Psoriasis developed first in 55% of the cases. Studies that were useful for distinguishing photosensitive from nonphotosensitive patients with SLE included determination of antibodies to extractable nuclear antigens (67% versus 14%), double-stranded DNA (64% versus 9%), and skin biopsy for direct immunofluorescence (58% versus 27%). Occasional patients have features suggestive of photosensitivity with or without signs or symptoms of LE. These patients may have atypical psoriatic plaques occasionally yielding routine histology diagnostic of psoriasis with direct immuno-fluorescence results suggestive of lupus. Frequently, connective tissue serology findings are positive, and affected patients require close follow-up for the development of LE. In patients with psoriasis and suspected photosensitivity, we recommend a careful history and examination, skin biopsy for routine histology and direct immunofluorescence, blood tests including determination of antibodies to antinuclear antibodies (Hep-2 substrate if negative on routine substrate), extractable nuclear antigens, and double-stranded DNA, and phototesting when indicated. Large-scale prospective studies are required before the most appropriate therapy for patients with psoriasis and LE can be recommended.

Adolescent↗
PubMed2001

A candidate gene analysis of three related photosensitivity disorders: cutaneous lupus erythematosus, polymorphic light eruption and actinic prurigo.

BACKGROUND: Polymorphic light eruption (PLE) is a common inherited photosensitivity disorder, which may predispose to several related but distinct conditions, including subacute cutaneous lupus erythematosus (SCLE), discoid lupus erythematosus (DLE) and actinic prurigo (AP). OBJECTIVES: To examine specific candidate genes for shared susceptibility alleles between these related phenotypes. METHODS: Eighty-five caucasian patients with annular SCLE or DLE were recruited, in addition to 102 first-degree relatives. The prevalence of PLE in both the patient and relative groups was determined by detailed interview and clinical examination. Eighty-five patients with pure PLE and 59 patients with AP were also recruited. Candidate genes were analysed by typing of single nucleotide polymorphisms of IL10 (-1082 G/A and -819 C/T), FCGR2A (131 R/H), SELE (128 S/R), ICAM1 (241 G/R and 469 E/K), IL1A (+ 4845 G/T), IL1B (-511 C/T and + 3954 C/T), IL1RN (+ 2018 T/C) and TNF (-308 G/A) using polymerase chain reaction (PCR) with sequence-specific primers and 5'-nuclease PCR. RESULTS: A significant association was found between SCLE and the rare TNF -308 A allele when compared with patients with DLE (P = 0.043), PLE (P = 0.001), AP (P < 0.001) and healthy controls (P < 0.001). However, there was strong linkage disequilibrium between TNF -308 A and the HLA A*01, B*08, DRB1*0301 haplotype. A negative association was also found between SCLE and the IL1B + 3954 T allele (P = 0.039), but the significance was lost on correction for multiple testing. CONCLUSIONS: We have demonstrated the association of SCLE with the rare TNF -308 A allele, which may be pathogenic or, alternatively, a marker allele for the extended HLA A*01, B*08, DRB1*0301 haplotype that is associated with a number of autoimmune conditions. Although many of the other loci that we chose failed to demonstrate an association, a candidate gene approach remains the most logical one, and the most likely to yield positive results in the future.

Alleles↗
PubMed2002

Photosensitivity disorders: cause, effect and management.

