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CD30-positive primary cutaneous anaplastic large cell lymphoma (C-ALCL) is an indolent type of cutaneous lymphoma with favourable clinical prognosis. Pseudocarcinomatous hyperplasia (PCH) is a rare benign epithelial condition that can resemble invasive squamous cell carcinoma both clinically and histopathologically. PCH predominantly occurs in CD30-positive lymphoproliferative disorders. We report a 75-year-old woman with PCH in a multifocal C-ALCL located on the scalp and right retroauricular area, which rapidly responded to treatment with psoralen ultraviolet A photochemotherapy. Comprehensive virological analyses for potential oncogenic viruses, including Epstein-Barr virus, human herpesvirus-8, human papillomaviruses, the recently discovered cutavirus and nine different human polyomaviruses, were negative.

Summary Background Psoralen ultraviolet A (PUVA) and narrowband (NB)-UVB have been shown to be efficacious in the treatment of vitiligo. With large and repeated doses, UVA may lead to immediate skin darkening and to delayed tanning. Our previous experience with broadband (BB)-UVA in vitiligo showed encouraging results. Aim To test the efficacy of BB-UVA in vitiligo and to evaluate if it could provide an alternative treatment for this condition. Methods This prospective, randomized, controlled, comparative clinical trial enrolled 45 patients with vitiligo, who were randomly divided into three groups, with group A receiving UVA 15 J/cm2/session, group B receiving UVA 10 J/cm2/session, and group C receiving PUVA. The patients received three sessions/week for 5 months, with 60 sessions in total. Results At the mid-point of treatment, clinical response was significantly higher in patients receiving PUVA than in the other two groups At the end of the study, clinical response was comparable for groups A and C (UVA 15 J/cm2 and PUVA, respectively), and both were significantly higher than the group receiving UVA 10 J/cm2. Patients in the PUVA group responded mainly with perifollicular pigmentation, whereas those receiving UVA responded mainly with lesional tanning. Conclusions BB-UVA at a dose of 15 J/cm2/session gives results for vitiligo that are comparable to PUVA, suggesting it might be useful when oral psoralens are contraindicated.

Treatment of severe hand eczema (HE) that is resistant to topical potent corticosteroid treatment is challenging. In 2013, we surveyed 194 UK dermatologists to obtain information about their usual treatment pathways to inform the choice of the comparator in a trial of alitretinoin in severe HE (ALPHA trial); the results indicated that the treatment approaches favoured by UK dermatologists differ. Psoralen combined with ultraviolet A (PUVA) and alitretinoin were identified as the most frequent first-line treatment options for hyperkeratotic HE, whereas oral corticosteroids were identified as the most frequent first-line treatment for vesicular HE, followed by PUVA and alitretinoin. In terms of potential adverse effects of long-term or repeated use, oral steroids and ciclosporin A were reported to cause most concern. There is uncertainty about which treatment gives the best short and long-term outcomes, because of a lack of definitive randomised controlled trials evaluating the effectiveness of different treatment pathways in severe HE.

Summary Background. Effective treatment options for morphoea remain limited. As a result, there has been increasing interest in the role of phototherapy in the management of this condition. Aims. We report the findings of a study in which 13 patients with localized morphoea were treated with oral (n = 11) and topical (n = 2) psoralen ultraviolet (PUVA) phototherapy. Methods. The clinical effect on disease activity was assessed using a skin score adapted from the modified Rodnan score. The effect on serological and immunohistochemical markers was also measured. Results. In total, 11/13 patients showed an improvement in their skin score after phototherapy, with the mean reduction in score being 62.9% (P = 0.003, Wilcoxon signed rank test). After treatment with PUVA, there was a fall in circulating levels of vascular cell adhesion molecule in 10/13 patients (P = 0.059) and a significant increase in tumour necrosis factor-α in 9 of 13 patients (P = 0.036). In the five patients in whom CD3 and CD4 was measured, all showed a reduction in CD3 (P = 0.025), with a fall in CD4 (P = 0.046) seen in four of the five patients. Conclusions. PUVA is associated with clinical improvement in patients with morphoea, as shown by significant improvement in the skin score. However, in one patient who had been simultaneously started on ciclosporin, this improvement could not be attributed to the phototherapy alone. Although the sample size was small, we also found that certain lymphocyte markers, adhesion molecules and cytokines are affected by this treatment, helping to further clarify the mechanism of action of PUVA treatment.

