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.     

Epstein-Barr virus-associated lymphoproliferative lesions presenting as a hydroa vacciniforme-like eruption: an analysis of six cases

BACKGROUND: There are many reports of patients with a severe hydroa vacciniforme (HV)-like eruption in which cutaneous lesions occur in both sun-exposed and non-exposed areas, unlike in true HV. Several patients have died from a malignant haematological neoplasm. In most cases, a latent Epstein-Barr virus (EBV) infection has been detected in the skin lesions. OBJECTIVES: To describe the clinical and laboratory features of six additional patients with an EBV-associated HV-like eruption. METHODS: The clinical, histological and immunohistochemical features were reviewed. T-cell receptor gamma gene rearrangements were studied using polymerase chain reaction (PCR) and heteroduplex analysis. In-situ hybridization was performed to detect mRNA for EBV in skin biopsy specimens. PCR was performed to screen for EBV infection in the skin lesions of three patients and blood of two patients. Photoprovocation with repeated ultraviolet (UV) A exposure was performed in three patients. RESULTS: The severity of the skin lesions and the clinical course varied among the patients. Skin lesions were induced by repeated UVA exposure in three patients and a latent EBV infection was demonstrated in the photoprovoked lesions. CONCLUSIONS: Three different clinical courses were found in six patients with an HV-like eruption associated with chronic EBV infection: (i) spontaneous remission; (ii) clearing after photoprotection; and (iii) continuous recurrence irrespective of sun exposure. It is possible that there are two patterns of HV-like eruption associated with chronic EBV infection. One is characterized by recurrent necrotic papulovesicles of the face and the other by nodules and facial swelling. It was demonstrated that the skin lesions could be triggered by repeated UVA exposure in the patients showing recurrent necrotic papulovesicles of the face.     

低剂量长波紫外线诱导培养的人皮肤黑素细胞适应性反应观察

目的 探讨低剂量长波紫外线(UVA)诱导培养人皮肤黑素细胞适应性反应的程度及特点。方法 以具有致死作用的86.4J/cm²UVA照射经7.2J/cm²低剂量UVA单次或多次预照射的培养人皮肤黑素细胞.光镜、电镜观察细胞形态学变化,流式细胞仪检测细胞凋亡的比例,单细胞凝胶电泳检测DNA损伤的程度。结果 单次或多次7.2J/cm²UVA预照射处理后的培养皮肤黑素细胞使随后86.4J/cm²UVA照射诱导的形态学上的毒性反应减轻,细胞凋亡的比例下降,DNA链断裂减少及修复加快,与未经预处理86.4J/cm²UVA照射的相应细胞比较差异有统计学意义(P<0.01或P<0.05);单次7.2J/cm²UVA预照射诱导培养皮肤黑素细胞的适应性反应在预照射12h后消失,而当低剂量UVA预照射的累积剂量达到28.8J/cm²以上时,预照射的培养细胞即使是14d后对86.4J/cm²UVA照射仍有明显的防护作用。结论 低剂量UVA照射可诱导培养的皮肤黑素细胞出现对随后高剂量UVA照射的适应性反应,其效应滞留期及强度与低剂量UVA的累积剂量有关。     

Hydroa vacciniforme. Diagnosis by repetitive ultraviolet-A phototesting

Hydroa vacciniforme is a rare disorder manifested in early childhood by recurrent photoinduced vesicles that heal with scarring. We report a case in which repetitive exposures to artificial ultraviolet light in the A range reproduced the clinical findings induced by natural sunlight. Phototesting may be viewed as an important diagnostic aid, as the induction of lesions clinically identical to hydroa vacciniforme can provide a reliable criterion for the diagnosis.     

