Poikilodermatous Mycosis Fungoides: Comparative Study of Clinical, Histopathological and Immunohistochemical Features.

BACKGROUND: Poikilodermatous mycosis fungoides (pMF) is characterized by poikiloderma areas, typically involving the major flexural areas and trunk. Its presentation can be generalized or admixed with other forms of MF. Previous studies fail to correlate the clinical presentation with prognosis and laboratory findings. Some reports show pityriasis lichenoides chronica (PLC) preceding the poikiloderma. OBJECTIVES: Correlate prognostic, histopathological and molecular aspects of pMF with its clinical presentation. METHODS: Retrospective analysis of 14 cases of generalized pMF (GpMF), 22 of localized pMF (LpMF) and 17 of pMF admixed with other forms of MF (mix-pMF). RESULTS: Female predominance and lower age at diagnosis was found in all groups compared to classic MF, a high prevalence of PLC-like lesions in the GpMF group and a high rate of hypopigmented lesions in the mix-pMF group. There were 2 deaths within the GpMF group. Histology was similar to previously reported findings, as was the prevalence of CD4 T-cell infiltrate, compared to CD8. The T-cell clonality positivity was lower in the GpMF group, compared to other groups (27% GpMF, 80% LpMF and 100% mix-pMF). DISCUSSION: This is the first article to categorize the different forms of pMF and correlate them with clinical and laboratory findings. The dermatological presentation differs among the groups. There was a high frequency of PLC-like lesions within the GpMF group and of hypopigmented lesions in mix-pMF. The histological and immunohistochemical findings were similar to those previously reported. Aggressive treatments are not recommended due to the good prognosis of all pMF forms. The low positivity of T-cell clonality in the GpMF group should be investigated.

The rash with maculopapules and fever in adults.

There is a broad differential diagnosis for the presentation of fever and maculopapular rash in an adult. Although some causative conditions are benign, others are medical emergencies that require prompt diagnosis. We describe various conditions that result in a fever and maculopapular rash in adults. These include infectious processes (meningococcemia, infectious mononucleosis, West Nile virus, zika virus, rubella, primary human immunodeficiency virus, parvovirus B19, ebolavirus), tick-borne illnesses (Rocky Mountain spotted fever, ehrlichiosis), and hypersensitivity reactions (exanthematous drug reactions). We also provide an algorithm to aid in the diagnosis of the patient with fever and maculopapular rash. Such conditions that can occur in adults but are seen predominantly in children are discussed in the article "Rash with maculopapules and fever in children" of this issue.

The rash with maculopapules and fever in children.

Several medical conditions can cause children to present with fever and a maculopapular rash Although some presentations are benign, others may be medical emergencies, which warrant a prompt diagnosis. We review some of the more common causes of fever and maculopapular dermatitirs, rash including infectious processes (roseola; rubeola; rubella; parvovirus B19; hand, foot, and mouth disease; scarlet fever; meningococcemia; Epstein-Barr virus infection), hypersensitivity reactions (exanthematous drug reactions), and vasculitis syndromes (Kawasaki disease). We have included a diagnostic algorithm to facilitate rapid identification of the etiology of the rash and fever. Those conditions that can occur in children but are seen predominantly in adults are discussed in the contribution "Rash with maculopapules and fever in adults" in this issue.

Tailor systemic therapy to the patient with severe psoriasis.

There is no standard definition regarding the severity of psoriasis, and a number of factors should be considered, including the extent and stability of skin disease, involvement of joints, response to treatment, and impact on quality of life. Erythrodermic psoriasis and pustular psoriasis are severe conditions and the patient may be systemically unwell and febrile. NICE recommends that four key areas should be evaluated and recorded when assessing patients: severity, using the static Physician's Global Assessment (sPGA); disease impact on physical, psychological and social wellbeing using the Dermatology Life Quality Index (DLQI); the presence of psoriatic arthritis; and comorbidities. Ideally, patients should be assessed annually for psoriatic arthritis: the Psoriasis Epidemiology Screening Tool is a validated tool to screen for psoriatic arthritis in primary and secondary care. Patients with severe psoriasis should undergo cardiovascular risk assessment at presentation and every five years, or more frequently if indicated. Referral to secondary care should be made for patients with any type of psoriasis with poor response to topical therapy (after 2 or 3 months according to SIGN) and for extensive psoriasis. Cases where the psoriasis is having a significant physical or psychological impact on an individual's quality of life warrant early referral, as do those where the diagnosis is uncertain. Patients with generalised pustular psoriasis or erythroderma should be referred urgently for same-day specialist input. Patients with acute guttate psoriasis who may require phototherapy should also be referred. Children and adolescents with any type of psoriasis should be referred to a specialist at initial presentation.

