Standardization of regimens in Narrowband UVB and PUVA in early stage mycosis fungoides: position paper from the Italian Task Force for Cutaneous Lymphomas.

UV-based (PUVA and narrowband UVB) phototherapy is broadly and commonly used in the treatment of Cutaneous T-cell Lymphomas (CTCL), yet unfortunately, the evidence for the efficacy of these treatments is based only on case series or prospective but non-randomized studies. Therefore, no internationally approved guidelines exist and no standardization of schedules has been proposed. Recently, consensus guidelines have been published by the United States Cutaneous Lymphoma Consortium. The aim of this study was to review the biological and clinical evidences on PUVA and NB-UVB in CTCL and to critically evaluate acceptability and feasibility of these guidelines in the real-life setting from the perspective of the Cutaneous Lymphoma Task Force of the Italian Lymphoma Foundation (Fondazione Italiana Linfomi, FIL).

Comparison of Phototherapy Guidelines for Psoriasis: A Critical Appraisal and Comprehensive Review.

Psoriasis is a common, chronic immune-mediated inflammatory skin disorder that significantly impacts quality of life and has potential systemic complications. The majority of psoriatic patients have mild to moderate disease and are adequately controlled with topical medications. However, approximately 20% of patients have moderate-to-severe disease. Phototherapy has remained a mainstay option for patients with moderate-to-severe psoriasis resistant to topical treatments due to its efficacy, cost-effectiveness, and relative lack of side effects, in particular a lack of systemic immunosuppression seen with traditional and biologic systemic therapies. There are several well-established guidelines for phototherapy treatment of psoriasis proposed in the United States by the American Academy of Dermatology (AAD), in Europe by the European S3, and in the United Kingdom by the National Institute for Health and Care Excellence (NICE). The guidelines set by these groups are largely based on current evidence or expert panel consensus where evidence is lacking. This article reviews and compares the current recommendations of these guidelines for psoriasis phototherapy in regards to the initial clinical encounter, dosage, adverse reactions, and special considerations.

J Drugs Dermatol. 2016;15(8):995-1000.

In vitro assay of the effects of psoralens plus ultraviolet radiation on human lymphoid cells.

An in vitro assay system that mimics some of the features of in vivo oral methoxsalen photochemotherapy (PUVA) in relation to human lymphoid cells was developed. In this assay, suspension cultures of human lymphoid cells were subjected to therapeutic concentrations of 8-MOP and to a UVA radiation spectrum modified to approximate the dermal exposure. The assay permitted correlation and quantitation of multiple 8-MOP plus UVA-induced biological and physical alterations in the same assay system. Thus were demonstrated inhibition of DNA synthesis, reduction of cell survival, production of DNA cross-links, and loss of mixed leukocyte reactivity induced by combinations of 8-MOP and UVA in or near the presumed therapeutic range. This assay may be useful for predicting lymphoid cellular toxicity of other photoactive agents as well as for examining the molecular effects of these agents.

Psoriasis: therapeutic aspects.

Some of the more common features of psoriasis are reviewed here as an introduction to therapeutic modalities that can be employed in the management of this disorder in the New Zealand situation. An approximate indication of costs for the various therapies is included. This article is sponsored by the therapeutics section of the Department of Health, however, the views of the contributors do not necessarily reflect departmental policies.

Topical PUVA therapy for vitiligo.

Vitiligo, a pigmentary disorder of the skin, causes the appearance of white patches which are profoundly devastating to the person afflicted with this disease. This article outlines the safe use of topical PUVA therapy in the office for treating those patients with less than 20% skin surface involvement. This treatment has a response rate of approximately 50%.

Long-term efficacy of PUVA treatment in lichen planus: comparison of oral and external methoxsalen regimens.

Forty-three patients with lichen planus (LP) were treated with either oral methoxsalen PUVA (10 cases), bath methoxsalen PUVA (13 cases) or no PUVA (20 cases). In the bath treatment series, a good or excellent clearing of the lesions was registered in 10 out of 13 cases after courses of 8-46 irradiations, while in the orally medicated group only 5 out of 10 had a similar response after 8-30 irradiations. In addition, 11 of the 23 PUVA-treated patients showed progressive improvement of their condition during the first few months after PUVA therapy. A clinical follow-up study was made 15 and 10 months later in the oral and bath PUVA groups, respectively, and at 10 months in the no-PUVA group. Six of the 23 PUVA-treated patients (23%) were found to be totally clear, but the remaining 17 patients (74%) had clinical signs of LP. Eleven of the 20 no-PUVA-treated patients (55%) showed total clearing. It is concluded that while both oral and bath methoxsalen PUVA regimens have a distinct immediate (1-2 months) clearing effect on LP, the late outcome of the rash is not better than with no UV treatment; PUVA treatment may possibly even prolong the ultimate course of LP.

Guidelines for topical PUVA: a report of a workshop of the British photodermatology group.

Psoralen photochemotherapy [psoralen ultraviolet A (PUVA)] plays an important part in dermatological therapeutics, being an effective and generally safe treatment for psoriasis and other dermatoses. In order to maintain optimal efficacy and safety, guidelines concerning best practice should be available to operators and supervisors. The British Photodermatology Group (BPG) have previously published recommendations on PUVA, including UVA dosimetry and calibration, patient pretreatment assessment, indications and contraindications, and the management of adverse reactions.1 While most current knowledge relates to oral PUVA, the use of topical PUVA regimens is also popular and presents a number of questions peculiar to this modality, including the choice of psoralen, formulation, method of application, optimal timing of treatment, UVA regimens and relative benefits or risks as compared with oral PUVA. Bath PUVA, i.e. generalized immersion, is the most frequently used modality of topical treatment, practised by about 100 centres in the U.K., while other topical preparations tend to be used for localized diseases such as those affecting the hands and feet. This paper is the product of a recent workshop of the BPG and includes guidelines for bath, local immersion and other topical PUVA. These recommendations are based, where possible, on the results of controlled studies, or otherwise on the consensus view on current practice.

