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Erythropoietic protoporphyria is a genetic disease characterized by sensitivity to sunlight caused by the accumulation of protoporphyrin IX. Photoprotection against ultraviolet A and visible light is necessary for erythropoietic porphyria patients because the absorption spectrum of protoporphyrin IX lies in both ultraviolet A and visible light region. We developed a novel index, in vitro porphyrin protection factor, based on the protoporphyrin IX absorbance spectrum. We also selected appropriate photoprotective products designed according to protoporphyrin IX absorbance. The porphyrin protection factors of a combination of make‐up base with a powder as well as with a liquid foundation were significantly higher than those of a conventional sunscreen product, even at a small application dose. An in‐use test carried out for 6 months showed that the efficacy of these products was 78.3%, and no adverse reactions were observed. Male subjects preferred liquid foundation, whereas all female subjects used powder foundation. The preference of the subjects could lead to the long‐term use of the tested products. In conclusion, this study provided a new approach to improve photoprotection in erythropoietic protoporphyria patients.

Xeroderma pigmentosum (XP) is an autosomal recessive genetic disorder characterized by extraordinary sensitivity to sunlight, resulting in cutaneous malignant tumors. Among XP, XP-F presents relatively uniquely in Japanese. To clarify the characteristics of this group, we describe a case of XP-F and review Japanese cases previously reported. A 50-year-old Japanese woman was referred to us with multiple, variously sized, light- or dark-brown macules on the face and sunlight-exposed extremities. She had experienced bulla formation with approximately 10 min of sunlight exposure during her elementary school years. Her parents had been first cousins, and her mother and sister had photosensitivity. She showed no neurological or developmental abnormalities. Ultraviolet (UV) irradiation testing revealed normal levels for minimal erythema dose with UV-A and UV-B. Sensitivity to UV-C and DNA repair ability in the patient's fibroblasts were indicated between that in normal individuals and that in an XP-A patient. Complementation assay revealed that transfection of the XPF gene led most efficient DNA repair compared with the other XP genes. Therefore, the patient was diagnosed with XP-F. Twenty-three cases of Japanese patients (six males, 17 females) with XP-F have been reported, including the present case. Our review suggested a relatively high prevalence of 50% (11/22) for cutaneous malignant tumors. A significant difference was evident in the mean age at first medical consultation between patients with cutaneous malignant tumors (53.6 years) and patients without such tumors (30.8 years). This suggests that cutaneous malignant tumors could occur in the age range of 30-50 years in XP-F patients.

L-carnosine (CAR) is an endogenous dipeptide. We aimed to determine the effects of CAR and its zinc complex polaprezinc (PLZ) on pressure ulcer healing in institutionalized long-term care patients.This study was a nonrandomized controlled trial with a maximum 4-week follow-up. Forty-two patients with stage II-IV pressure ulcers for 4 or more weeks were allocated to 1 of 3 groups in order of recruitment: the control group (n = 14) was untreated, the PLZ group (n = 10) orally received 150 mg/d PLZ (containing 116 mg CAR and 34 mg zinc), and the CAR group (n = 18) orally received 116 mg/d CAR. Pressure ulcer severity was measured weekly using the Pressure Ulcer Scale for Healing (PUSH) score.At baseline, no significant differences were found among groups in demographic and nutrition parameters and pressure ulcer characteristics (severity, size, and staging). After 4 weeks, the rate of pressure ulcer healing, assessed by the mean weekly improvement in PUSH score, was significantly greater in the CAR (1.6 ± 0.2, P = .02) and PLZ groups (1.8 ± 0.2, P = .009) than in the control group (0.8 ± 0.2). The difference between the CAR and PLZ groups was not significant (P = .73). Actual dietary intakes over this period did not differ significantly among groups.Our results suggest that CAR and PLZ may almost equally accelerate pressure ulcer healing during 4 weeks. The results need confirmation by randomized controlled trials with larger sample sizes.