Abnormal photosensitivity syndromes form a significant and common group of skin diseases. They include primary (idiopathic) photodermatoses such as polymorphic light eruption (PLE), chronic actinic dermatitis (CAD), actinic prurigo, hydroa vacciniforme and solar urticaria, in addition to drug- and chemical-induced photosensitivity and photo-exacerbated dermatoses. They can be extremely disabling and difficult to diagnose. PLE, characterized by a recurrent pruritic papulo-vesicular eruption of affected skin within hours of sun exposure, is best managed by restriction of ultraviolet radiation (UVR) exposure and the use of high sun protection factor (SPF) sunscreens. If these measures are insufficient, prophylactic phototherapy with PUVA, broadband UVB or narrowband UVB (TL-01) for several weeks during spring may be necessary. CAD manifests as a dermatitis of chronically sun-exposed skin. Again, UVR exposure needs to be restricted; cyclosporine, azathioprine or PUVA may also be necessary. Actinic prurigo is characterized by the presence of excoriated papules and nodules on the face and limbs, most prominent and numerous distally. Actinic prurigo is managed again by restriction of UVR and the use of high SPF sunscreens; PUVA or broadband UVB therapy, or low doses of thalidomide may be necessary. Hydroa vacciniforme causes crops of discrete erythematous macules, 2 to 3mm in size, that evolve into blisters within a couple of days of sun exposure. Treatment for this rare disease is difficult; absorbent sunscreens and restricted UVR exposure may help. Solar urticaria is characterized by acute erythema and urticarial wealing after exposure to UVR. Treatment options for solar urticaria include non-sedating antihistamines such as fexofenadine and cetirizine; other options include absorbent sunscreens, restriction of UVR at the relevant wavelength, maintenance of a non-responsive state with natural or artificial light exposure and plasmapheresis. Industrial, cosmetic and therapeutic agents can induce exogenous drug- or chemical-induced photosensitivity. The clinical pattern is highly varied, depending on the agent; treatment is based on removal of the photosensitizer along with restriction of UVR exposure. Predominantly non-photosensitive dermatoses may also be exacerbated or precipitated by UVR; exposure to UVR should be reduced and sunscreens should be advocated, along with appropriate treatment of the underlying disease.

Acute Disease↗
PubMed1995

UVs syndrome, a new general category of photosensitive disorder with defective DNA repair, is distinct from xeroderma pigmentosum variant and rodent complementation group I.

Previously, we reported two DNA repair-defective siblings who did not belong to any complementation group of xeroderma pigmentosum (XP) or Cockayne syndrome (CS). By surveying other photosensitive patients whose fibroblasts showed similar biochemical phenotypes, we found another nonconsanguineous Japanese patient belonging to the same complementation group as our previous cases. Postreplication repair of the cells derived from these patients was normal, indicating that they cannot be classified as XP variant. Neither transfection nor microinjection of the cells with the human DNA repair gene ERCC1, which is known not to correct any complementation groups of XP or CS, failed to correct the defect of these cells, indicating that they do not belong to the rodent complementation group 1. However, the defect in recovery of RNA synthesis (RRS) after UV irradiation was restored by microinjection of HeLa cell extract. Although clinical manifestations of these patients--such as acute sunburn, dryness, freckling, pigmentation anomalies on sun-exposed skin, and teleangiectasia without neurological abnormalities or tumors--are similar to a mild XP phenotype, cellular characteristics such as UV sensitivity and defective RRS after UV irradiation with normal unscheduled DNA synthesis (UDS) are reminiscent of CS. On the basis of these results, we propose that these patients be included under a general category designated "UV-sensitive" (UVs) syndrome.

Abnormalities, Multiple↗
PubMed2004

Recurrent mutations in kindlin-1, a novel keratinocyte focal contact protein, in the autosomal recessive skin fragility and photosensitivity disorder, Kindler syndrome.

Kindler syndrome (OMIM 173650) is a rare autosomal recessive disorder characterized by trauma-induced blister formation (especially in childhood) and photosensitivity. Other features include mucocutaneous scarring and progressive poikiloderma. There is also an increased risk of skin and mucous membrane malignancy. The disorder was recently mapped to 20p12.3 and pathogenic mutations were identified in a new gene, KIND1. This gene encodes a 677 amino acid protein, kindlin-1, a component of focal contacts in keratinocytes. In this study, we identified four new recurrent mutations in KIND1 in 16 individuals with Kindler syndrome from 13 families of Pakistani (676insC), UK Caucasian (E304X), Omani (W616X), or Italian (958-1G > A) origins. Haplotype analysis demonstrated common ancestral mutant alleles for each mutation, apart from one of the six Pakistani families in which the mutation 676insC (which occurs in a repeat of seven cytosines) was present on a different genetic background. All mutations were homozygous, apart from the three UK Caucasian cases that were all compound heterozygotes (second allele mutations: L302X, 1161delA, 1909delA). All mutations were associated with markedly reduced or absent skin immunostaining with an antikindlin-1 antibody. These loss-of-function KIND1 mutations demonstrate the importance of kindlin-1 in maintaining epithelial integrity, although the mechanism linking this mutant protein to photosensitivity and poikiloderma remains to be determined. Delineation of these recurrent mutations is also relevant to optimizing mutation detection strategies in Kindler syndrome patients from particular ethnic backgrounds.