Phototherapy for atopic dermatitis.

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.

Psoriasis: assessment and management

This guideline covers assessing and managing psoriasis in adults, young people and children. It aims to improve long-term disease control and quality of life for people with psoriasis. NICE has produced a COVID-19 rapid guideline on dermatological conditions treated with drugs affecting the immune response. It recommends changes to usual practice to maximise the safety of patients and protect staff from infection during the COVID-19 pandemic. In September 2017, we revised the guideline throughout to link to other NICE guidance (including technology appraisals) and some relevant non-NICE guidelines, as well as including new MHRA safety advice and updated licensing information.

Lesiones ampollosas como forma de presentación de una enfermedad de injerto contra huésped crónica / Chronic graft-versus-host disease presenting as bullous lesions

La enfermedad de injerto contra huésped (EICH) continúa siendo la primera causa de morbilidad y mortalidad en los enfermos con trasplante de médula ósea, de modo que un tratamiento precoz es importante para reducir la gravedad y las consecuencias de esta complicación. Las lesiones cutáneas son en muchas ocasiones la forma de presentación de una EICH y anuncian la afectación visceral. Presentamos el caso de una paciente de 45 años con mieloma múltiple a la que se realizó primero un trasplante autólogo y posteriormente un trasplante alogénico de médula ósea con precursores hematopoyéticos. Desarrolló lesiones ampollosas con eliminación de líquido en el abdomen y los miembros inferiores. Se realizó una biopsia que fue compatible con una EICH y se aumentó el tratamiento inmunodepresor. La paciente posteriormente presentó lesiones liquenoides orales, síndrome seco y las lesiones ampollosas que evolucionaron a úlceras dolorosas, que cicatrizaron con un intenso aspecto esclerodermiforme e importante hiperpigmentación. Las lesiones ampollosas son una forma de presentación excepcional en la EICH crónica, y en estos casos la sospecha inicial del diagnóstico puede ser difícil, sobre todo cuando se presentan de forma aislada sin afectación de órganos internos (AU)

Graft-vs-host disease is still the leading cause of morbidity and mortality in patients undergoing bone marrow transplantation. It is important to start treatment early to reduce the severity and consequences of this complication. Cutaneous lesions are often the presenting compliant of graft-vs-host disease and presage visceral involvement. We present the case of a 45-year-old woman with multiple myeloma who underwent autologous and subsequently allogeneic bone marrow transplantation with hematopoietic precursors. She developed bullous lesions with fluid elimination on the abdomen and legs. Biopsy findings were compatible with graft-vs-host disease and immunosuppressive therapy was increased. She subsequently presented oral lichenoid lesions and sicca syndrome. The bullous lesions progressed to painful ulcers that healed leaving highly sclerodermatous skin with substantial hyperpigmentation. Bullous lesions are a rare form of presentation of chronic graft-vs-host disease. In such cases, the diagnosis may not be suspected initially, particularly when the lesions are isolated and internal organs are not involved (AU)

Histiocitoma eruptivo generalizado / Generalized eruptive histiocytoma

Presentamos el caso de un varón de 64 años que desarrolló, a lo largo de un periodo de 4 meses, múltiples pápulas asintomáticas distribuidas simétricamente por el tronco y las regiones proximales de las extremidades, respetando cuero cabelludo, palmas, plantas y mucosas. Las lesiones eran discretas, eritematoparduscas, redondeadas, desde ligeramente elevadas a hemisféricas, de 1 a 3 mm de diámetro, y firmes al tacto. Los estudios clínicos, histopatológicos, inmunohistoquímicos y ultraestructurales fueron de histiocitoma eruptivo generalizado. Las pruebas de laboratorio y los estudios radiológicos no mostraron hallazgos anormales. Tras someterse a fotoquimioterapia, las lesiones remitieron, pero más adelante aparecieron nuevos brotes de pápulas. El histiocitoma eruptivo generalizado pertenece a un espectro de enfermedades que pueden solaparse e incluso progresar las unas a las otras. Puesto que la enfermedad podría progresar hacia una dolencia más grave, habría que considerar seriamente revisiones regulares con exámenes clínicos, histológicos e inmunohistoquímicos

We present the case of a 64-year-old male who developed multiple asymptomatic papules over a four-month period, distributed symmetrically on the trunk and proximal regions of the limbs, while the scalp, palms, soles and mucous membranes were spared. The lesions were discrete papules, brownish-erythematous, rounded, ranging from slightly raised to hemispherical, from 1 to 3 mm in diameter and firm to the touch. Clinical, histopathological, immunohistochemical and ultrastructural studies were consistent with generalized eruptive histiocytoma. Laboratory tests and radiological studies did not show any abnormal findings. After being subjected to photochemotherapy, the lesions regressed, but later on new outbreaks of papules appeared. Generalized eruptive histiocytoma belongs to a spectrum of diseases that may overlap; some may even develop into others. As the disease may progress into a more serious ailment, regular checkups with clinical, histological and immunohistochemical examinations should definitely be considered