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Among rheumatic diseases in children, a special place belongs to juvenile localized scleroderma, which is highlighted as a separate form in the structure of scleroderma diseases and is characterized by: the onset of the disease up to 16 years, skin lesions, often focal or linear (hemiforma), a tendency to form contractures and the development of limbs abnormalities. This form of the disease is characterized by damage to the skin mainly involving the surrounding muscles. The cause of juvenile localized scleroderma is unclear. Three main components underlie the development of the disease: damage to the vascular bed, activation of the immune system with a violation of its regulation and release of cytokines, as well as altered collagen synthesis with proliferation of fibroblasts and the subsequent development of fibrosis. Several forms of juvenile localized scleroderma have been identified, differing in clinical manifestations and depth of lesion of subjacent tissues, and among the skin manifestations, five subtypes are distinguished: plaque, generalized, linear, pansclerotic, and mixed. Diagnosis is based on an analysis of the history and typical clinical picture of the disease. Specific laboratory tests to confirm the disease has not been developed. Modern treatment principles for juvenile localized scleroderma involve the use of different groups of drugs, including methotrexate, corticosteroids, tacrolimus, cyclosporine, imatinibum, mycophenolate mofetil, retinoids, PUVA therapy (Psoralen + UltraViolet A), use of slow calcium channel antagonists (nifedipinum group), bosentan. The studies currently being conducted in the world on the standardization of basic therapy of juvenile localized scleroderma are likely to reveal the optimal variant and duration of treatment for each form of the disease and improve the prognosis of the disease. The article also presents the results of own clinical observations.

Photochemotherapy with psoralen and ultraviolet A (PUVA) is widely used for refractory skin diseases. Bathwater delivery of 8-methoxypsoralen (8-MOPS) with subsequent UVA irradiation (bath-PUVA) or oral administration of 8-MOPS with UVA is used to treat mycosis fungoides. We retrospectively analyzed 62 patients with mycosis fungoides (8 stage IA, 30 stage IB, 5 stage IIB, 18 stage IIIA, and 1 stage IVA2) treated with bath-PUVA at the Dermatology Clinic of Nagoya City University Hospital from November 2004 to December 2013. A complete response was achieved in 37 (59.7%) patients, a partial response was achieved in 16 (25.8%), and stable disease was achieved in 6 (9.7%). Progressive disease was observed in 3 (4.8%) patients. Almost all patients in stage IA/IB achieved a complete response. Of the 5 stage IIB patients, 2 achieved a partial response, 1 achieved stable disease, and 2 had progressive disease. The serum concentrations of soluble interleukin-2 receptor and lactate dehydrogenase decreased significantly following treatment with bath-PUVA (p

Psoriasis is a chronic, recurrent, and immune-mediated inflammatory disease that affects 2–3% of the world population. The present study aimed to evaluate serum folate level before and after oral PUVA therapy in psoriatic patients. Patient and methods: A total of 40 psoriatic patients without any other morbidities (age ranged from 19 to 63 years, including 20 males and 20 females), All the patients recieved oral 8-methoxypsoralen at a dose of 0.6 mg/kg, followed 2 hours later by ultraviolet A exposure (oral PUVA therapy), For each patient the therapy will be twice weekly for eight weeks. Patients in the PUVA group were treated twice times weekly with a starting dose of 2 J/cm2, increasing by 20% each session, to a maximum of 15 J/cm2 per dose. Patients underwent up to 16 sessions. The dosage was adjusted if patients developed erythema. We have measured serum folate levels at baseline and after oral PUVA exposure. Also we use PASI score as objective method to visualize the relationship between improvement of psoriasis and changes in folate serum levels at baseline and after 16 exposures to PUVA for 8 weeks along with subjective methods which were done by photography and adverse effects were also reported. Results: There is statistically significant decrease of folic acid level after 16 sessions of phototherapy by average 20%. According to PASI score, Our results shows that there is statistically significant improvement in PASI score after phototherapy by average 60% .Conclusion: Folate deficiency should be evaluated and corrected in the patients, as it may be aggravated by UV therapy.

Alopecia areata is a common cause of nonscarring alopecia, with patchy, confluent or diffuse patterns, involving mainly the scalp and other hairy areas of the body. It's considered a therapeutic challenge due to prognosis, unpredictable course and variable efficacy of available therapies. Several studies have evaluated the efficacy of cryotherapy in the treatment of alopecia areata with varying success rates.Superficial cryotherapy using liquid nitrogen mainly was used in several studies for treating alopecia areata with various subtypes including mild, moderate and recalcitrant alopecia areata, and alopecia totalis with variable rates of therapeutically accepted terminal hair regrowth. Its main mechanisms of efficacy in alopecia areata are vascular changes and immunomodulation. Superficial cryotherapy was effective and safe in treating alopecia areata of the eyebrows. It was nearly as effective as topical steroids and topical PUVA therapy, and was combined with topical immunotherapy and topical steroid with increased efficacy. It's well-tolerated, convenient and simple office-based procedure, especially in children who are susceptible to side effects of other conventional therapeutic options.The reported side effects of superficial cryotherapy included vesiculation, erosion, crust formation, transient pigmentary alteration and partial leukotrichia, however they were transient. Key words: alopecia, cryotherapy, liquid nitrogen

UV phototherapy is an essential and efficient therapeutic option in the treatment of dermatological diseases. It is an integral part of multiple guidelines and maintains its high clinical significance despite the development of new therapeutic options for systemic treatment. Due to the difficult revenue situation, the market for ready-to-use products of psoralen and UV therapy devices is constantly changing.

Introduction Narrow band ultraviolet B (NB-UVB) and psoralen-ultraviolet A (PUVA) remain inexpensive and effective anti-psoriatic therapies adopted worldwide with different frequency protocols. We aimed to systematically assess the evidence on the effects of different frequency protocols of phototherapy in treating psoriasis. Evidence acquisition We used the following terms, namely "photochemotherapy", "phototherapy", "psoriasis", "UVB", "UVA" and "ultraviolet therapy", to search the Cochrane Controlled Register of Trials, MEDLINE and Embase databases on August 1, 2019. We organized results using a PRISM diagram and analyzed bias risks with RoB-2 tool. Evidence synthesis We included five randomized controlled trials (RCTs) on oral PUVA and three RCTs on NB-UVB. The five studies on PUVA included a total of 1452 patients with plaque psoriasis and did not find any significant difference in efficacy comparing two- vs. three- vs. four times weekly protocols. The three studies on NB-UVB included a total of 248 patients with plaque psoriasis. No differences in efficacy were reported in comparing different frequencies in delivering NB-UVB, namely twice vs. thrice weekly, twice vs. four times weekly, and thrice- vs. five times weekly protocols. Although protocols with higher treatments frequency per week achieved clearance faster than lower frequency ones, but they did not differ in terms of efficacy. Conclusions PUVA and NB-UVB remain an effective anti-psoriatic treatment; however further studies are needed to elucidate which protocol may be more effective in different skin phototypes.