A case of actinic prurigo showing hypersensitivity of skin fibroblasts to ultraviolet A (UVA)

We here report a patient with actinic prurigo. He had had erythematous papulovesicular eruptions on the sun-exposed sites from fall to early summer for 4 years. The lesions healed leaving atrophic scars. The histology showed epidermal necrosis and dermal dense perivascular lymphohistiocytic infiltration and edema. His minimal erythema doses to ultraviolet B (UVB) and UVA were normal and lowered, respectively. Skin lesions were produced by repeated irradiation with UVA plus UVB, but not with UVA alone. Then he was diagnosed as having actinic prurigo. Skin fibroblasts from the patient were hypersensitive to UVA. We believe that the hypersensitivity relates to the pathomechanisms of the photosensitivity in the case. UVA sensitivity of fibroblasts may be useful for differentiating actinic prurigo, hydroa vacciniforme, and other similar photosensitive disorders.     

重型种痘样水疱病的临床研究

９例重型种痘样水疱病患者均为日晒后暴露部位出现红斑、丘疹、水疱、糜烂、溃疡等，反复发作，渐致畸形。５例有手部畸形，表现为１、２、３指关节强直或屈曲、错位；２例指骨部分吸收破坏；５例耳廓部分缺损；２例鼻梁塌陷，软骨部分破坏吸收；１例下唇瘢痕挛缩，门齿外露；４例有角膜混浊。实验室检查均可排除卟啉代谢障碍性疾病，光试验对ＵＶＡ反应异常。因患者均有不同部位的畸形，故称为重型种痘样水疤病，并认为夏令痒疹、夏令水疱病、种痘样水疱病、重型种痘样水疱病是由光所致的谱性疾病。     

Phototests in Japanese patients with papulovesicular light eruption: positive provocation with UVA

In six Japanese patients with papulovesicular light eruption (PVLE), photoprovocative tests were performed by irradiating UVA or UVB on the skin of the back on three consecutive days. One patient developed the typical lesion of PVLE at the test site with two consecutive daily irradiations of UVA. The remaining 5 patients had no abnormal phototest reactions. It was obvious that UVA plays an etiological role in at least some cases of PVLE.     

Failure of physiologic doses of pure UVA or UVB to induce lesions in photosensitive cutaneous lupus erythematosus: implications for phototesting

BACKGROUND: Phototesting studies in cutaneous lupus erythematosus have yielded variable results, with most trials reporting photo-induction of lesions by both UVA and UVB in substantial numbers of patients. OBJECTIVES: To determine the minimal erythema dose in patients with subacute cutaneous lupus erythematosus (SCLE) and controls. PATIENTS/METHODS: We phototested nine patients with SCLE and 14 skin type-matched controls, using repetitive dosing of UVA1 and UVB, but with filters that removed most of the shorter UVC and longer infrared and visible light. In addition, DNA was isolated from anticoagulated blood to genotype the TNF-alpha 308 region in each patient and control. RESULTS: We were unable to demonstrate a difference in minimal erythema dose (MED) between patients and controls, or any correlation of MED with either TNF genotype or systemic drug therapy for SCLE. In addition, no SCLE skin lesions were induced in the nine patients with either UVA or UVB, and one patient cleared a skin lesion after low-dose UVA1 irradiation. CONCLUSIONS: The potential role of wavelengths outside the UVA and UVB range in the photo-induction of cutaneous lupus skin lesions needs to be investigated, and there is a need to standardize phototesting equipment and procedures for patients with cutaneous lupus erythematous.     

Ultraviolet A1 (340-400 nm) phototherapy for cutaneous T-cell lymphoma.

BACKGROUND: The results of a recent study suggested that ultraviolet A1 radiation (UVA1R; 340-400 nm) phototherapy for atopic dermatitis works through induction of apoptosis in skin-infiltrating helper T cells, indicating the possibility that other helper T cell-mediated skin diseases may respond to UVA1R as well. OBJECTIVE: The purpose of this open pilot study was to assess the therapeutic effectiveness of UVA1 phototherapy for cutaneous T-cell lymphoma (CTCL). METHODS: UVA1 phototherapy was used as monotherapy in patients (n = 3) with histologically proven CTCL (stages IA and IB). For daily whole body UVA1 irradiations, either a high-dose (n = 2; 130 J/cm2 UVA1 per exposure) or medium-dose (n = 1; 60 J/cm2 UVA1) regimen was used. Therapeutic effectiveness was assessed clinically and histologically. RESULTS: In each of the 3 patients, skin lesions began to resolve after only a few UVA1 radiation exposures. Complete clearance was observed between 16 and 20 exposures, regardless of whether the high- or medium-dose regimen had been employed. CONCLUSION: These studies suggest that patients with CTCL stages IA and IB can be treated effectively with UVA1 phototherapy.     