Summary Background. Recent evidence suggests that systemic psoralen plus ultraviolet A (PUVA) therapy may have a stimulatory effect on melanocytes, not only locally but also systemically. Aim. We aimed to assess endothelin-1 (ET-1), a potent melanocyte mitogen, in plasma of PUVA-treated paients with vitiligo. Methods. ET-1 was sequentially assessed (using ELISA) in patients with nonsegmental vitiligo treated with PUVA (n = 20), at 8, 16 and 24 h following the PUVA session. Evaluations took place at 0, 1 and 3 months of therapy. Patients with psoriasis (n = 15) treated identically and healthy subjects not receiving any therapy (n = 15) served as controls. Vitiligo Area Scoring Index (VASI) and Psoriasis Area Severity Index (PASI) scores were simultaneously evaluated. Results. ET-1 was significantly lower in vitiligo than in psoriasis at month 0 (8.2 ± 3.6 vs. 13.7 ± 5.4 pg/mL; P = 0.03) and it was significantly higher in both than in healthy controls at all time points of the PUVA sessions (P 0.05). VASI score significantly decreased at month 3 (19 ± 9.6 vs. 11.9 ± 7.3; P

Vitiligo is an idiopathic leukoderma often with a progressive course causing destruction of melanocytes. The best methods for achieving cosmetically acceptable re-pigmentation of affected skin appear to be both local and systemic PUVA. They may, however, cause serious side effects, which is an argument for conducting research into new, equally effective photo-chemotherapeutic agents. One of these agents is khellin. We conducted a pilot study in 33 patients to evaluate the effectiveness of local KUVA and systemic PUVA therapy for vitiligo and to compare them in terms of the degree of re-pigmentation, duration of treatment, number of procedures, total UVA dose and side effects. Local KUVA required longer duration of treatment and higher UVA doses. KUVA-induced re-pigmentation depended on the age of the patients (r = -0.61, P = 0.001), and better results were achieved with younger individuals [% re-pigmentation = 81.76 - (1.48 x age in years)]. No side effects were observed in cases of local KUVA treatment. Erythema, itching and gastro-intestinal disturbances occurred with some patients treated with PUVA. The results demonstrate that local KUVA may effectively induce re-pigmentation of vitiligo-affected skin areas to a degree comparable to that achieved when using systemic PUVA, provided that treatment duration is long enough.

Palmoplantar pustulosis or chronic palmoplantar pustular psoriasis (PPP) is an uncommon but disabling and recalcitrant pustular dermatosis affecting the palms and soles. Its onset is strongly linked to cigarette smoking and once it is established it tends to progress gradually over years or decades. Its relationship to psoriasis is disputed. Treatment is unsatisfactory and many different therapeutic manoeuvres have been advocated. We have performed a Cochrane systematic review of PPP in order to assess the evidence for efficacy of treatments for PPP. Randomized controlled trials (RCTs) of interventions for PPP were sought by interrogating the Cochrane Controlled Trials Register, Medline and Embase for the terms (PALM* or PLANT* or SOLE* or BACTERID) and (PUSTUL* or PSORIA*). Additional searches were undertaken by cross-checking with two databases of psoriasis trials collated in our department and by the European Epidermo-Epidemiology Network (EDEN). We found 23 RCTs that met our inclusion criteria. These comprised eight trials of systemic retinoid monotherapy [six vs. placebo, one vs. psoralen photochemotherapy (PUVA), one comparing two retinoids]; four trials of PUVA or retinoid-PUVA (two PUVA vs. placebo, two PUVA vs. PUVA plus systemic retinoid); two trials each in which cyclosporin, hydroxyurea, a tetracycline or colchicine was compared with placebo; one trial of Grenz ray therapy vs. placebo; and two trials of topical corticosteroids under hydrocolloid gel occlusion compared either with steroid alone or with additional PUVA therapy. Many of the studies were of short duration with small numbers of participants. Outcome measures used varied considerably between studies. The following interventions were shown to be significantly better than placebo in reducing disease severity scores in PPP: etretinate, liarazole, PUVA using oral psoralen, low dose cyclosporin, Grenz ray therapy, tetracycline antibiotics. Acitretin was of equal efficacy to etretinate. Overall however, few patients achieved good or excellent results. Potent topical corticosteroids were shown to be more effective if used under occlusion. The addition of PUVA to such therapy was of no extra benefit. No RCT of methotrexate therapy was found but evidence from one open study suggests that it is of limited efficacy. The following interventions were also of no or minimal benefit: PUVA using topical psoralen, hydroxyurea, colchicine. The best results were achieved with systemic retinoid therapy both on its own (40% good or excellent response) and in conjunction with oral PUVA (68% clearance) when it was more effective than topical or oral PUVA alone. The value of systemic retinoids for long-term therapy is, however, diminished by their side-effects. The ideal therapy for PPP remains elusive.