Clinical reasoning. There is a need to optimize the use of UV-B-311 nm and PUVA-therapy in patients with mycosis fungoides to determine the duration of the treatment regimen, the number of sessions per week, and ultraviolet irradiation regimen. Goal of research. Evaluation of the effect of the type and duration phototherapy on efficacy in patients with mycosis fungoides. Principle. A comparative, non-randomized study of the effectiveness of phototherapy in patients with mycosis fungoides in the early stages. Evaluation of the effectiveness of UV-B-311 nm and PUVA therapy was performed using the BSA index (area of skin lesions) and Modified Severity-Weighted Assessment Tool (mSWAT), as well as according to the criteria proposed by the International Society for Skin Lymphomas (ISCL), the European Organization for the Study and Cancer Treatment (EORTC) and United States Cutaneous Lymphoma Consortium (USCLC). Results. The study included 14 patients with mycosis fungoides, 5 of whom received treatment with UV-B-311 nm, 9 PUVA therapy. A strong correlation was found between the duration of UV-B-311 nm therapy with mSWAT delta (R = 0.90; p = 0.038) and BSA delta (R = 0.90; p = 0.038), while similar correlation was not found in the PUVA-therapy group (mSWAT (R = 0.24; p = 0.527); BSA (R = 0.09; p = 0.823)). When comparing the effectiveness of therapy between the treatment group UV-B-311 nm and PUVA therapy, delta mSWAT and BSA at the 20th procedure, delta mSWAT and BSA after the end of therapy did not have a statistically significant difference between the UVB-311 nm and PUVA groups. Conclusion. Statistically significant correlation was found between the number of procedures and the effectiveness of therapy In the UV-B-311 nm group. An increase in the number of PUVA therapy procedures (after 20) does not lead to a statistically significant increase in the effectiveness of treatment. Additional research is needed to increase the level of evidence of the results and develop optimal phototherapy regimens.

Introduction: Vitiligo is an acquired pigmentary disease, characterized by depigmented macules or patches which distracts the psychosocial behaviour of patients. Various therapeutic modalities have been admintered over time with variable outcome. Psoralen followed by irradiation with UV-A (PUVA) is one of the effective treatment methods for chronic vitiligo cases who had failed to react the other treatment methods. Aim: To assess the efficacy of therapeutic effect of oral psoralen followed by ultraviolet-A irradiation (PUVA) therapy for chronic vitiligo cases. Materials and Methods: A total 36 patients of both sexes with acral, acrofacial and generalised vitiligo with more than 20% spread over body, between age group 15-60 years were recruited. 8 MOP tablets are given with empty stomach according to body weight. All cases were undergone to UVA exposure artificial phototherapy chamber starting with a dose of 4 J/m2 (Dosage depends on Fitzpatrick skin type) over whole body after 90 min. Clinical response was evaluated as marked response rate. Results: Generalised vitiligo was seen in 66.6% cases, acral was seen in 16.6% cases, acrofacial was seen in 16.6% cases. Erythema was the common side effect followed by xerosis, pruritus, nausea and vomiting, bulla and burning. The mean duration of treatment was 14.52 months with mean no of sessions is 62.18. Conclusion: PUVA therapy is a well-established treatment modality for vitiligo with very minimal risk of malignancies and has less side effects like erythema and xerosis. PUVA is safe, effective, and cosmetically acceptable. Generalized vitiligo was responded well to the treatment. Keywords: Psoralen and ultraviolet A (PUVA), Generalised vitiligo, Acral vitiligo, Acrofacial vitiligo.

Introduction: PUVA (psoralen + UV-A) has been used since long time ago in psoriatic patients, which has some adverse effects. Phototherapy in form of Narrowband UVB is advancement in the treatment of psoriasis and it has been found to be same efficacious and having lesser side effects than PUVA. Aim: The study was aimed to compare NBUVB and PUVA in chronic plaque psoriasis in terms of their efficacy and adverse effects. Methods: The study included 43 cases of chronic plaque psoriasis which were randomly divided into two groups. Patients were examined for body surface area involvement and PASI scoring. Narrow-band UV-B phototherapy was given to one group and the other group received PUVA therapy thrice-weekly for a duration of 3 months. Then they were compared for clearance of lesions and side effects. Results: Both groups showed decrease in the PASI scoring by at least 75%. It was observed that lesser number of treatment sessions and fewer days were required for clearance of the lesions in PUVA group than the group receiving NBUVB therapy. But in NBUVB group, mean cumulative clearance dose and adverse reactions were significantly lower. The percentage of patients maintaining remission at six months were more in PUVA group as compared to NBUVB group. Conclusion: Both modes of phototherapy are useful in psoriasis. Patients got clearance of the lesions faster in the PUVA group but the side effects were markedly less in the NBUVB group. Keywords: Narrow-band UV-B, Psoralen UVA, Plaque psoriasis.

Background Phototherapy has been a first-line treatment for early-stage mycosis fungoides (MF) since 1976. Oral 8-methoxypsoralen plus ultraviolet A (oral PUVA) and narrow-band ultraviolet B (NB-UVB) are favorable modalities owing to their availability. In previous studies, phototherapy was conducted thrice per week initially, which is not feasible for many patients. Objectives To evaluate the initial clinical responses and time to relapse in patients with early-stage MF treated with oral PUVA and NB-UVB at a twice-weekly regimen. Methods We reviewed the records of patients with biopsy-proven MF who received oral PUVA or NB-UVB in 2002-2014. Demographic data, staging, response to initial course of phototherapy, and initial relapse-free interval were collected. Results Among 70 patients, 14 (20%) and 56 (80%) were treated with oral PUVA and NB-UVB, respectively. The majority had early-stage MF (IA, 22.9%, IB, 57.1%, and IIA, 4.3%). Oral PUVA led to a complete response (CR) in 2 (14.3%) patients and partial response (PR) in 7 (50%) patients; 17 (30.4%) and 25 (44.6%) patients, respectively, achieved CR and PR with NB-UVB. The number of treatments was similar in both groups. The cumulative dose was 520.7 J/cm2 for PUVA and 41.6 J/cm2 for NB-UVB. There was no initial relapse in the 2 (100%) patients and in 10 (58.8%) patients treated with oral PUVA and NB-UVB at 18 months and 9.14 months of follow-up, respectively. Conclusion Patients with early-stage MF can achieve clinical response with oral PUVA and NB-UVB, with a twice per week regimen. The initial relapse-free interval was longer than 1 year.

Intermediate ultraviolet (UVB) therapy is considered a relatively safe method of treating skin diseases with an autoimmune component in development compared to medical drug methods, including PUVA therapy. This is due to the small depth of penetration of the rays of this wavelength range into skin, which provides a purely local effect on the human body. Excimer lamps are an alternative to the expensive excimer laser for phototherapy of psoriasis or vitiligo. However, for effective phototherapy using UVB lamps, the distance from an emitter to a patient’s skin must be considered. In this paper, we report on treatment of patients using an excimer lamp, the control unit of which is equipped with an optical system for controlling of ultraviolet radiation dose, which allows automatically calculating the time for a set UVB dose. The article describes the results of phototherapy using an excimer lamp of several cases of psoriasis, vitiligo and other forms of dermatitis with a good therapeutic and cosmetic effect. When using an excimer lamp, not a single case of exacerbation of dermatological diseases was established.