Summary Background Xeroderma pigmentosum (XP) is a photosensitive genodermatosis with increased susceptibility to skin cancers. Patients are typically diagnosed with XP when they consult a dermatologist for skin cancers. Case/Methods The genetic analysis and 2–8 years of follow-up for three school-age patients with XP-V is described. The patients were referred to us because of increased pigmented freckles; they had not experienced abnormal sunburn or developed skin cancer at their first visit. All patients harbored a genetic mutation in the POLH gene. XPV9KO was diagnosed at age 13 with a homozygous del1661A that creates a stop codon in the non-catalytic domain of POLH. The patient practiced sun protection, effectively preventing the development of skin cancer by age 21. XPV19KO was diagnosed at age 11 with a compound heterozygous mutation of G490T and C1066T, causing POLH truncation in the catalytic domain. This patient developed basal cell carcinoma at ages 12 and 13. XPV18KO was referred to us at age 11 and diagnosed with compound heterozygous variants of c.1246_1311del66 (exon 9 skipping), a novel mutation, and c.661_764 del104 (exon 6 skipping). Conclusion Freckle-like pigmentation on sun-exposed skin is sometimes the only sign of XP-V, and early diagnosis is extremely important for children.

Sensitive skin syndrome was first described in 1977; however, no robust study has been carried out to evaluate its prevalence in Japan. A national representative sample of the Japanese population over the age of 18 years was taken. Individuals were questioned by telephone and selected according to the quota method. When asked "Do you have a sensitive skin?", 52.84% of men and 55.98% of women answered "rather sensitive" or "very sensitive". There was no significant difference (P = 0.22) between the two sexes. The non-response rate among respondents was zero, suggesting that the term "sensitive skin" held a meaning for the majority of the population. Concerning questions about the onset of a rash, tingling or irritation in the presence of various factors, such as emotional issues, cold, heat, sun, dry air, air-conditioning, water, air pollution and temperature variations, respondents with rather sensitive or very sensitive skin responded "yes" more often than others: approximately three-times more often for water (18.97%/6.15%), air pollution (39.29%/12.45%) and warm climatic conditions (29.74%/9.8%). To our knowledge, this epidemiological study is the first to focus on sensitive skin among Japanese people of this century. It is of particular interest for two reasons: (i) it was conducted on a representative sample of the Japanese population; and (ii) the methodology used was identical to that used for sensitive skin assessment studies conducted in Europe and the USA, making it possible to draw certain comparisons.

A 15-year-old boy presented with vesiculopapular eruptions confined to sun-exposed areas from the age of 3. Histopathological examination of biopsy specimens of repetitive UVA-irradiated areas revealed reticular degeneration of the epidermis, and dermal infiltrates in the photoinduced lesions showed a latent Epstein-Barr virus (EBV) infection. At the age of 21, the number of skin lesions had increased, and his anti-EBV antibody titers revealed an abnormal profile: an undetectable anti-EBV nuclear antigen antibody titer despite a detectable antiviral capsid antigen IgG antibody titer. No infectious mononucleosis-like symptoms, such as prolonged or intermittent fever, lymphoadenopathy, or liver damage were evident up till then. Severe hydroa vacciniforme (HV)-like eruptions were diagnosed considering the increased number of the skin lesions with increasing age and the unusual anti-EBV antibody titers, in addition to the histopathological findings. In the same year, he suddenly developed high fever and died from disseminated intravascular coagulation syndrome without any spontaneous regression of the skin lesions. In this patient with severe HV-like eruptions, the skin lesions had been confined to sun-exposed areas until his death, and the photo-provocation test showed a positive reaction. Severe HV-like eruptions may have clinical features suggestive of HV.

Background Sweet's syndrome with involvement of the central nervous system (CNS) is rarely reported. Methods We describe a Japanese woman with Sweet's syndrome associated with acute-onset encephalitis and review literatures. Results Examination of the cerebrospinal fluid (CSF) revealed pleocytosis with lymphocytes predominant. Magnetic resonance imaging (MRI) revealed increased signal intensity on T2-weighted scans in the left temporal lobe. To our knowledge, 22 cases of Sweet's syndrome associated with CNS involvement have been reported. The mean age is 47.6 years (n = 22). Sex distribution (male : female) is 12 : 10 (n = 22). The most common neurologic symptoms are convulsions, headaches, and disturbance of consciousness. CSF cell count is increased with lymphocytes predominant in 8 cases (n = 12). Certain (HLA) types (B54 and Cw1) may be characteristic findings in Sweet's syndrome accompanied with the CNS involvement in Japanese patients, as these were found in four previous cases similar to the present case. Conclusions Although Sweet's syndrome with neurologic manifestations is rarely reported, it may be needed to investigate neurologic manifestations.