A 10-year retrospective analysis of the use of psoralen photochemotherapy (PUVA) in the treatment of vitiligo was undertaken at the St John's Institute of Dermatology, London, UK. Of 97 patients included in this study, eight had complete or almost complete repigmentation, 59 moderate to extensive repigmentation, and 30 showed little or no response. However, 24 of those who had responded to PUVA with extensive repigmentation did not consider their response satisfactory because of persistence of vitiligo at cosmetically sensitive sites, and poorly matching, speckled repigmentation. Fifty-seven patients who initially improved with PUVA therapy subsequently relapsed, in most cases within a year of stopping treatment. Relapses in 22 patients were on the same cutaneous sites as previously affected, while vitiligo at new sites developed in 20 patients and both new and old sites were affected in a further 15 patients. Patients who retained their pigmentation after 2 years appeared to have a better chance of permanent remission. The only statistically significant prognostic indicator of relapse was patient age at the start of treatment, younger patients tending to retain their pigmentation longer than older patients. This study emphasizes the need for careful patient counselling before PUVA therapy as this treatment seldom achieves extensive repigmentation that is cosmetically acceptable, and treatment response is often followed by relapse.

Long-term management of psoriasis requires an individualized approach. Some treatments such as calcipotriol, methotrexate and acitretin may be used as maintenance treatment for many months. However, most anti-psoriasis treatments should be prescribed for restricted periods of time. Rotational treatment is a practical approach to reduce the cumulative toxicity of anti-psoriasis treatments. The selection of a treatment is based on the clinical presentation of psoriasis and whether contraindications might exist. Combination treatment is another approach, which is used by the majority of patients. Useful combinations are calcipotriol-acitretin, calcipotriol-cyclosporin, calcipotriol-PUVA, calcipotriol-topical corticosteroids, dithranol-UVB, dithranol-tar, coaltar-UVB, acitretin-UVB and acitretin-PUVA. Combinations which are contraindicated are coaltar-PUVA, UVB-cyclosporin, PUVA-cyclosporin and methotrexate-acitretin. Combined use of UVB-methotrexate, UVB-PUVA; PUVA-methotrexate; methotrexate-cyclosporin and cyclosporin-acitretin require careful monitoring and might be helpful in patients with severe and recalcitrant psoriasis. Depending on the individual presentation of psoriasis, previous anti-psoriatic treatments and side-effects, treatment adjustments are made.

The beneficial effects of ultraviolet (UV) radiation on atopic dermatitis has been appreciated for many years. While broadband UVB and psoralen UVA have been the mainstay of phototherapy for some time, the past 5 years have seen the introduction of phototherapeutic modalities, including UVA-1 and 311nm UVB. The best modality and mode of usage is dependent on the type of atopic dermatitis, severity and body site. T lymphocytes play an important role in disease pathogenesis and UV radiation has profound effects on skin and systemic immune responses.

Oral lichen planus is a relatively common inflammatory disease affecting between 0.5% and 2.2% of the population in epidemiological studies. In contrast with cutaneous lichen planus (LP), in which the clinical course is often mild and resolves within 2 years, mucosal LP tends to follow a more chronic course often punctuated by acute exacerbations. Furthermore, although distinct clinical subtypes such as reticular, atrophic, hypertrophic and erosive forms are well recognized, more than one clinical phenotype may be seen at a time. The rare association with oral neoplasia should always be considered and high-risk patients must be kept under close observation. Thus the management of this disorder will vary widely both between patients, and for individual patients, with fluctuations in disease activity. Here we discuss the therapeutic options available and review the evidence for their use.

Bath-PUVA is an alternative to oral-PUVA for the treatment of psoriasis. This study compares the effectiveness of the two methods in two groups, each consisting of 17 patients with plaque psoriasis. Three patients who failed to improve with oral-PUVA were transferred to bath-PUVA and subsequently cleared. Another seven patients who returned with a further episode of psoriasis received the alternative treatment; this gave a group of 10 patients in whom a cross-over comparison was possible. In both comparisons bath-PUVA was as effective as, or more effective than, oral-PUVA and required, overall, less than 50% of the total UVA, although this saving was not as great as in previous reports. Bath-PUVA caused fewer immediate problems and was preferred by many patients. It is suitable for those taking other systemic medications and we recommend it as a valuable therapeutic option that should be available at all treatment centres.