Cutaneous T‐cell lymphoma (CTCL) is a chronic condition with low malignancy. International treatment guidelines for CTCL are widely followed in Europe and the USA. Combination therapy with therapeutic agents for CTCL and phototherapy is effective on the basis of European data. The efficacy and safety of combination therapy for Japanese CTCL patients are not established. We investigated the efficacy and safety of combination therapy with photo(chemo)therapy and bexarotene in Japanese CTCL patients. Twenty‐five patients received daily oral bexarotene (300 mg/m2 body surface), followed by bath‐psoralen plus ultraviolet (UV)‐A (PUVA) or narrowband UV‐B. Treatment results were evaluated using the modified Severity‐Weighted Assessment Tool (mSWAT) and the Physician Global Assessment of Clinical Condition (PGA) up to week 24. Safety was also assessed. Twenty‐four weeks after initiating treatment, the total response rate was 80.0% (mSWAT) and 84.0% (PGA). Response rates did not differ when stratified by disease stage. Number of days (mean ± standard deviation) for time to response, duration of response and time to progression determined by the mSWAT were 20.7 ± 9.62, 117.0 ± 43.0 and 163.6 ± 28.8, respectively. T‐helper 2 chemokine levels in patients at stage IIA or more decreased significantly at weeks 12 and 24. All patients experienced adverse events and adverse drug reactions. Serious adverse drug reactions included sepsis, anemia and congestive cardiac insufficiency (n = 1 each). Other adverse drug reactions were of mild to moderate severity. Combination therapy with bexarotene and PUVA was safe and effective in Japanese CTCL patients.

Purpose. To present the results of the treatment of disseminated form of granuloma annulare with remote long-wave ultraviolet therapy and low level laser therapy. Material and methods. Two female patients at the age of 50 and 53 years with complaints of generalised eruptions on the skin of the trunk, upper and lower limbs and a histologically confirmed diagnosis were treated using a combination of remote long-wave ultraviolet therapy and low level laser therapy of infrared spectrum. 16 sessions of remote long-wave ultraviolet therapy and 10 sessions of low level laser therapy were conducted directly on the lesions. Considering the multidirectionality of the mechanisms of action of phototherapy and laser therapy, the procedures were carried out on different days or with many hours long intervals. Results. Combined use of the remote long-wave ultraviolet therapy and laser therapy led to a significant improvement by the 21st day of the treatment. No side effects were noticed during the treatment, tolerance of the sessions was good. Conclusion. Combined use of the remote long-wave ultraviolet therapy and low level laser therapy of infrared spectrum is effective in treatment of disseminated form of granuloma annulare.

The management of psoriasis has evolved considerably over the past 100 years. This has occurred in parallel with our understanding of the pathogenesis of this common, complex and enigmatic disease. It should be celebrated as an outstanding example of successful translational research. With precise targeting of immune pathways for the treatment of psoriasis with new biologics and small molecules has come the realisation that the most effective approach to patient management is a holistic one which encompasses the biopsychosocial nature of the disease. This involves a stratified medicine approach to identifying the best drug for an individual allied to patient education, screening for comorbidity, and regular review as both the clinical presentation and the patient’s needs will change over time. Al­though there is not yet a cure for psoriasis – the whole person, systems approach to patient management, that is in part dependent on early intervention, should help to ensure an optimal outcome.

Introduction: psoriasis is a chronic immune-mediated disease that is associated with several comorbidities, including an increased risk of malignancies, particularly skin cancer. A large number of studies have investigated whether psoriasis itself, psoriasis-associated comorbidities, or psoriasis treatment could lead to an increased risk of neoplasms. Methods: we reviewed the literature using the most important databases (PubMed, MEDLINE, ETHERIA). All articles pertaining to skin cancer associated with psoriasis disease and psoriasis therapy were included. In this review, we also discuss some of the potential underlying mechanisms for these associations, particularly regarding the multiple psoriasis therapies currently available, and their possible implications in higher incidences of skin cancer in these patients. Conclusion: evidence suggests that these patients might have a higher risk of cutaneous malignancies, especially for NMSC, compared with psoriasis-free patients. The reasons for this increased risk remain to be determined. However, high dose PUVA therapy, the immunosuppressive treatments used, and the comorbidities and habits frequently described in these patients seem to play a role in the pathogenesis of these tumors. Because of these facts, periodic screening for skin cancer is recommended in this population.

BACKGROUND Patients of color are disproportionately impacted by vitiligo. Access to treatment depends greatly on insurance coverage. We, therefore, assessed current vitiligo treatment coverage policies across major United States health insurers to determine current patterns and coverage gaps for vitiligo. METHODS The study surveyed 15 commercial health care insurers, 50 BlueCross BlueShield (BCBS) plans, Medicare, Medicaid, and Veterans Affairs. Information on treatment coverage for vitiligo, specifically pimecrolimus and tacrolimus, excimer laser therapy, PUVA, and narrow-band (nb)UVB, was collected via an online review of insurance policy documents, confirmed with phone calls to organization representatives, or via a survey of Medicaid providers, and state Medicaid directors. RESULTS Of 17 organizations with regional or national coverage policies, 12% did not cover topical calcineurin inhibitors, 56% did not cover nbUVB phototherapy, 53% did not cover PUVA phototherapy, and 41% did not cover laser therapy. For BCBS, pimecrolimus and tacrolimus were not covered in 39% and 35% of states, respectively. NbUVB and PUVA therapy were not covered in 20% and 10% of states, respectively. Excimer laser therapy was not covered in 82% of states. Out of 32 states with accessible Medicaid information, 11 did not cover topicals, 5 did not cover nbUVB, 4 did not cover PUVA, and 7 did not cover laser. Two commonly cited reasons for coverage denial were that the treatment indication was considered cosmetic, and certain therapies are not FDA-approved. CONCLUSIONS There is inequity in the distribution of health among vitiligo patients given current patterns of insurance coverage for treatment, which may have disproportionate impact on patients of color.

Currently, phototherapy has been widely used in the treatment of various skin diseases, including mycosis fungoides. The article provides a literature review on the use of various spectra ultraviolet therapy in the treatment of patients with early stages of mycosis fungoides. The mechanisms of action of medium-wave ultraviolet therapy (UVB-311 nm) and PUVA therapy, causing the immunosuppressive, anti-inflammatory and anti-proliferative effect, are described. The results of retrospective studies of PUVA therapy and UVB-311 nm efficacy in patients with cutaneous T-cell lymphoma are presented, as well as modern literature evidence of the need for standardization of phototherapy methods for patients with mycosis fungoides.