Dietary fish oil as a photoprotective agent in hydroa vacciniforme

Hydroa vacciniforme is a troublesome and scarring photosensitivity disorder for which treatment is unsatisfactory. Dietary fish oil rich in ω-3 polyunsaturated fatty acids reportedly increases the resistance to ultraviolet-induced erythema and rash provocation in polymorphic light eruption. We report for the first time the response of hydroa vacciniforme to dietary fish oil. Three Caucasian boys with the condition were placed on MaxEPA, five capsules daily. Phototesting was performed at baseline and after 3 months supplementation. At baseline, low erythemal thresholds were seen to monochromated UVA at 350 and 370 nm in all three boys, while one also had a low threshold to 320 nm (UVA) and another showed a low threshold to 300 nm (UVB). Broad-band UVA provocation challenge produced typical skin lesions in all the subjects. Following fish oil, all the boys showed reduced erythemal sensitivity to UVA and one also showed reduced sensitivity to UVB. Provocation challenge revealed a reduced response in all three children. Clinically, these changes were accompanied by pronounced improvement in one child, mild improvement in the second child, but no improvement in the third. The third boy subsequently showed good clinical response to azathioprine.     

Phototesting in Oriental patients with lupus erythematosus

Light sensitivity is an important clinical characteristic of several forms of lupus erythematosus (LE). Recently, investigations have been able to induce LE-like lesions in LE patients with UVA as well as UVB, although most of these studies were conducted in Caucasians. Thus, there is insufficient data on phototesting in Oriental patients with LE. The aim of this study was to evaluate light sensitivity in Oriental patients with LE. Fifteen patients with various forms of LE were tested. Patients were evaluated by provocative phototesting, and threshold doses of UVA and UVB radiation that produced erythema and pigmentation were determined. The minimal erythema doses (MED) of UVB, immediate pigment darkening (IPD), and minimal tanning doses (MTD) were within the normal range in LE patients compared to a control group. Skin lesions clinically and histologically compatible with LE were induced in two of six patients with SLE, and four of nine patients with DLE. These lesions developed in about 2 weeks (range 5 to 23 days) after irradiation and lasted approximately 1 to 3 months (47±24 days). The action spectrum of the induced lesions was within the UVB range in four patients, in the UVA range in one patient, and in the UVB and UVA ranges in one patient. We found no correlation between a positive history for UV sensitivity and phototest reactions. In conclusion, the incidence of positive phototest reactions in Oriental patients with LE seems to be similar to or a little lower than in Caucasians. There was no correlation between a positive history for UV sensitivity and phototest reactions.     

A case of hydroa vacciniforme with unusual ear mutilation

A 22 year-old man visited our department with a 18-year-history of recurrent vesicular eruption on his skin when exposed to the sun. History revealed that the skin lesions developed as vesicles at first, then over the next several days, they formed crusts and healed with scarring. We were able to induce skin lesions by a repetitive UV-A provocation test. By the clinical and histologic features of the induced lesions, the case was diagnosed as hydroa vacciniforme (HV). However, no vesicular lesions were found on physical examination. Instead, in addition to varioliform scarring, we found various unusual clinical manifestations: burn-like lesions and crusts, flexion contracture of the digitum, and ear lobe mutilation. The ear lobe mutilation, which had not been reported previously in HV, was especially interesting.     