We report a patient who developed Bowen's disease of the finger and bowenoid papulosis of the perianal area after cardiac transplantation. Human papillomavirus (HPV) type 16 only, not any skin-related or epidermodysplasia verruciformis-related types, was detected in both lesions by in situ hybridization and polymerase chain reaction. The same virus type was identified in both the tumor of the finger and the perianal area, which suggests contact transmission. HPV 16 has often been associated with malignant changes and may be at least one source of the malignancies that are more common in immunosuppressed patients. The presence of a potentially oncogenic type of the HPV in an immunosuppressed patient highlights the importance of regular follow-up of such patients.

We report a case of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) in a 21-year-old man. This disease is a severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) and is characterized by the sudden onset of diffuse ulcerations associated with high fever and systemic symptoms. It is sometimes lethal especially in elderly patients. In the present case, intense generalized maculopapular erythematous plaques with central necrosis developed progressively in association with a high fever. Initial treatment with systemic betamethasone had been unsuccessful and the skin lesions, which covered about 50% of the body surface, became severely ulcerated. Although the development of new lesions had ceased spontaneously, widespread ulceration of the skin remained. Debridement of the necrotic skin and skin grafting using cultured epidermal autografts and meshed allografts of cadaver skin led to prompt reepithelization.

Background: In parallel with the popular usage of topical ketoprofen, the number of reported cases of ketoprofen-induced photoallergic contact dermatitis has been increasing. It is clinically important to know the cross-reactivity of ketoprofen in order to avoid cross-sensitization caused by several structurally similar non-steroidal anti-inflammatory drugs (NSAID) on the market. Methods: To evaluate the spectrum of cross sensitization, photopatch testing was performed on five patients with ketoprofen-induced photoallergic contact dermatitis using ketoprofen and other structurally similar chemicals, such as oxybenzone, tiaprofenic acid and suprofen. Results: All five patients reacted positively to ketoprofen or ketoprofen plaster on photopatch testing. All four patients photopatch tested with related chemicals showed cross-photosensitization with tiaprofenic acid and suprofen. However, none of the patients reacted positively to oxybenzone. Conclusion: Either the diphenylketone moiety or a structurally similar tiophene-phenylketone moiety is important as the antigenic determinants of ketoprofen photoallergy. The arylpropionic acid side chain would not be involved.

Background The amount of nocturnal scratching can be an indirect correlate of itch in pruritic dermatoses. We have previously used an infrared video camera to measure nocturnal scratching in atopic dermatitis (AD). Although this is a reliable method of measuring nocturnal scratching, it is not suitable for routine monitoring in clinical use. Objectives To find a simplified way of monitoring itch. Methods We tried using a wrist activity monitor (ActiTrac®) for the measurement of nocturnal scratching in patients with AD. ActiTrac® is a wristwatch-shaped device that contains a piezoceramic sensor to measure and record limb movement over a pre-set time interval. The acceleration signal produced by motion of the hands is stored and downloaded into a personal computer. The average value of acceleration (AVA, 10−3 g min−1) was calculated and compared with total scratching time as a percentage of total recording time (TST%) measured with the use of an infrared video camera in 63 recordings of 21 patients with AD. For 261 recordings in 29 patients with AD, the AVA was measured and correlated with disease severity, and compared with the AVA of five non-itchy controls. Results There was a significant correlation between the AVA and TST% (r = 0·91, P

We investigated the effect of nitrazepam on nocturnal scratching in 1() adult out-patients with atopic dermatitis (AD ) using a double-blind placebo-controlled crossover method. Patients were given either nitrazepam (Benzalin tablets containing 5 mg nitrazepam) or a placebo on 3 successive nights, with a washout interval of 4 days. We used an infrared video camera to identify bouts of scratching lasting more than 5 s. These were counted and the duration of all the bouts of scratching (total scratching time. TST) was calculated. The percentage of TST to total recording time (TST%) was used as an index of nocturnal scratching. The frequency with which bouts of scratching (bouts/h) occurred was reduced by 10 mg nitrazepam (7.7 ± 3.6 with nitrazepam vs. 9.6 ± 3.6 with placebo, P < 0.05). However, the mean duration (s/bout) of the bouts of scratching was longer with 10 mg nitrazepam (32.3 ± 23.4 with nitrazepam vs. 19.1 ± 10.0 with placebo, P < 0.05). As a result, there was no significant difference between TST% (6.5 ± 4.2 with nitrazepam vs. 5.4 ± 3.8 with placebo, not significant). All the above values are mean ± SD. The degree of itching and the condition of the AD did not change during the 2 weeks of the study. We conclude that taking l0 mg nitrazepam is not an effective way of reducing the total duration of nocturnal scratching in AD patients, although it decreases the frequency with which bouts of nocturnal scratching occur.