The word Shvitra means in Sanskrit is Shvitra, which is white patch, without Kandu and inflammation, it present on anywhere of body, In this disease white spots or white discoloration takes place on the skin so it is named as Shvitra. Vitiligo is a long-term condition where pale white patches develop on the skin. It's caused by the lack of melanin, a pigment in the skin. In modern Science PUVA therapy and corticosteroid are used for treatment of disease but they may cause very harmful effect like skin burn, nausea dermatoheliosis that is ultraviolet ageing of skin. There is need Safe easier less complicate cost effective Ayurvedic treatment. Abhyanga stimulates Bhrajjak pitta which is present in the skin and helps restoring color. Jyotishamati Taila has extra medicinal property so it treats disease more comfortably.

Introducao: A fototerapia, em particular a fotoquimioterapia com psoralenos associados a radiacoes ultravioleta do tipo A (PUVA) e a fototerapia com radiacoes ultravioleta do tipo B de banda estreita (nbUVB), e um dos pilares do tratamento da micose fungoide (MF). Metodos: Foram analisados os resultados de doentes com MF que fizeram tratamento de primeira linha com PUVA ou nbUVB na Unidade de Fotodermatologia do Servico de Dermatologia do Centro Hospitalar Universitario do Porto, de 2007 a 2017. O resultado primario foi a resposta ao tratamento. O resultado secundario foi a sobrevida livre de recidiva. Resultados: De um total de 34 doentes com MF, nove (26,5%) foram diagnosticados no estadio IA e 25 (73,5%) no estadio IB. Destes doentes, 30 (88,2%) foram tratados com PUVA e quatro (11,8%) com nbUVB. Globalmente, 24 (80%) doentes tratados com PUVA e dois (50%) doentes tratados com nbUVB tiveram respostas completas. Foi observada recidiva em 17 (70,8%) doentes tratados com PUVA e em dois (100%) doentes tratados com nbUVB. A mediana da sobrevida livre de recidiva foi de 12 meses para o grupo PUVA e 6 meses para o grupo nbUVB. Conclusao: A fotoquimioterapia com PUVA e uma terapeutica eficaz na MF em estadios precoces. O UVB de banda estreita pode ser uma alternativa terapeutica valida para estes doentes. Todavia, a amostra reduzida do grupo nbUVB neste estudo limita as conclusoes sobre a sua eficacia.

Background Mycosis fungoides is the most common type of primary cutaneous T cell lymphomas. Doxycycline promoted apoptosis in different human malignant cell lines and in vivo models. Objectives To test for the therapeutic efficacy of doxycycline in comparison to PUVA in early stages of classic MF and its effect on T cell apoptosis. Methods Thirty-six patients were randomized into either: doxycycline 200 mg daily (n = 18) or PUVA (3 weekly sessions) (n = 18) for 12 weeks. The primary outcome (therapeutic efficacy) was defined in terms of objective response rate (ORR) which was measured according to changes in the modified severity weighted assessment tool (mSWAT). Results Doxycycline achieved significantly less ORR (partial response) in comparison to PUVA (11.1%, 50%, respectively, p = .016). The percent reduction in mSWAT, CAILS, histopathology score and CD3 expression was significantly less in the doxycycline group (p = .001, p = .001, p ˂ .001, and p = .004, respectively). Within the doxycycline group, changes in mSWAT and CAILS showed no correlation with changes in the CD3 or Bcl-2 expression. Gastric upset was significantly more encountered in the doxycycline group (p = .001). Conclusion Doxycycline is not suitable as a sole agent in the treatment of early stages of classic MF, acting mainly by anti-inflammatory rather apoptotic function. Register number NCT03454945 (www.clinicaltrials.gov).

Background: Chronic acrodermatitis continua of Hallopeau (ACH) is a rare form of pustular psoriasis predominantly affecting the distal phalanges of the fingers and toes. The disease manifests by pustular rash with marked infiltration, fissures, and often results into severe dystrophy of nail plates. ACH is refractory to most of psoriasis standard of care (SOC) therapies. Objective: The objective of this study is to assess the prospects of secukinumab therapy of ACH based on current clinical observation. Methods: We observed a female patient with ACH. Number of SOC treatments were applied in that case including local PUVA therapy, systemic retinoids, methotrexate, and biologic agents. Result: Secukinumab, a IL-17 inhibitor, demonstrated pronounced clinical effect in the case of ACH refractory to other SOC therapies. Conclusion: IL-17 inhibition provided by secukinumab was linked to clinically meaningful improvement in the heavily pretreated ACH. Further exploration and clinical studies may be important to provide more data on secukinumab effects in ACH.

Nevus depigmentosus (ND), also known as nevus achromicus or achromic nevus, is an uncommon congenital hypomelanosis of the skin that is often characterized as being nonprogressive and having serrated borders. It needs to be distinguished from other hypopigmented skin conditions such as nevus anemicus, hypomelanosis of Ito, Fitzpatrick patches (ash leaf spots) of tuberous sclerosis, vitiligo, indeterminate leprosy, and pigment demarcation lines. Treatment may be desired for aesthetic and possible psychosocial considerations. We review and update knowledge about ND and its simulants.

Diagnosis of mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma (CTCL), can be challenging given the overlapping clinical and pathologic features with various other conditions. High-throughput TCR sequencing (HT-TCRseq) has an outstanding capacity to facilitate diagnosis of CTCL1 , identify aggressive forms of MF in early-stage2 , and associate burden of malignant cells with response to PUVA treatment3 .

T-cell lymphomas (TCLs) constitute a rare subset of non-Hodgkin lymphomas, with mycosis fungoides/Sezary syndrome (MF/SS) being the most common subtype of cutaneous TCLs (CTCLs). Considered an incurable but treatable disease, MF/SS management presents several challenges including diagnostic delays, debilitating effect on patients' quality of life, need for several lines of therapies, multidisciplinary care and cumulative drug toxicities limiting duration of use. The present review intends to provide an overview of the recent advances in our understanding of the biology of CTCL and how these are being leveraged to provide additional treatment options for management of advanced and recurrent disease. In addition, the discussion of the different modalities of treatment is summarised to further outline the importance of multidisciplinary care and early referral to CTCL centres.

Patients suffering from alopecia areata (AA) can lose hair in focal regions, the complete scalp, including eyelashes and eyebrows, or even the entire body. The exact pathology is not yet known, but the most described theory is a collapse of the immune privilege system, which can be found in some specific regions of the body. Different treatment options, local and systemic, are available, but none of them have been proven to be effective in the long term as well for every treatment there should be considered for the possible side effects. In many cases, treated or non-treated, relapse often occurs. The prognosis is uncertain and is negatively influenced by the subtypes alopecia totalis and alopecia universalis and characteristics such as associated nail lesions, hair loss for more than 10 years and a positive familial history. The unpredictable course of the disease also makes it a mental struggle and AA patients are more often associated with depression and anxiety compared to the healthy population. Research into immunology and genetics, more particularly in the field of dendritic cells (DC), is recommended for AA as there is evidence of the possible role of DC in the treatment of other autoimmune diseases such as multiple Sclerosis and cancer. Promising therapies for the future treatment of AA are JAK-STAT inhibitors and PRP.