In this retrospective study the clinical, laboratory and histological features, treatment methods and disease course in 17 patients with papuloerythroderma (PE) are reviewed. The median age at diagnosis was 72 years and the female/male ratio was 1:4:7. The most common abnormal laboratory findings were eosinophilia and an elevated serum IgE level. Psoralen photochemotherapy (PUVA) and oral prednisolone 10-20 mg daily given in combination or alone were very efficient treatments, while UVB phototherapy in combination with topical steroids was also successful. However, potent topical corticosteroids alone or in combination with oral antihistamines were not effective. The patients were observed for a median of 19 months from diagnosis, three being followed for more than 5 years. Five of the patients relapsed, but only one had multiple relapses, two developed cutaneous T-cell lymphoma (CTCL) and two others had histological features suggestive of that disorder. Six of the patients died, cardiovascular disease being the most common cause. PE is a distinct clinical entity with a polymorphous aetiology which frequently includes an association with CTCL or visceral malignancy. PUVA, oral corticosteroids and UVB in combination with topical corticosteroids appear to be effective therapeutic modalities.

Summary The aim of our study was to evaluate the long-term results of topical psoralen ultraviolet A (PUVA) in patients with necrobiosis lipoidica (NL), in whom conventional methods (pentoxifylline, vitamin E, tretinoin, and topical or intralesional corticosteroids) had failed. The study comprised 10 women (age range 17–44 years), six of whom were insulin-dependent diabetics and four were diabetes-free. Duration of NL ranged from 3 to 10 years. The patients were treated with a 0.005% aqueous solution of 8-methoxypsoralen, applied topically for 30 min, and subsequently irradiated with UVA three times weekly. All the patients experienced almost complete remission (softening of skin lesions, no hyperpigmentation, lack of lesion progression) after a mean of 47 sessions (mean UVA cumulative dose 69.5 J/cm2). They were followed up for 12–24 months, during which time two recurrences, both in diabetic patients, were observed after 8 and 12 months of treatment cessation, which further resolved after another course of topical PUVA. We conclude that topical PUVA is well tolerated by NL patients and may serve as an alternative therapeutic regimen.

We report two patients in whom episodes of polymorphic light eruption were followed by recurrent erythema multiforme on exposed and nonexposed sites. Treating the polymorphic light eruption with prophylactic PUVA and/or oral prednisolone or cyclosporin prevented the development of erythema multiforme, suggesting that the two events are related. It is possible that erythema multiforme develops as a response to the same causative antigen as polymorphic light eruption.

We obtained a successful response to PUVA therapy in a 51-year-old Japanese man who had multiple nodules of the trunk, extremities and scalp along with polyclonal hypergammaglobulinaemia; the scalp lesions were associated with extensive alopecia. Examination of skin biopsies showed a dense infiltrate of mature plasma cells in the dermis and hair follicles; no systemic disease or functional involvement of other organs was detected. The clinical and histological findings were compatible with cutaneous plasmacytosis and treatment with topical PUVA gradually reduced the size and number of the lesions.

Psoralen plus ultraviolet A irradiation (PUVA therapy) is commonly used for the management of vitiligo in which perifollicular repigmentation is the usual response pattern. However, excessive PUVA therapy may be associated with adverse effects. We report a case of generalized vitiligo that has been extensively treated with topical and systemic PUVA therapy for several years with the development of extensive and widespread stellate and irregularly shaped black and brown macules (lentigines). Interestingly, the lentigines were observed not only in the normally pigmented skin but also within the depigmented lesions that were lacking the perifollicular response pattern. The lesions developed in the exposed and unexposed skin areas. No evidence of skin malignancy was observed clinically and no melanocyte atypia was detected histopathologically. Cryotherapy may be used in the management of the lentigines; however, because of the extent of lesions this was impractical in our case.

We report a case of a 69-year-old gentleman who presented with a 3-month history of unexplained fevers and malaise who developed generalized pruritus, alopecia and an ichthyosiform erythematous eruption on his forearms, legs, chest and back. Skin histology, immunophenotyping and molecular features were consistent with granulomatous mycosis fungoides. He has been successfully treated with twice weekly PUVA photochemotherapy.

Summary The response to treatment of all patients enrolled over an 18-month period for localized oral or topical psoralen photochemotherapy (PUVA) of chronic hand and foot dermatoses was retrospectively reviewed. There were broadly similar success rates for the two groups for complete clearance: 61.5% (eight of 13 patients who completed therapy)—oral PUVA, 47.8% (11 of 23 patients who completed therapy)—topical PUVA, and for significant improvement: 23.1% (three of 13 patients)—oral PUVA, 30.4% (seven of 23 patients)—topical PUVA; there were no significant differences in response when diagnostic subgroupings of the hand dermatoses were taken into account. The mean number of treatments (22 for oral PUVA and 24 for topical), treatment durations (122 and 129 days), maximum UVA doses (11.2 and 12.3J/cm2) and to a lesser extent cumulative UVA doses (189.3 and 237.0 J/ cm2) for the therapies were similar in the two groups; adverse effects were minimal for both treatment protocols. However, at least five of the eight patients in the oral PUVA group and five of the 11 in the topical group who cleared completely relapsed after a mean 86 (range 19.245) and 174 (range 23-596) days, respectively. These findings are in broad agreement with those of previous studies. Therefore to avoid generalized photo-sensitivity and a higher likelihood of adverse effects with systemic therapy, as well as a possible slower relapse rate, topical therapy seems preferable.