We report a case of extramammary Paget's disease, representing a swollen left leg with reddish maculae on the thigh after resection of primary lesion on scrotum. Histologically, this lesion revealed the lymph duct's ectasis containing the mass of aggregated tumor cells, which were positive by immunohistochemical staining using PAS+Alucian blue, anti-CEA, and anti-c-erbB-2 antibodies. These were compatible with those of Paget's disease. Clinical appearance and histological findings of reddish lesion seen in this patient might be one of the characteristics of metastasis and infiltration of Paget's cells to lymph ducts.

Panton-Valentine leukocidin (PVL) secreted by Staphylococcus aureus is known to cause severe skin, soft tissue and lung infections. To assess the prevalence and genetic characteristics of PVL-positive S. aureus in our hospital, we investigated 86 S. aureus isolates isolated from skin and soft tissue pus between September 2011 and May 2012 at Daisan Hospital, the Jikei University School of Medicine (Tokyo, Japan). All isolates were investigated for the mecA gene and PVL gene by PCR amplification. The MRSA isolates confirmed were genotyped using SCCmec typing. PVL-gene positive isolates confirmed by the PVL-RPLA (reverse passive latex agglutination) assay were characterized by agr typing and multilocus sequence typing (MLST). Overall 6 (3 MSSA isolates and 3 MRSA isolates) PVL-positive strains (7.0%) were detected. The PVL prevalence was 11.1% in MRSA and 5.1% in MSSA. PVL-positive strains were isolated from young adults (range: 8-47 years) outpatient. Patients infected with PVL-positive MRSA were significantly younger than those infected with PVL-negative MRSA(32 and 68 years, respectively; P = 0.009, t-test). The 6 PVL positive strains were assigned by the MLST to 6 STs that were prevalent among PVL-positive strains. The SCCmec type of the PVL-positive MRSA were classified into 2 types (type IV or V) that were generally characteristic of CA-MRSA. Our data are consistent with some previous reports showing that PVL gene is found in certain ST strains. The PVL-positive strain must be taken into account when S. aureus is isolated from young adult SSTI.

紫外線は皮膚の構造・機能に大きな影響を与える環境因子のひとつである。紫外線照射による急性皮膚傷害はサンバーン, サンタンとしてみられ, 慢性の紫外線曝露は老人性色素斑, 光線性弾性線維症, 腫瘍発生などの光老化として長期の潜伏期間の後に出現する。その起点は主にUVBによるDNA損傷と, UVAによる細胞内外の光感作物質の励起で生ずる活性酸素種の発生を介した細胞傷害である。その結果, 皮膚の炎症, 構造的異常, 遺伝子変異, 光発癌, 免疫反応の抑制, 光線過敏症などが生ずる。それらの症状ならびに機序について概説すると共に, それを軽減または予防するための対策について皮膚科学的見地から最近の知見をまとめてみた。

Xeroderma pigmentosum (XP) is a photosensitive genodermatosis with increased susceptibility to skin cancers. Patients are typically diagnosed with XP when they consult a dermatologist for skin cancers.The genetic analysis and 2-8 years of follow-up for three school-age patients with XP-V is described. The patients were referred to us because of increased pigmented freckles; they had not experienced abnormal sunburn or developed skin cancer at their first visit. All patients harbored a genetic mutation in the POLH gene. XPV9KO was diagnosed at age 13 with a homozygous del1661A that creates a stop codon in the non-catalytic domain of POLH. The patient practiced sun protection, effectively preventing the development of skin cancer by age 21. XPV19KO was diagnosed at age 11 with a compound heterozygous mutation of G490T and C1066T, causing POLH truncation in the catalytic domain. This patient developed basal cell carcinoma at ages 12 and 13. XPV18KO was referred to us at age 11 and diagnosed with compound heterozygous variants of c.1246_1311del66 (exon 9 skipping), a novel mutation, and c.661_764 del104 (exon 6 skipping).Freckle-like pigmentation on sun-exposed skin is sometimes the only sign of XP-V, and early diagnosis is extremely important for children.