Background Phototherapy is a mainstay for the treatment of MF. However, there is scarce evidence for its use, mostly due to the lack of a unified schedule. Aims The primary aim of this study was to establish the first structured, expert-based consensus regarding the indications and technical schedules of NB-UVB and PUVA for MF. The secondary aim was to determine the consensus level for each specific item. Materials & methods E-delphi study. Item-specific expert consensus was defined as the number of "Totally Agree" results to ≥80% of the panelists. Cronbach alpha index ≥0.7 was used as a measure of homogeneity in the responses among questions related to the same topic. Results Overall, there was a high homogeneity among responders (0.78). On specific topics, the highest grade was observed for technical items (0.8) followed by indications for early (0.73) and advanced stages (0.7). Conclusions Items related to the most canonical indications of phototherapy and to treatment schedules showed the highest agreements rates. There is consensus about the use of standardized treatment schedules for the induction and consolidation phases for NB-UVB and PUVA in MF.

BACKGROUND: Mycosis fungoides (MF) is the most common type of cutaneous T‐cell lymphoma, a malignant, chronic disease initially affecting the skin. Several therapies are available, which may induce clinical remission for a time. This is an update of a Cochrane Review first published in 2012: we wanted to assess new trials, some of which investigated new interventions. OBJECTIVES: To assess the effects of interventions for MF in all stages of the disease. SEARCH METHODS: We updated our searches of the following databases to May 2019: the Cochrane Skin Specialised Register, CENTRAL, MEDLINE, Embase, and LILACS. We searched 2 trials registries for additional references. For adverse event outcomes, we undertook separate searches in MEDLINE in April, July and November 2017. SELECTION CRITERIA: Randomised controlled trials (RCTs) of local or systemic interventions for MF in adults with any stage of the disease compared with either another local or systemic intervention or with placebo. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by Cochrane. The primary outcomes were improvement in health‐related quality of life as defined by participants, and common adverse effects of the treatments. Key secondary outcomes were complete response (CR), defined as complete disappearance of all clinical evidence of disease, and objective response rate (ORR), defined as proportion of patients with a partial or complete response. We used GRADE to assess the certainty of evidence and considered comparisons of psoralen plus ultraviolet A (PUVA) light treatment as most important because this is first‐line treatment for MF in most guidelines. MAIN RESULTS: This review includes 20 RCTs (1369 participants) covering a wide range of interventions. The following were assessed as either treatments or comparators: imiquimod, peldesine, hypericin, mechlorethamine, nitrogen mustard and intralesional injections of interferon‐α (IFN‐α) (topical applications); PUVA, extracorporeal photopheresis (ECP: photochemotherapy), and visible light (light applications); acitretin, bexarotene, lenalidomide, methotrexate and vorinostat (oral agents); brentuximab vedotin; denileukin diftitox; mogamulizumab; chemotherapy with cyclophosphamide, doxorubicin, etoposide, and vincristine; a combination of chemotherapy with electron beam radiation; subcutaneous injection of IFN‐α; and intramuscular injections of active transfer factor (parenteral systemics). Thirteen trials used an active comparator, five were placebo‐controlled, and two compared an active operator to observation only. In 14 trials, participants had MF in clinical stages IA to IIB. All participants were treated in secondary and tertiary care settings, mainly in Europe, North America or Australia. Trials recruited both men and women, with more male participants overall. Trial duration varied from four weeks to 12 months, with one longer‐term study lasting more than six years. We judged 16 trials as at high risk of bias in at least one domain, most commonly performance bias (blinding of participants and investigators), attrition bias and reporting bias. None of our key comparisons measured quality of life, and the two studies that did presented no usable data. Eighteen studies reported common adverse effects of the treatments. Adverse effects ranged from mild symptoms to lethal complications depending upon the treatment type. More aggressive treatments like systemic chemotherapy generally resulted in more severe adverse effects. In the included studies, CR rates ranged from 0% to 83% (median 31%), and ORR ranged from 0% to 88% (median 47%). Five trials assessed PUVA treatment, alone or combined, summarised below. There may be little to no difference between intralesional IFN‐α and PUVA compared with PUVA alone for 24 to 52 weeks in CR (risk ratio (RR) 1.07, 95% confidence interval (CI) 0.87 to 1.31; 2 trials; 122 participants; low‐certainty evidence). Common adverse events and ORR were not measured. One small cross‐over trial found once‐monthly ECP for six months may be less effective than twice‐weekly PUVA for three months, reporting CR in two of eight participants and ORR in six of eight participants after PUVA, compared with no CR or ORR after ECP (very low‐certainty evidence). Some participants reported mild nausea after PUVA but no numerical data were given. One participant in the ECP group withdrew due to hypotension. However, we are unsure of the results due to very low‐certainty evidence. One trial comparing bexarotene plus PUVA versus PUVA alone for up to 16 weeks reported one case of photosensitivity in the bexarotene plus PUVA group compared to none in the PUVA‐alone group (87 participants; low‐certainty evidence). There may be little to no difference between bexarotene plus PUVA and PUVA alone in CR (RR 1.41, 95% CI 0.71 to 2.80) and ORR (RR 0.94, 95% CI 0.61 to 1.44) (93 participants; low‐certainty evidence). One trial comparing subcutaneous IFN‐α injections combined with either acitretin or PUVA for up to 48 weeks or until CR indicated there may be little to no difference in the common IFN‐α adverse effect of flu‐like symptoms (RR 1.32, 95% CI 0.92 to 1.88; 82 participants). There may be lower CR with IFN‐α and acitretin compared with IFN‐α and PUVA (RR 0.54, 95% CI 0.35 to 0.84; 82 participants) (both outcomes: low‐certainty evidence). This trial did not measure ORR. One trial comparing PUVA maintenance treatment to no maintenance treatment, in participants who had already had CR, did report common adverse effects. However, the distribution was not evaluable. CR and OR were not assessable. The range of treatment options meant that rare adverse effects consequently occurred in a variety of organs. AUTHORS' CONCLUSIONS: ​​There is a lack of high‐certainty evidence to support decision making in the treatment of MF. Because of substantial heterogeneity in design, missing data, small sample sizes, and low methodological quality, the comparative safety and efficacy of these interventions cannot be reliably established on the basis of the included RCTs. PUVA is commonly recommended as first‐line treatment for MF, and we did not find evidence to challenge this recommendation. There was an absence of evidence to support the use of intralesional IFN‐α or bexarotene in people receiving PUVA and an absence of evidence to support the use of acitretin or ECP for treating MF. Future trials should compare the safety and efficacy of treatments to PUVA, as the current standard of care, and should measure quality of life and common adverse effects.

Background and objectives Primary cutaneous lymphomas (PCL) often strongly differ in clinical behavior and prognosis from systemic lymphomas of the same histopathologic type. The aim of the study was to investigate the distribution of PCL subtypes, the average time from disease manifestation to diagnosis, the importance of diagnostic procedures, the occurrence of secondary malignancies and the different treatment modalities. Patients and methods Retrospective analysis of 152 patients with PCL examined at the Department of Dermatology of the University Hospital Tubingen from 2010-2012. Results 105 patients with CTCL (69.1 %) and 47 patients with CBCL (30.9 %) were included. The average time from disease manifestation to diagnosis was four years. The most common diagnosed lymphoma was mycosis fungoides (MF) (47.4 %). First-line therapies here include phototherapy only (psoralen-UV-A [PUVA], n = 48; UVB 311 nm, n = 7) or combination therapies primarily phototherapy with systemic retinoids (n = 18). Most frequent second-line therapy was interferon (INF)-α plus PUVA (n = 15). The outcome was favorable (45.2 % remission, 28.6 % stable disease, 22.6 % progressive disease). Malignant comorbidities were observed more frequently compared to a healthy control group. Conclusions The diagnosis of lymphoma often takes several years. The value of staging procedures is still low and the treatment modalities for MF in earlier stages are mainly based on phototherapy.