Summary The action spectrum for producing minimal phototoxic erythema after oral administration of 8-methoxypsoralen (8-MOP) was determined in the range of 312-368 nm in 12 human volunteers using six different UV radiation sources. The peak sensitivity was found to be at 343 nm. The 8-MOP photosensitivity was at a high level (1.75 of maximum) between 336 and 355 nm. Conventional UVA radiation sources, like the Philips TL/09R tube, have a high energy output within 335 and 355 nm, and are therefore highly recommended in oral psoralen plus UVA radiation treatment.

Palmoplantar psoriasis (PPP) produces significant morbidity and requires prompt treatment. Topical agents form the mainstay of therapy. We compared the efficacy and side-effect profile of a steroid/coal-tar combination with topical psoralen and solar ultraviolet A (PUVAsol) in PPP.In total, 52 patients with PPP were randomized to receive either a combination of clobetasol propionate cream and coal tar daily (group 1) or topical PUVAsol on alternate days (group 2) for 16 weeks. Response was assessed as change in Psoriasis Activity and Severity Index (PASI) and Patient Global Assessment (PGA).Of the 52 patients, 43 completed the treatment phase. There was a reduction in PASI for the palms and soles in both treatment groups throughout the treatment period until week 16. There was a greater reduction in PASI in palmar psoriasis with topical PUVAsol, and a greater reduction in psoriasis of the soles with the steroid/coal-tar combination. In both groups, patients perceived 'good improvement'. Improvement or cure in palmar lesions was observed in 90% of cases in the topical steroid/coal-tar group and in 75% of cases in the topical PUVAsol group; for the soles, these figures were 76% and 79%, respectively. No adverse effects were experienced with the steroid/coal-tar combination, whereas for the topical PUVAsol, phototoxicity occurred in 22% of cases.Both treatments had comparable efficacy. In both groups, patients experienced 'good improvement' after 16 weeks of therapy.

Summary Pre-treatment plaque thickness determined the rate at which psoriasis responded to PUVA treatment, taking longer with thicker plaques. For dithranol, the rate of response to treatment was independent of pre-treatment plaque thickness. Plaques of psoriasis that showed the greatest evaporative water loss before treatment took longest to clear with PUVA. At the time of clinical clearing the water loss had returned to that of adjacent, uninvolved skin. With dithranol the evaporative water loss did not return to normal levels but remained considerably elevated when the plaque was clinically clear.

Graham Little-Piccardi-Lassueur syndrome (GLPLS) is a rare lichenoid dermatosis defined by scarring alopecia, loss of pubic and axillary hairs and progressive development of horny follicular papules variously located. Topical or systemic corticosteroids, retinoids or PUVA therapy are the treatments usually proposed and these have partial and temporary benefits. We describe the effectiveness of cyclosporin A in a case of GLPLS at the dosage of 4 mg/kg/day. At the end of treatment, substantial reduction of both perifollicular erythema and follicular hyperkeratotic papules was observed. After 3 months of follow-up, besides the results already obtained, a few areas of hair regrowth in the scarring patches and a more consistent improvement of the follicular papules were detected. We believe that cyclosporin A could be effective mainly in the initial phases of this rare variant of lichen planopilaris, before the development of severe follicle damage, either by interfering with the acute inflammatory processes or by limiting the progression of the disease. To the best of our knowledge, this is the first report showing a good and persistent therapeutic effect of cyclosporin A in GLPLS.

Summary There is a dearth of studies comparing the efficacy of psoralen ultraviolet A (PUVA) and narrowband (NB)-UVB in psoriasis in South Asian patients. Patients having plaque psoriasis with > 20% body surface area involvement were randomly assigned to one of two groups (group A: NB-UVB, group B: PUVA). The response to treatment was assessed by the Psoriasis Area and Severity Index (PASI) at baseline and every 2 weeks thereafter. The maximum possible treatment duration was 16 weeks. In total, 43 patients (21 NB-UVB, 22 PUVA) completed the study. Marked improvement was seen in 80.9% of the patients in group A and 81.8% in group B (NS: P > 0.05). The mean ± SD time taken to achieve marked improvement was 9.9 ± 3.3 and 9.9 ± 3.5 weeks, respectively. In total, 29 patients were available for the analysis of the remission data at 6 months after treatment completion; 26.7% of the patients in group A and 42.8% in group B were in remission (NS: P > 0.05). Both methods seem to be equally effective in achieving clearance and maintaining remission of severe chronic plaque psoriasis in patients with Fitzpatrick skin type 4 and 5.