A 15-year-old boy presented with vesiculopapular eruptions confined to sun-exposed areas from the age of 3. Histopathological examination of biopsy specimens of repetitive UVA-irradiated areas revealed reticular degeneration of the epidermis, and dermal infiltrates in the photoinduced lesions showed a latent Epstein-Barr virus (EBV) infection. At the age of 21, the number of skin lesions had increased, and his anti-EBV antibody titers revealed an abnormal profile: an undetectable anti-EBV nuclear antigen antibody titer despite a detectable antiviral capsid antigen IgG antibody titer. No infectious mononucleosis-like symptoms, such as prolonged or intermittent fever, lymphoadenopathy, or liver damage were evident up till then. Severe hydroa vacciniforme (HV)-like eruptions were diagnosed considering the increased number of the skin lesions with increasing age and the unusual anti-EBV antibody titers, in addition to the histopathological findings. In the same year, he suddenly developed high fever and died from disseminated intravascular coagulation syndrome without any spontaneous regression of the skin lesions. In this patient with severe HV-like eruptions, the skin lesions had been confined to sun-exposed areas until his death, and the photo-provocation test showed a positive reaction. Severe HV-like eruptions may have clinical features suggestive of HV.

Psychosocial factors play an important role in the course of adult atopic dermatitis (AD). Nevertheless, AD patients are rarely treated for their psychosomatic concerns. The purpose of the present study was to develop and validate a brief self-rating scale for adult AD in order to aid dermatologists in evaluating psychosocial factors during the course of AD. A preliminary scale assessing stress-induced exacerbation, the secondary psychosocial burden, and attitude toward treatment was developed and administered to 187 AD patients (82 male, 105 female, aged 28.4 +/- 7.8, 13-61). Severity of skin lesions and improvement with standard dermatological treatment were assessed by both the dermatologist and the participant. Measures of anxiety and depression were also determined. In addition, psychosomatic evaluations were made according to the Psychosomatic Diagnostic Criteria for AD. Factor analysis resulted in the development of a 12-item scale (The Psychosomatic Scale for Atopic Dermatitis; PSS-AD) consisting of three factors: (i) exacerbation triggered by stress; (ii) disturbances due to AD; and (iii) ineffective control. Internal consistency indicated by Cronbach's alpha coefficient was 0.86 for the entire measure, 0.82 for (i), 0.81 for (ii), and 0.77 for (iii), verifying the acceptable reliability of PSS-AD. Patients with psychosomatic problems had higher PSS-AD scores than those without. PSS-AD scores were positively associated with the severity of the skin lesions, anxiety and depression. The scores were negatively associated with improvement during dermatological treatments. In conclusion, PSS-AD is a simple and reliable measure of the psychosomatic pathology of adult AD patients. It may be useful in dermatological practice for screening patients who would benefit from psychological or psychiatric interventions.

Mutations in the human DDB2 gene give rise to xeroderma pigmentosum group E, a disease characterized by increased skin tumorigenesis in response to UV-irradiation. Cell strains derived from xeroderma pigmentosum group E individuals also have enhanced resistance to UV-irradiation due to decreased p53-mediated apoptosis. To further address the precise function(s) of DDB2 and the consequence of non-naturally occurring DDB2 mutations, we generated mice with a disruption of the gene. The mice exhibited significantly enhanced skin carcinogenesis in response to UV-irradiation, and cells from the DDB2 –/– mice were abnormally resistant to killing by the radiation and had diminished UV-induced, p53-mediated apoptosis. Notably, the cancer-prone phenotype and the resistance to cellular killing were not observed after exposure to the chemical carcinogen, 7,12-dimethylbenz[ a ]anthracene (DMBA), to which mice carrying defective nucleotide excision repair genes respond with enhanced tumors and cell killing. Although cells from heterozygous DDB2 +/– mice appeared normal, these mice had enhanced skin carcinogenesis after UV-irradiation, so that XP-E heterozygotes might be at risk for carcinogenesis. In sum, these results demonstrate that DDB2 is well conserved between humans and mice and functions as a tumor suppressor, at least in part, by controlling p53-mediated apoptosis after UV-irradiation.