Postburn leukoderma is challenging to treat with different surgical and nonsurgical treatments resulting in variable outcomes. We report a case of a 56-year-old female with postburn leukoderma treated successfully with topical daylight psoralen UVA therapy for 6 months. The treatment was well tolerated and showed excellent improvement. In conclusion, we report the successful use of topical daylight PUVA for postburn leukoderma with almost complete repigmentation. This is a simple, convenient, and cheap nonsurgical treatment option.

Vitiligo is a relatively common disorder characterized by areas of depigmented skin. It may be associated with social stigma and adversely affects the quality of life. Although many treatment options are available, none is curable. The search continues for an effective therapeutic option. New targeted options include biologics and other immunomodulatory agents, with varying degrees of evidence. We have discussed briefly the therapeutic options with special emphasis on the newer immunomodulatory agents. We undertook a comprehensive English literature search across multiple databases such as PubMed, SCOPUS, EMBASE, MEDLINE, and Cochrane using keywords (alone and in combination) and MeSH items such as "vitiligo," "treatment," "recent," and "immunomodulators" to obtain several relevant articles, priority being given to prospective randomized controlled trials. We scanned all the relevant articles and summarized them to obtain the latest information about the treatment of this condition to prepare the current article.

The aim of this prospective study in a phototherapy unit was to describe adverse events (AEs) associated with discontinuation of phototherapy in a clinical setting. A total of 872 included patients received 1,256 courses of phototherapy treatment: 76.9% narrowband UVB (NBUVB); 9.6% systemic psoralen plus UVA (PUVA); 11.4% topical PUVA; and 2.1% UVA. Approximately a fifth of the treatments (n= 240, 19.1%) were associated with AEs, the most frequent of which was erythema (8.8%). Systemic PUVA had the highest rate of AEs (32.5%). Mycosis fungoides was the dermatosis with the highest rate of AE (36.9%). A total of 216 (17.2%) patients stopped treatment: 23.6% because of AEs (4.1% of all treatments). Treatment suspension due to AEs was associated with PUVA, both topical and systemic (p < 0.001), and diagnoses of mycosis fungoides (p < 0.001), palmoplantar psoriasis (p = 0.002), hand eczema (p = 0.002) and pityriasis lichenoides (p = 0.01). In conclusion, one in every 5 patients receiving phototherapy had an AE, but few stopped treatment for this reason.

Graft-versus-host disease (GvHD) is a common complication of allogeneic hematopoietic stem cell transplantation (allo-HSCT) with high morbidity and mortality rates. The purpose of this study was to demonstrate the efficacy of Manual Lymph Drainage (MLD) and a home-based exercise programme on range of motion (ROM) and flexibility in a patient diagnosed with chronic cutaneous GvHD. A 29-year-old male who was diagnosed as acute lymphoblastic leukemia underwent allo-HSCT after induction chemotherapy. He developed extended chronic cutaneous skin GvHD. He received systemic immunosuppressive treatment and Psoralen and ultraviolet radiation (PUVA) for 20 sessions. He was then consulted to physiotherapy department for the limitation of multiple ROM due to severe GvHD. The range of motions of shoulder, elbow, hip, knee and ankle joints were evaluated with universal goniometer. The chair sit, reach and back scratch tests were performed. MLD was applied for 2 weeks. Additionally, exercise recommendations were maintained as a home-programme. After the therapy, ROM values were better in wrist extension and hip abduction/adduction and the back scratch test result improved. According to chair sit and reach tests, the results decreased from 25 to 22 cm distance after 2 weeks. With MLD treatment with exercise, ROM has been preserved and even improved in this refractory case. In addition, the flexibility test results were found to be increased. The efficacy of MLD and exercise in chronic cutaneous GvHD should be investigated in further studies. (C) 2019 Elsevier Masson SAS. All rights reserved.