Psoriasis vulgaris (PV) is characterized by keratinocyte hyperproliferation. Altered expression of cell-cycle regulatory genes involved in the cyclin D1 ⁄ p16 INK4-pRb pathway may contribute to this epidermal hyperproliferation.To assess the expression of cyclin D1 and p16 in psoriasis, and to evaluate the effect of phototherapy on their expression.The study population comprised 25 patients with PV and 10 healthy controls. Patients were treated with 24 sessions of either narrowband ultraviolet (UV) B or psoralen UVA. Skin biopsies were taken from the affected skin of each patient before and after treatment, and from the healthy controls, to examine cyclin D1 and p16 expression.Before phototherapy, the mean value of cyclin D1 concentration in patients was significantly greater than that in controls and the mean value of p16 concentration in patients was significantly lower than that in controls. Following treatment, we detected a significant decrease in cyclin D1 and a significant increase in p16.Cyclin D1 upregulation and p16 downregulation may play a role in the pathogenesis of psoriasis. Normalization of the levels of both parameters may be a mechanism by which phototherapy induces remission in psoriasis.

Summary CsA therapy for psoriasis is hampered by side-effects. Topical CsA therapy is not effective. Therefore improvement of the risk-benefit ratio must be achieved by improvement of dose-regimens, combination therapy of CsA with another therapy for psoriasis or combination therapy of CsA with a therapy that may influence the pathogenesis of the most serious side-effect(s) of CsA. It seems that only therapies that influence the pathogenesis of CsA- induced acute renal-impairment, like oral fish oil or perhaps thromboxane synthesis inhibitors, may be beneficial. However, further studies are warranted to validate these findings.

We report a 38-year-old woman with psoriasis who developed multiple atypical lentigines following psoralen photochemotherapy (PUVA). The lentigines first appeared 12 years ago, 3 years after she commenced intermittent PUVA treatment. New lesions continued to develop over the subsequent years with further photochemotherapy. Clinically, the lentigines were strikingly atypical, deeply pigmented, dark brown or black, large stellate macules. Histology of a representative lesion was consistent with a PUVA lentigo and no atypical melanocytes were seen. At present, a link between malignant melanoma and PUVA lentigines has not been established. Instead, limited evidence suggests that PUVA lentigines may be more closely linked with the risk of nonmelanoma skin cancer.

The cutaneous changes seen in systemic sclerosis (SSc) can result in considerable patient morbidity.We previously reported on the beneficial effect of psoralen ultraviolet A (PUVA) phototherapy in 13 patients with morphoea. We now report the findings of a study in which patients with SSc were treated with PUVA.Twelve patients with SSc were treated with PUVA phototherapy. The effect on cutaneous disease activity was assessed using the modified Rodnan score, and the effect on serological and immunohistochemical growth factors and adhesion molecules was also measured.The median Rodnan score at baseline was 24.5 [interquartile range (IQR) 18.5-26.0]. The median number of treatments with PUVA was 24 exposures (IQR 20-26) with a median cumulative exposure of 68.3 J/cm(2) (IQR 28.6-139.8). Of the 12 patients, 11 responded well to phototherapy with a mean change in Rodnan score of 6.58 (36.98%) (P0.01, Wilcoxon signed ranks test). After treatment with PUVA there was a significant increase in circulating tumour necrosis factor-alpha levels in 8/12 patients (P = 0.03). In 7/12 patients there was an increase in E-selectin and vascular cell adhesion molecule, although this was not significant.PUVA treatment is associated with a significant improvement in cutaneous symptoms in patients with SSc as measured by the Rodnan score (P0.01). Specific lymphocyte markers, adhesion molecules and cytokines are also affected by this treatment, helping to clarify further the mechanism of action of PUVA treatment and our understanding of the primary pathological process.

Summary Photochemotherapy (PUVA) and methotrexate (MTX) are treatments frequently prescribed in patients with severe psoriasis. However, both treatments have carcinogenic potential. A group of 227 patients, who had been treated with PUVA, MTX or the combination, was investigated for the presence of cancer of the skin. As a control, the partners of these patients were investigated at the same visit. Cancers of the skin were observed in 15 out of 227 psoriatics, compared to one out of 95 control subjects. In the psoriatic patients a previous history of skin cancer, arsenic intake and X–ray treatment were observed more frequently. The increased frequency of skin cancers in psoriatics treated with PUVA, MTX and the combination of both, justifies regular surveillance on skin cancer in these patients.