Adult↗
PubMed1997

[Ketoprofen-induced contact photosensitivity disorders: 5 cases].

BACKGROUND: Nonsteroidal antiinflammatory drugs (NSAID) are widely used in topical applications for benign diseases. Adverse skin reactions include contact eczema and photocontact dermatitis. Among the NSAID used in topical applications, arylpropionic derivatives, notably ketoprofen, are frequently implicated. CASE REPORTS: We observed 5 patients who developed eczema lesions after application of Ketum, a gel containing ketoprofen used on healthy skin after exposure to sunlight. Photoallergy explorations evidenced positive photopatch-tests for ketoprofen with UVA and total light. The anamnesis suggested a photoallergic mechanism which was confirmed by histological examination of the biopsy of a UVA positive photopatch-test and by negative photopatch-tests in 10 healthy controls. DISCUSSION: The photosensitizing potential of ketoprofen in the UVA spectrum is well known. Although the number of adverse reactions is quite small compared with widespread use, physicians should be aware of this photosensitivity and report all cases to the pharmacovigilance center.

Administration, Topical↗
PubMed2004

Investigation of photosensitive disorders.

Investigation of photodermatosis is based primarily on the history and clinical findings, histological, immunological and biochemical findings are variably helpful depending on the clinical picture. Formal testing for photosensitivity may be the only definitive test proving photosensitivity on occasion. This article delineates the important features which allow classification of patients which then enables appropriate treatment.

Dermatitis, Photoallergic↗
PubMed2006

The use of systemic fluoroquinolones.

The only indications for which a fluoroquinolone (ie, ciprofloxacin) is licensed by the US Food and Drug Administration for use in patients younger than 18 years are complicated urinary tract infections, pyelonephritis, and postexposure treatment for inhalation anthrax. Nonetheless, approximately 520,000 prescriptions for fluoroquinolones were written in the United States for patients younger than 18 years in 2002; 13,800 were written for infants and children 2 to 6 years of age, and 2750 were written for infants younger than 2 years. Clinical trials of fluoroquinolones in pediatric patients with various diagnoses have been published and are reviewed. Fluoroquinolones cause arthrotoxicity in juvenile animals and have been associated with reversible musculoskeletal events in both children and adults. Other adverse events associated with fluoroquinolones include central nervous system disorders, photosensitivity, disorders of glucose homeostasis, prolongation of QT interval with rare cases of torsade de pointes (often lethal ventricular arrhythmia in patients with long QT syndrome), hepatic dysfunction, and rashes. The increased use of fluoroquinolones in adults has resulted in increased bacterial resistance to this class of antibacterial agents. This report provides specific guidelines for the systemic use of fluoroquinolones in children. Fluoroquinolone use should be restricted to situations in which there is no safe and effective alternative to treat an infection caused by multidrug-resistant bacteria or to provide oral therapy when parenteral therapy is not feasible and no other effective oral agent is available.

Adolescent↗
PubMedDate not supplied

A case of comorbidity between panic disorder and photosensitive epilepsy.

Panic disorder and epilepsy usually are distinct entities which require different and specific therapeutic strategies. While anticonvulsant medication is the treatment of choice for seizure disorders, behavioral methods have proven to be effective in panic disorder. We here report a case of comorbidity between panic disorder and photosensitive epilepsy. Special attention is given to the different symptomatic presentations of the disorders, because a thorough knowledge of both disorders may save unnecessary diagnostic procedures. Therefore, the necessity of taking a careful patient's history is underlined. Furthermore the different possible relationship between panic disorder and epilepsy are discussed.

Adult↗
PubMed1995

Photodistributed hypertrophic lichen planus in association with acquired immunodeficiency syndrome: a distinct entity.

The cutaneous manifestations of human immunodeficiency virus infection include papulosquamous diseases, viral and fungal infections, and neoplastic disorders. Eczematous photosensitivity disorders have been reported in patients infected with the human immunodeficiency virus. We describe a patient with advanced acquired immunodeficiency syndrome who developed photodistributed hypertrophic lichen planus. We believe this is a distinct cutaneous manifestation of human immunodeficiency virus infection.

Adrenal Cortex Hormones↗