We report a patient with pemphigus vulgaris (PV) successfully treated with single filtration plasmapheresis. A 40-year-old man with PV was started on therapy with prednisolone (PSL). Although the dosage of PSL was doubled, and both cyclosporin A (CyA) and pulse therapy were added, the disease was not controlled. After single filtration plasmapheresis began, most of the eroded lesions on the trunk reepithelialized. A switch to double filtration was followed by recurrence. Finally, additional treatments with single filtration plasmapheresis were required to obtain remission. To evaluate the efficacy of the treatment, circulating antibodies were measured by immunofluorescence (IIF) and enzyme-linked immunosorbent assays (ELISAs) using recombinant desmoglein (Dsg) 3. IIF titer and the ELISA scores correlated with the clinical disease activity. It is suggested that ELISA was more sensitive than IIF.

Patients with atopic dermatitis (AD) are known to suffer from nocturnal itch, and the resultant scratching may worsen the skin lesions. We observed nocturnal scratching for 112 nights in 35 adult patients with AD, using an infrared video camera system. To quantify the amount of scratching, we counted scratching bouts lasting more than 5 s and calculated the duration of all the scratching bouts (total scratching time, TST). The percentage of TST in the total recording time (TST%) was used as an index of nocturnal scratching. Mean +/- SD TST% was 14.3 +/- 13.9 for patients with severe AD, 6.2 +/- 3.7 for those with moderate AD and 0.7 +/- 0.4 for those with mild AD. The higher TST% in the severely affected group was attributed mainly to a longer duration rather than a higher frequency of bouts. Patients scratched more in the first third of the night than in the later two-thirds. Both the group of patients whose disease distribution pattern was generalized and those who showed a head-neck-shoulder type distribution scratched their heads, faces and necks for longer than other parts of the body. Repeated measurement performed on individual subjects resulted in a similar TST% when there was little change in skin lesions. TST% reduced by 15 +/- 21% when the patients showed marked improvement. The measurement of nocturnal scratching helps to evaluate the severity of itch in AD. In addition, the infrared video successfully detected the location and nature of nocturnal scratching in AD.

We report a 56-year-old man with Paget's disease occurring near the left areola without any underlying breast carcinoma. Histologically, there was no evidence suggesting continuity with a lactiferous duct, accessory breast, or microscopic gynecomastia. We review previous case reports of Paget's disease occurring in unusual sites and discuss their nomenclature from the histogenetic point of view.

Skin responses to ultraviolet irradiation in patients with atopic dermatitis were studied to evaluate the role of sunlight in the exacerbation of atopic dermatitis. A total of 15 patients, seven males and eight females, with atopic dermatitis who complained of exacerbation of their dermatitis after sun exposure were examined by photo testing with UVB and UVA irradiation and photopatch tests. Nine out of 15 patients showed abnormal skin reactions. Lowered minimal erythema doses (MEDs) to a single exposure of UVB, papular or erythematous responses after single or repeated exposures to UVB or UVA and positive photopatch test reactions to sunscreen ingredients and fragrances were found. There are apparently multiple mechanisms of photoexacerbation in patients with atopic dermatitis.

We used an infrared video camera and video tape recorder system to record and analyze nocturnal scratching in seven patients with atopic dermatitis (AD) and three non-itchy healthy controls. The patterns and the locations of scratching were successfully observed. Scratching bouts lasting more than 5 sec were counted, and the summation of the duration of all the scratching bouts (total scratching time, TST) was used as an index of nocturnal scratching. TST in patients with AD turned out to be some hundreds to ten thousands of seconds; the TST in controls was between zero and a hundred seconds. The infrared video camera system measured nocturnal scratching simply and reliably without disturbing the patients' sleep.

Background and Design: We studied the clinical and photobiologic features of 51 patients with chronic actinic dermatitis who were evaluated at three institutions. The following criteria for patient selection were used: (1) a persistent eczematous eruption in the sun-exposed areas of greater than 3 months' duration; (2) decreased phototest results; and (3) when available, histologic changes of a dermal infiltrate of lymphocytes and macrophages, with or without epidermal spongiosis and atypical mononuclear cells in the dermis and epidermis. Results: The 51 patients had a mean age of 62.7 years, a male-to-female ratio of 2.6:1, and a mean duration of eruption of 5.8 years. The most common abnormal results of the phototests were decreased minimal erythema doses to both UV-A and UV-B, followed by decreased minimal erythema doses to UV-A alone. Patients with abnormally low responses to UV-A or visible light and normal minimal erythema doses to UV-B had the same clinical profile as the overall patient population. Aside from protection from sunlight, treatment modalities that have been used include PUVA (8-methoxypsoralen and UV-A) photochemotherapy, azathioprine, hydroxychloroquine sulfate, and, for recalcitrant cases, cyclosporine. Conclusions: Chronic actinic dermatitis is a persistent photodermatosis associated with abnormal phototest responses to UV-A, and/or UV-B, and/or increased sensitivity to visible light; histopathologic changes are consistent with photodermatitis. Treatment consists of combinations of topical and oral medications. (Arch Dermatol. 1994;130:1284-1289)