Disseminated granuloma annulare is an uncommon disorder in which both topical and systemic therapy may have limited success. Anecdotal reports have suggested that PUVA may result in complete clearance of disease; however, maintenance PUVA therapy has usually been required in order to maintain remission. We report the successful treatment of a patient with 5-methoxypsoralen over a 7-month period who remained in remission during a 20-month follow up period.

It is well known that systemic corticosteroids arrest the progress of vitiligo and lead to repigmentation, but they may produce unacceptable side-effects. The use of high-dose prednisolone therapy to minimize the side-effects of systemic steroids has been reported, but there have been no reports on the effectiveness of such treatment combined with phototherapy. We evaluated the efficacy and safety of combination therapy with intravenous prednisolone and psoralen ultraviolet A (PUVA). In 36 patients with vitiligo, intravenous methylprednisolone for 3 days was followed by PUVA twice weekly. After 6 months, vitiligo lesions on the face were reduced in size by 57.5%, on the upper extremities by 34.5%, on the trunk by 30.4% and on the lower extremities by 26.3%. Overall, improvement was seen in 13 patients (36.1%), with >50% repigmentation. Side-effects were mild and transient. We conclude that combination treatment of high-dose prednisolone therapy and PUVA may represent a highly effective therapeutic option for generalized vitiligo.

Summary Background. Psoralen ultraviolet A (PUVA) is the standard photo(chemo)therapeutic regimen for patients suffering from subacute prurigo (SP). Hypothesis. Regarding efficacy, bath PUVA is not superior to medium-dose ultraviolet-A1 (MD-UVA1) and narrowband ultraviolet-B (NB-UVB), which may be considered the new photo(chemo)therapeutic options for SP. Methods. We performed a prospective randomised, controlled, three-arm photo(chemo)therapeutic study. Patients suffering from histopathologically proven SP with a clinical score (PIP score; papules, infiltration and pruritus) of at least 5 points were enrolled into the study. Treatment with bath PUVA was performed 4 times weekly and MD-UVA1 and NB-UVB 5 times weekly. Photo(chemo)therapy was administered over a 4-week period. Outcome measure was the severity of SP investigated by means of the PIP score after 4 weeks of therapy. Results. In total, 33 patients with SP were randomly allocated to photo(chemo)therapy. Bath PUVA (n = 9), MD-UVA1 (n = 11) and NB-UVB (n = 13) resulted in a significant reduction of the baseline PIP score as assessed on the basis of intention-to-treat (ITT) analysis (P = 0.003). However, ITT analysis revealed significantly higher PIP score reduction in patients who were treated with bath PUVA and MD-UVA1 compared with NB-UVB (P

Cyclosporin and mycophenolate mofetil (MMF) are immunosuppressant agents now used frequently in the field of organ transplantation. More recently cyclosporin has been used for the treatment of a number of dermatological conditions, including severe psoriasis and eczema. Extensive diffuse morphoea is very difficult to treat. PUVA, UVA and a number of immunomodulating drugs have been used to attempt improvement but are most beneficial only in early disease. Combination treatments are often used in psoriasis, for example, but are not reported in morphoea. We present the case of a patient treated initially with cyclosporin and PUVA and subsequently with MMF and PUVA, with considerable improvement in his condition.

PUVA is known to be carcinogenic when used in the treatment of psoriasis. To date skin cancer has not been demonstrated after PUVA treatment of vitiligo. We report a patient in whom multiple squamous cell carcinomata and keratoses developed in vitiligo areas after a prolonged course of PUVA.

PUVA is a well-established and effective treatment for plaque stage mycosis fungoides (MF) but its use is limited on a long-term basis because of the risk of cutaneous carcinogenesis. A further disadvantage is that nonexposed areas (sanctuary sites) often develop persistent disease. Therefore it is important to find alternative methods of treatment. Extracorporeal photopheresis (ECP) is a form of photochemotherapy that involves exposure of white blood cells to UVA with psoralens and can be effective in Sezary syndrome and erythrodermic cutaneous T-cell lymphoma. The aim of this study was to compare the efficacy of PUVA and ECP in the treatment of patients with T2 plaque stage (Stage 1B) MF who had a detectable peripheral blood T-cell clone. The study was of a cross-over design. Sixteen patients were randomized to receive either PUVA twice weekly for 3 months followed by ECP once monthly for 6 months at relapse, or vice-versa. Response was assessed by monthly skin scores and peripheral blood T-cell clonality. Ten patients received PUVA initially and six ECP initially. Eight patients completed the study. Skin scores taken at the completion of each treatment arm in patients who completed the study were 113 units better (confidence interval, 42-184 units) following 3 months PUVA than 6 months ECP (P = 0.002). Peripheral blood T-cell clones were detectable in all patients post-treatment. This study indicates that ECP is not effective in the treatment of plaque stage (1B/T2) MF even in patients with molecular evidence of a peripheral blood T-cell clone. Although PUVA was more effective than ECP, neither treatment modality cleared malignant T-cells from the peripheral blood.