Background Chronic plaque psoriasis is an immune‐mediated, chronic, inflammatory skin disease, which can impair quality of life and social interaction. Disease severity can be classified by the psoriasis area and severity index (PASI) score ranging from 0 to 72 points. Indoor artificial salt bath with or without artificial ultraviolet B (UVB) light is used to treat psoriasis, simulating sea bathing and sunlight exposure ; however, the evidence base needs clear evaluation. Objectives To assess the effects of indoor (artificial) salt water baths followed by exposure to artificial UVB for treating chronic plaque psoriasis in adults. Search methods We searched the following databases up to June 2019: the Cochrane Skin Group Specialised Register, CENTRAL, MEDLINE, Embase, and LILACS. We also searched five trial registers, and checked the reference lists of included studies, recent reviews, and relevant papers for further references to relevant trials. Selection criteria Randomised controlled trials (RCTs) of salt bath indoors followed by exposure to artificial UVB in adults who have been diagnosed with chronic plaque type psoriasis. We included studies reporting between‐participant data and within‐participant data. We evaluated two different comparisons: 1) salt bath + UVB versus other treatment without UVB ; eligible comparators were exposure to psoralen bath, psoralen bath + artificial ultraviolet A UVA) light, topical treatment, systemic treatment, or placebo, and 2) salt bath + UVB versus other treatment + UVB or UVB only ; eligible comparators were exposure to bath containing other compositions or concentrations + UVB or UVB only. Data collection and analysis We used standard methodological procedures expected by Cochrane. We used GRADE to assess the certainty of the evidence. The primary efficacy outcome was PASI‐ 75, to detect people with a 75% or more reduction in PASI score from baseline. The primary adverse outcome was treatment‐related adverse events requiring withdrawal. For the dichotomous variables PASI‐75 and treatment‐ related adverse events requiring withdrawal, we estimated the proportion of events among the assessed participants. The secondary outcomes were health‐related quality of life using the Dermatology Life Quality Index, (DLQI) pruritus severity measured using a visual analogue scale, time to relapse, and secondary malignancies. Main results We included eight RCTs: six reported between‐participant data (2035 participants ; 1908 analysed), and two reported within‐ participant data (70 participants, 68 analysed ; 140 limbs ; 136 analysed). One study reported data for the comparison salt bath with UVB versus other treatment without UVB ; and eight studies reported data for salt bath with UVB versus other treatment with UVB or UVB only. Of these eight studies, only five reported any of our pre‐ specified outcomes and assessed the comparison of salt bath with UVB versus UVB only. The one included trial that assessed salt bath plus UVB versus other treatment without UVB (psoralen bath + UVA) did not report any of our primary outcomes. The mean age of the participants ranged from 41 to 50 years of age in 75% of the studies. None of the included studies reported on the predefined secondary outcomes of this review. We judged seven of the eight studies as at high risk of bias in at least one domain, most commonly performance bias. Total trial duration ranged between at least two months and up to 13 months. In five studies, the median participant PASI score at baseline ranged from 15 to 18 and was balanced between treatment arms. Three studies did not report PASI score. Most studies were conducted in Germany ; all were set in Europe. Half of the studies were multi‐ centred (set in spa centres or outpatient clinics) ; half were set in a single centre in either an unspecified settings, a psoriasis daycare centre, or a spa centre. Commercial spa or salt companies sponsored three of eight studies, health insurance companies funded another, the association of dermatologists funded another, and three did not report on funding. When comparing salt bath plus UVB versus UVB only, two between‐ participant studies found that salt bath plus UVB may improve psoriasis when measured using PASI 75 (achieving a 75% or more reduction in PASI score from baseline) (risk ratio (RR) 1.71, 95% confidence interval (CI) 1.24 to 2.35 ; 278 participants ; low‐certainty evidence). Assessment was conducted at the end of treatment, which was equivalent to six to eight weeks after start of treatment. The two trials which contributed data for the primary efficacy outcome were conducted by the same group, and did not blind outcome assessors. The German Spas Association funded one of the trials and the funding source was not stated for the other trial. Two other between‐ participant studies found salt bath plus UVB may make little to no difference to outcome treatment‐related adverse events requiring withdrawal compared with UVB only (RR 0.96, 95% CI 0.35 to 2.64 ; 404 participants ; low‐certainty evidence). One of the studies reported adverse events, but did not specify the type of events ; the other study reported skin irritation. One within‐ participant study found similar results, with one participant reporting severe itch immediately after Dead Sea salt soak in the salt bath and UVB group and two instances of inadequate response to phototherapy and conversion to psoralen bath + UVA reported in the UVB only group (low‐certainty evidence). Authors' conclusions Salt bath with artificial ultraviolet B (UVB) light may improve psoriasis in people with chronic plaque psoriasis compared with UVB light treatment alone, and there may be no difference in the occurrence of treatment‐ related adverse events requiring withdrawal. Both results are based on data from a limited number of studies, which provided low‐ certainty evidence, so we cannot draw any clear conclusions. The reporting of our pre‐specified outcomes was either non‐existent or limited, with a maximum of two studies reporting a given outcome. The same group conducted the two trials which contributed data for the primary efficacy outcome, and the German Spas Association funded one of these trials. We recommend further RCTs that assess PASI‐75, with detailed reporting of the outcome and time point, as well as treatment‐related adverse events. Risk of bias was an issue ; future studies should ensure blinding of outcome assessors and full reporting.

Psoralen ultraviolet A (PUVA) therapy has thrived as a promising treatment for psoriasis. However, overdose of PUVA treatment will cause side-effects, such as melanoma formation. And these side-effects are often ignored during PUVA therapy. Hence, in situ monitoring therapeutic response of PUVA therapy is important to minimize side-effects. Aberrant expression of tyrosinase (TYR) has been proved to be associated with melanoma, indicating that TYR is a potential target for evaluation of PUVA therapy. Herein, we reported a strategy for in situ monitoring TYR activity during PUVA therapy by using a cell-array chip-based SERS platform. The cell-array chip was used to simulate cell survival environment for cell culture. Capture of single cells and living cell analysis were realized in the isolated microchambers. An enzyme-induced core-shell self-assembly substrate was used to evaluate TYR activity in living cells during PUVA therapy. The gold nanoparticle modified with a SERS reporter, 4-mercaptobenzonitrile (4-MBN), was used as the core. In the presence of oxygen and TYR, hydroxylation of l -tyrosine occurred, leading to the reduction of silver ion on the surface of gold cores. The growth of silver shells was accompanied by the increased SERS intensity of the reporter, which is related directly to TYR activity. The detection limit for TYR activity is 0.45 U/mL. Upregulation of TYR activity was successfully monitored after PUVA therapy. Notably, real-time and in situ information of therapeutic response can be obtained through monitoring PUVA therapy by using a cell-array chip-based SERS platform, which has great potential to guide the clinical application of PUVA therapy.

The review presents modern data on the role of ultraviolet (UV) radiation in the pathogenesis of non-melanoma skin cancer (NMSC), the problem of THE risk of developing NMSC, in particular, squamous cell and basal cell skin cancer both in the population and in long-term repeated irradiation of phototherapy (PUVA therapy, UVB therapy, UVB-311 therapy) in patients with psoriasis. The paper considers the mechanisms of UV-induced cell damage by different spectral ranges (UVA, UVB) including the formation of photoproducts, damage to genomic DNA and other cellular structures, violation of the regulation of signaling pathways, the development of chronic inflammation, secondary immunosuppression. The review summarizes the results of large epidemiological studies discussing the role of gene polymorphisms in the homologous DNA repair XRCC3, gene telomerase TERT-CLPTMI, cytokine IL10 gene, MTHFR gene, encoding the folate synthesis, genes involved in pigmentirovanie MC1R, EXOC2, UBAC2 in the modulation of risk of carcinogenic effect of UV radiation. According to the authors’ opinion, the most vital and significant is data on the role of vitamin D receptor (VDR) gene polymorphisms as possible predictors of the risk of NMSC development. The further prospects of academic research on the cumulative role of the genome and environmental factors in the risk assessment of NMSC are revealed.

Blisters associated with PUVA treatments are an adverse effect of photochemotherapy that has been reported in the literature. Asymptomatic blisters appear spontaneously mainly on the lower limbs and resolve without treatment. The differential diagnoses to consider include a phototoxic reaction, pseudoporphyria, and PUVA-induced bullous pemphigoid. We describe the clinical and histologic features in 5 cases of blistering secondary to PUVA treatment. If this adverse effect is accurately diagnosed, photochemotherapy need not be interrupted, and unnecessary diagnostic procedures and additional treatments can be avoided.

No abstract available.

Aims To determine the role of total skin electron irradiation (TSEI) as a cause of second malignancies in mycosis fungoides patients. Materials and methods Mycosis fungoides patients referred to TSEI were followed in a longitudinal study. Other diagnosed malignancies were obtained after cross-matching with the Israel National Cancer Registry database. Results Between 1974 and 2010, 197 patients were treated: 134 (68%) men, 63 (32%) women; mean age 58 ± 17years. Topical/systemic treatment was given to 134 (68%) patients. TSEI was given to 104 (68.9%) patients. Seven (4.6%) received sub-TSEI and 40 (26.5%) received focal electron irradiation fields. Forty-six (23%) patients did not receive radiotherapy. The second primaries rate was 6.7 times higher in male mycosis fungoides patients and 13.1 times higher in female mycosis fungoides patients than in the general Israeli population. Malignant melanoma developed in eight patients after radiotherapy, in one patient without irradiation. The skin-related cancer rate after irradiation versus no irradiation was higher (P = 0.018). Combination radiotherapy with psoralen + ultraviolet A and/or nitrogen mustard yielded 11 cases of skin cancer versus no cases without irradiation. Conclusions Mycosis fungoides patients have a high incidence of sequential malignancies. TSEI is associated with higher ‘skin-related cancer’ rates. Close longitudinal follow-up of mycosis fungoides patients is obligatory.