We have investigated the roles of mast cells and the complement system in the immediate phase of hematoporphyrin-induced phototoxicity in guinea pigs. Clinically, i.v. injection of hematoporphyrin, followed by irradiation with a light source containing 400-405 nm wavelength, resulted in the immediate onset of erythema and edema, which subsided partially in 30-60 min. This was followed by the appearance of delayed erythema and edema, which peaked at 6-12 h after irradiation. Histologic examination of the response of the immediate phase, using a 1 micron-thick section, revealed eosinophil infiltration and mast cell degranulation. The immediate phase of the clinical response was further quantitated by the extravasation of intravenously injected [125I]bovine serum albumin. Pretreatment of the guinea pig skin with the intradermal injection of compound 48/80 significantly suppressed the increase in vascular permeability induced by hematoporphyrin and irradiation (p less than 0.05). This hematoporphyrin-induced alteration in vascular permeability was also significantly inhibited by antihistamines, either H1 receptor antagonist alone (p less than 0.05) or a combination of H1 and H2 receptor antagonists (p less than 0.05). Guinea pigs depleted of complement also showed significantly less vascular permeability changes (p less than 0.05). These results indicate that functionally intact mast cells, and the complement system, are required for the full development of the immediate phase of phototoxicity induced by hematoporphyrin.

An 80-year-old Japanese woman with temporal arteritis was treated with systemic recombinant human interleukin-2 (IL-2) (1 x 10(6) unit/day for six weeks). The presenting symptoms of headache and skin necrosis and abnormal laboratory findings, such as an elevated erythrocyte sedimentation rate and CRP, promptly improved without any serious side effects. Although the pathogenesis of temporal arteritis and the mechanism(s) of the beneficial effect of IL-2 on it still remain unknown, this preliminary study highly encourages further investigations.

Patients with xeroderma pigmentosum variant show clinical photosensitivity, skin neoplasias induced by ultraviolet light, and defective postreplication repair, but normal nucleotide excision repair. We recently reported an alternative, simple method for the diagnosis of xeroderma pigmentosum variant that measures by autoradiography three cellular markers for DNA repair after ultraviolet irradiation: unscheduled DNA synthesis, recovery of RNA synthesis, and recovery of replicative DNA synthesis. Among hereditary photosensitive disorders, including other xeroderma pigmentosum groups, Cockayne syndrome, and a newly established ultraviolet-sensitive syndrome, only xeroderma pigmentosum variant cells exhibited normal unscheduled DNA synthesis, normal recovery of RNA synthesis, but reduced recovery of replicative DNA synthesis (51 +/- 6% the rate relative to normal controls). This reduction of recovery of replicative DNA synthesis was enhanced in the presence of a nontoxic level of caffeine to 36 +/- 5%. In this study we assess the cellular markers in two independent families that included two photosensitive patients that were identified as xeroderma pigmentosum variant. Cells from heterozygotic parents showed normal levels of unscheduled DNA synthesis, recovery of RNA synthesis, and recovery of replicative DNA synthesis, but reduced rates of recovery of replicative DNA synthesis in the presence of 1 mM caffeine (53 +/- 8% relative to the normal control). Furthermore, with a colony-forming assay, the cells showed normal survival by ultraviolet without caffeine, but slightly reduced survival by ultraviolet with 1 mM caffeine present. In one family, we confirmed inheritance of two heterozygous mis-sense mutations. One mutation is an A--G transition at nucleotide 1840 that generates a K535E mis-sense mutation. Another mutation is an A--C transversion at nucleotide 2003 that generates a K589 mis-sense mutation. Each of these mutations were absent in 52 unrelated Japanese individuals. These results suggest that xeroderma pigmentosum variant heterozygotes can be identified by their sensitivity to ultraviolet irradiation in the presence of nontoxic levels of caffeine.