A Case of Hydroa Vacciniforme

Hydroa vacciniforme (HV) is a rare and chronic pediatric disorder that is characterized by photosensitivity and recurrent vesicles that heal with vacciniforme scarring. The pathogenesis of HV is unknown; no chromosome abnormality has been identified. HV patients have no abnormal laboratory results, so the diagnosis of HV is based on identifying the associated histological findings in a biopsy specimen and using repetitive ultraviolet phototesting to reproduce the characteristic vesicles on a patient''s skin. Herein, we present a case of HV in a 7-year-old female who was diagnosed with HV according to histopathology and ultraviolet phototesting.     

PHOTOCHEMOTHERAPY (PUVA) AND PSORIASIS: COMPARISON OF 8-MOP AND 8-MQP/5-MOP

ABSTRACT: Twenty-eight psoriatic patients received PUVA treatment (psoraien and long ultraviolet irradiations. Two preparations were used; 8-methoxypsoralen and a mixture of 5-methoxypsoralen and 8-meth-oxypsoralen. Both gave considerable improvement, but in 6 cases the lesions reappeared after 2 to 8 weeks, in spite of maintenance treatment. In this report, photochemotherapy of psoriasis was compared, using a UVA emitting lamp, 8-MOP, and a mixture of 8–MOP and 5 methoxypsoralen (5-MOP)^1–7.     

Hydroa Vacciniforme with Inflammatory Keratitis and Secondary Anterior Uveitis

A 6-year-old boy had numerous episodes of hydroa vacciniforme. Several of these episodes were accompanied by an anterior uveitis with corneal clouding and stellate keratic precipitates. Wearing sunglasses prevented new eye lesions from developing despite recurrences of skin lesions. Phototesting on facial skin revealed reproduction of skin lesions with ultraviolet B but not ultraviolet A. One should be aware of eye involvement in hydroa vacciniforme, and children who experience this form of photodermatitis should have a careful eye examination and be advised to wear protective sunglasses.     

Persistent light reaction: induction in the guinea pig

Persistent light reactions similar to those in humans were observed in the study of photoallergenicity of chemicals in guinea pigs. The animals photoinduced with chemicals reacted to long-wavelength ultraviolet (UVA) radiation in the absence of test materials at the challenge stage. The sensitivity of the animals to UVA persisted for more than 1 year. The minimum erythema dose to UVB of animals in the treated group was less than that in the control group. Our investigations indicate that the main factors influencing the elicitation of persistent light reaction were the amount of Freund's complete adjuvant (FCA) used to enhance the allergic response in animals, and the UVA dose at the induction stage. Based on these findings, we have developed a method to make animals persistent light reactors with high frequency. This method consists of 1 intradermal injection of 1.2 ml emulsified FCA, 5 irradiations with 20.4 J/cm2 of UVA, and topical applications of 5% p-aminoethylbenzoate (benzocaine) at the induction stage. We also found that photosensitivity could be induced using FCA and UVA without photosensitizers.     

Successful ultraviolet A1 phototherapy in the treatment of localized scleroderma: a retrospective and prospective study

Background Ultraviolet (UV) A1 phototherapy is an effective anti‐inflammatory treatment modality that influences fibroblast functions. Objectives To document the effects of UVA1 treatment in patients with localized scleroderma (LS) in a retrospective study (at least 6 months after UVA1 treatment) and in a prospective study before and immediately after medium‐dose UVA1 irradiation. Methods In total, 30 patients (retrospective study n = 17, prospective study n = 13) with LS receiving UVA1 phototherapy five times weekly (for 3–6 weeks) were investigated. Improvement was documented using standardized questionnaires and clinical evaluation (using modified Rodnan skin score, Cutometer and 7·5‐MHz ultrasound measurements). Levels of collagen I and collagen III metabolites were measured in serum and urine. Results In the retrospective study, medium‐dose UVA1 phototherapy had been performed 6 months–3 years earlier (cumulative dose 750–1400 J cm^?2; mean ± SD number of irradiations 19·3 ± 3·8). Fourteen of 17 patients (82%) reported an improvement in symptoms following UVA1 therapy. In the prospective study, skin elasticity increased in 77% of the patients following medium‐dose UVA1 phototherapy (cumulative dose 750–1250 J cm^?2; mean ± SD number of irradiations 20·8 ± 4·0). 7·5‐MHz ultrasound measurements showed a mean reduction of lesional skin thickness of 13% compared with skin thickness before UVA1 phototherapy. The ratio of deoxypyridinoline to creatinine was significantly elevated in about two‐thirds of the patients. Conclusions This open study showed a positive short‐ and long‐term efficacy of UVA1 phototherapy in patients with LS, with a reduction in sclerotic plaques, an increase in skin elasticity and a reduction of lesional skin thickness. UVA1 phototherapy had a significant effect on collagen metabolism. UVA1 phototherapy can be regarded as a safe treatment modality for patients with LS.     