Data on the treatment of early folliculotropic mycosis fungoides, a recently defined clinicopathological subgroup of folliculotropic mycosis fungoides with an indolent course, is limited. Treatment outcomes were studied in a retrospective cohort of 47 adults with early folliculotropic mycosis fungoides, with a focus on psoralen plus ultraviolet A (PUVA) monotherapy, including dosimetric data, and the findings were compared with data for PUVA in 18 adults with early-classic mycosis fungoides. PUVA was given to 27 patients with early folliculotropic mycosis fungoides: 70% achieved complete response and 26% partial response. Significantly more treatments were needed to achieve complete response in stage IB compared with stage IA. There was no significant difference in the complete response rate from classic plaque-stage disease, although the early folliculotropic mycosis fungoides group required more treatments to achieve complete response, and a higher cumulative dose of UVA. Thus, PUVA is an effective treatment for early folliculotropic mycosis fungoides. Its complete response rate might be equal to early-classic mycosis fungoides; however, a longer induction phase is needed to achieve complete response.

Prurigo nodularis occurs with chronic pruritus and the presence of single to multiple symmetrically distributed, hyperkeratotic, and intensively itching nodules. Diverse dermatologic, systemic, neurologic, or psychiatric conditions can lead to prurigo nodularis. Structural analysis demonstrated a reduced intraepidermal nerve fiber density and increased dermal levels of nerve growth factor and neuropeptides such as substance P and calcitonin gene-related peptide. Novel therapy concepts such as inhibitors at neurokinin-1, opioid receptors, and interleukin-31 receptors have been developed. The mainstays of prurigo nodularis therapy comprise topical steroids, capsaicin, calcineurin inhibitors, phototherapy, and the systemic application of anticonvulsants, μ-opioid receptor antagonists, or immunosuppressants.

Background Fumaric acid esters (FAEs) are an established systemic treatment for moderate‐to‐severe psoriasis. However, the long‐term clinical safety and effectiveness of continuous FAE monotherapy and combination therapy have not been established. Objective To examine the long‐term safety and effectiveness of FAEs as monotherapy and in combination with phototherapy or methotrexate in patients with psoriasis treated at a single centre in Germany. Methods This monocentric, retrospective observational study, with a follow‐up period of up to 32.5 years, included 859 patients: 626 received FAE monotherapy, 123 received FAEs with concomitant phototherapy and 110 received FAEs with methotrexate. Results Approximately half of patients (49.0%) reported adverse events (566 total events), most of which involved the gastrointestinal tract. Serious adverse events were reported in 2.3% of patients, but none were deemed to have a causal relationship with any of the treatment regimens. Adverse events leading to treatment discontinuation were observed in 12.9% of patients. A median duration of 1 year was observed in all three treatment subcohorts (P = 0.70) from initiation of FAE treatment to a 50% response rate, where response was defined as achieving a cumulative static Physician's Global Assessment (PGA) score of ‘light’ and at least a 2‐point reduction in baseline PGA. A 50% response rate for the cumulative Psoriasis Area and Severity Index 75 was achieved in the FAE monotherapy subcohort after a median of 3 years of treatment, in the FAEs + phototherapy subcohort after 6.7 years and in the FAEs + methotrexate subcohort after 8.1 years (P = 0.001). Conclusion According to our data, FAEs as monotherapy or in combination with phototherapy or methotrexate are safe and beneficial for long‐term clinical use. However, multicentre, randomized controlled trials are required to establish the clinical value of monotherapy versus combination therapy and the optimal treatment duration.

Generalized pustular psoriasis (GPP) is a subtype of pustular psoriasis characterized by painful and occasionally disfiguring cutaneous manifestations with sepsis-like systemic symptoms. Affecting any age and race, GPP can occur with other forms of psoriasis or by itself. Stimuli for flares include medications, infections and environmental triggers. The interleukin family and caspase recruitment domain family have been implicated in its pathogenesis. Other forms of pustular psoriasis include impetigo herpetiformis, palmoplantar pustular psoriasis, annular pustular psoriasis and acrodermatitis continua of Hallopeau. Treatment is not well established, but includes the use of retinoids, methotrexate, cyclosporine, corticosteroids, TNF-alpha inhibitors, topical therapy and phototherapy. The use of TNF-alpha inhibitors may result in the formation of antidrug antibodies and should be administered with methotrexate.

Background: While phototherapy is a well-established treatment for many dermatoses, data from the literature regarding its use in elderly patients are quite limited. Objective: In this study, we aimed to determine the phototherapy indications in geriatric patients and to evaluate the effectiveness and reliability of phototherapy in this group. Methods: This study included 95 patients of 65 years of age and older who were treated in our phototherapy unit between 2006 and 2015. The data for this study were collected retrospectively from patient follow-up forms in the phototherapy unit. Results: Phototherapy was administered to 28 (29.5%) patients for mycosis fungoides, 25 (26.3%) patients foplaque type psoriasis, 12 (12.6%) patients for palmoplantar psoriasis, 12 (12.6%) patients for generalized pruritus, and 18 (19%) for other dermatoses. Of the patients, 64.2% had received a narrowband UVB (NB-UVB), 21.1% oral psoralen UVA (PUVA), and 14.7% local PUVA treatment. A complete response was achieved in 76.9-85.7% of the mycosis fungoides and in 73.71-100% of the psoriasis vulgaris patients treated with NB-UVB and PUVA, respectively. All the patients with generalized pruritus were treated with NB-UVB, and 80% of these patients achieved significant improvement. The erythema rate was found to be 0.43% per session for NB-UVB treatment and 0.46% per session for PUVA treatment as a side effect. Study limitations: The limitations of our study are that it was retrospective and the remission durations of the patients are not known. Conclusion: This study showed that phototherapy is effective and reliable in the elderly population with proper dose increases and close follow-up.

Background Mycosis fingoides (MF) is the most common subtype of primary cutaneous T-cell lymphomas. Current evaluation of disease extent and severity is based on mSWAT scoring system, which seems to be relatively subjective. The aim of this subject was to present the usefulness of 20 MHz in objective 5-year long monitoring of response to therapy in MF patients. Materials and methods The 5-years long follow-up based on 19 skin USG images of patients diagnosed as early stages of MF was studied. The assessed USG parameter was the mean diameter of subepidermal low echogenic band (SLEB). Results In every MF patient during exacerbation within lesional skin we could observe SLEB, which thinning or complete disappearance was detected after finishing the therapy. Lack of complete absence of SLEB was related to the lack of complete remission assessed by mSWAT. Conclusion We present for the first time the possibility of monitoring patients' clinical state on the base of non-invasive USG imaging. We recommend additional use of 20 MHz USG to reduce intra-observer variability and to assess residual disease. USG imaging can complement evaluation of skin lesions in MF and can support clinical judgement.