Polymorphic light eruption (PLE) is a common condition for which the most effective treatment is prophylactic phototherapy. This is traditionally given in courses of approximately 12–15 exposures. We compared the effect of six and 12 treatments of psoralen-UVA (PUVA) therapy, in the prophylaxis of PLE, in a side-to-side within-patient comparison pilot study. In six out of eight patients, six treatments provided as much protection as 12 treatments. Five of these six patients had reported relatively mild disease in the previous year, in comparison to the other two patients. In view of the inconvenience and increased incidence of adverse effects associated with long treatment courses, we recommend the use of short courses, particularly in patients who are relatively mildly affected.

Concerns were raised in our department when four of our patients receiving PUVA treatment developed internal malignancy. We reviewed the medical and phototherapy case notes of patients who received either systemic or bath PUVA therapy in our department between 1986 and 1999. Among the 197 patients for whom we were able to trace the hospital records we identified five patients with internal malignancies. Over the same period (1986-1999) we calculated, using the Kaplan-Meier nonparametric estimator, that 4.6 cases of internal malignancy would have been anticipated in our study population. Therefore PUVA therapy did not appear to be a risk factor for internal malignancy.

Summary Papuloerythroderma has been described as a distinct clinical entity. It is characterized by pruritus, red flat-topped papules with skin crease sparing, blood eosinophilia and lymphopenia. It has been described almost exclusively in elderly men. We report a case of cutaneous T-cell lymphoma which, at presentation, was indistinguishable from papuloerythroderma, in a 79-year-old male. An excellent clinical response to low dose oral corticosteroids and photochemotherapy was observed. There have only been two previous reports linking papuloerythroderma with T-cell lymphoma. This patient is in keeping with the two previously reported cases and would support the view that papuloerythroderma may predispose to, or in fact represent, an early form of cutaneous T-cell lymphoma.

A 53-year-old woman with systemic sclerosis developed numerous cutaneous nodules, resembling keloids, on her anterior chest wall. The nodules failed to respond to therapy with topical steroid, calcipotriol, or extended photochemotherapy. This is a rare, disfiguring variant of scleroderma, unresponsive to treatment, and poorly understood.

We report a case of a 69-year-old gentleman who presented with a 3-month history of unexplained fevers and malaise who developed generalized pruritus, alopecia and an ichthyosiform erythematous eruption on his forearms, legs, chest and back. Skin histology, immunophenotyping and molecular features were consistent with granulomatous mycosis fungoides. He has been successfully treated with twice weekly PUVA photochemotherapy.

Summary The development of naevus spilus-like hyperpigmentation at sites of resolving plaques of psoriasis has been reported previously.1–4 Although the mechanism is not understood, PUVA therapy has been implicated in most cases. We report an additional case in which lentigines, confined to sites of resolving psoriasis, occurred following oral PUVA. We also describe similar clinical features in two patients who had not received PUVA, which strengthens the observation that this treatment is not a prerequisite for development of this unusual pattern of pigmentation.

A 10-year retrospective analysis of the use of psoralen photochemotherapy (PUVA) in the treatment of vitiligo was undertaken at the St John's Institute of Dermatology, London, UK. Of 97 patients included in this study, eight had complete or almost complete repigmentation, 59 moderate to extensive repigmentation, and 30 showed little or no response. However, 24 of those who had responded to PUVA with extensive repigmentation did not consider their response satisfactory because of persistence of vitiligo at cosmetically sensitive sites, and poorly matching, speckled repigmentation. Fifty-seven patients who initially improved with PUVA therapy subsequently relapsed, in most cases within a year of stopping treatment. Relapses in 22 patients were on the same cutaneous sites as previously affected, while vitiligo at new sites developed in 20 patients and both new and old sites were affected in a further 15 patients. Patients who retained their pigmentation after 2 years appeared to have a better chance of permanent remission. The only statistically significant prognostic indicator of relapse was patient age at the start of treatment, younger patients tending to retain their pigmentation longer than older patients. This study emphasizes the need for careful patient counselling before PUVA therapy as this treatment seldom achieves extensive repigmentation that is cosmetically acceptable, and treatment response is often followed by relapse.