UVA rush hardening for the treatment of solar urticaria

Induction of tolerance by subsequent UV exposures is the most effective therapy for solar urticaria; however, it is time-consuming and takes a long time until protection is achieved. Three patients with solar urticaria were exposed to multiple UVA irradiations at 1-hour intervals per day. With this rush hardening regimen, protection was achieved within 3 days.     

低剂量长波紫外线诱导培养人皮肤角质形成细胞适应性反应观察

目的:观察低剂量长波紫外线(UVA)诱导培养人皮肤角质形成细胞适应性反应的程度及特点,探讨其对皮肤可能的保护作用。方法:以具有致死作用的86.4J/cm2UVA照射经7.2J/cm2低剂量UVA单次或多次预照射培养的人皮肤角质形成细胞,并在光镜、电镜下观察细胞形态学变化,用流式细胞仪检测细胞凋亡的比例,单细胞凝胶电泳检测DNA损伤的程度。结果:单次或多次7.2J/cm2UVA预照射处理后的培养人皮肤角质形成细胞,对随后86.4J/cm2UVA照射诱导的相应细胞在形态学上的毒性反应减轻,细胞凋亡的比例下降、DNA链断裂减少及修复加快,和未经预处理的86.4J/cm2UVA照射的相应细胞比较差异均有显著性(P<0.01或P<0.001);单次7.2J/cm2的UVA预照射诱导培养的人皮肤角质形成细胞的适应性反应在预照射12h后消失,而当低剂量UVA预照射的累积剂量>28.8J/cm2时,预照射的培养细胞即使是14d后,对86.4J/cm2UVA照射仍有明显的防护作用。结论:低剂量UVA照射可诱导培养的人皮肤角质形成细胞出现对随后高剂量UVA照射的适应性反应,其滞留期及强度与低剂量UVA预照射的累积剂量有关。     

"High-dose" UVA1 therapy of widespread plaque-type, nodular, and erythrodermic mycosis fungoides

BACKGROUND: UVA1 (340 to 400 nm) was found to be effective in the treatment of early-stage mycosis fungoides (MF). OBJECTIVE: The purpose of this study was to assess the efficacy of UVA1 phototherapy for widespread plaque-type, nodular, and erythrodermic MF. METHODS: Thirteen patients (8 with stage IB, 4 with IIB, and 1 with III MF) received 100 J/cm(2) UVA1 daily until remission. Four patients also had lesions inaccessible by UVA1 that were considered control lesions. Immunocytologic studies of skin infiltrates and circulating T cells were done before and after the therapy. RESULTS: Eleven patients showed complete clinical and histologic responses. Two patients had a partial improvement. Unirradiated control lesions never improved. Serious short-term side effects were not recorded. Circulating CD4(+)/CD45RO(+) and CD4(+)/CD95(+) lymphocytes were significantly reduced by the therapy. CONCLUSION: UVA1 therapy is an effective and well-tolerated treatment for advanced MF. The therapeutic relevance of the effects on circulating lymphocytes remains to be established because lesions in nonexposed cutaneous areas did not respond.