Long-term study of infliximab in Japanese patients with plaque psoriasis, psoriatic arthritis, pustular psoriasis and psoriatic erythroderma.

The efficacy and safety of infliximab in patients with plaque psoriasis, psoriatic arthritis, pustular psoriasis (excluding localized type) and psoriatic erythroderma were assessed in clinical practice. Without washout of the existing treatment of psoriasis, treatment was switched to infliximab, which was given at a dose of 5 mg/kg at weeks 0, 2 and 6 and then every 8 weeks up to week 46. The primary end-points were 75% improvement in Psoriasis Area and Severity Index score (PASI 75 response rate) for plaque psoriasis, 20% improvement in American College of Rheumatology criteria (ACR 20 response rate) for psoriatic arthritis, and global improvement in pustular psoriasis and psoriatic erythroderma. The PASI 75 response rate in plaque psoriasis was 72.2% at week 10 and 53.6% at week 50. The ACR 20 response rate in psoriatic arthritis was 66.7% at week 14 and 80.0% at week 46. The response defined as global improvement in pustular psoriasis was between 66.7% and 100.0% during the 2­50-week period. The response defined as global improvement in psoriatic erythroderma was between 75.0% and 100.0% during the week-2­50 period. There were 14 discontinued patients. The most frequently reported reason for discontinuation was the development of adverse events. However, there were no serious respiratory diseases, infections or infusion reactions. In patients with plaque psoriasis, psoriatic arthritis, pustular psoriasis and psoriatic erythroderma, infliximab was well tolerated, regardless of prior treatment, and also showed superior efficacy over a period of approximately 1 year.

The conundrum of parapsoriasis versus patch stage of mycosis fungoides.

Terminological confusion with benign dermatosis, such as parapsoriasis en plaques, makes it difficult to diagnose mycosis fungoides in the early patch stage. Early diagnosis of mycosis fungoides (MF) is important for deciding on type of therapy, prognosis and for further follow-up. However, until recently, there has been no consensus on criteria that would help in diagnosing the disease early. Some believe that large plaque parapsoriasis (LPP) should be classified with early patch stage of MF and should be treated aggressively. However, there is no firm clinical or laboratory criteria to predict which LPP will progress to MF and we can only discuss about statistical probability. Moreover, long-term outcome analysis of even patch stage of MF is similar to that of control population. We therefore believe that LPP should be considered as a separate entity at least to prevent the patient from being given a frightening diagnosis. We also feel that patients need not be treated with aggressive therapy for LPP and will need only a close follow-up. This article emphasizes the criteria for diagnosing early MF and has highlighted the importance of considering LPP as a distinct benign entity.

Varón joven con lesiones eritemato-escamosas de larga evolución / Young male with long standing erythemato-squamous lesions

La parapsoriasis agrupa un número de estados patológicos caracterizados por lesiones eritemato-escamosas recalcitrantes en piel que no llenan los criterios de malignidad, y reflejan situaciones intermedias. Presentamos el caso de un varón joven con lesiones eritemato-escamosas de largo tiempo de evolución sin alteraciones del estado general y sin compromiso visceral. Hacemos una breve revisión clínica de la enfermedad y sus hallazgos histopatológicos.

Parapsoriasis brings together a number of pathological conditions characterized by erythemato-squamous skin lesions that do not meet the criteria for malignancy, and reflect intermediate situations. We present the case of a young male with erythemato-squamous lesions of long time of evolution, without alterations of the general statement and without visceral compromise. We make a brief review of the clinical disease and its pathological findings.

Estudio epidemiológico de la PUVAterapia en la comunidad valenciana / Use of psoralen plus UV-A therapy in the autonomous Community of Valencia, Spain

Introducción. La fotoquimioterapia con 8-metoxipsoraleno y radiación ultravioleta de longitud de onda larga A (PUVA) es frecuentemente utilizada para el tratamiento de diferentes enfermedades cutáneas desde que en 1982, la Food and Drug Administration aprobase su uso. Métodos. En este estudio retrospectivo se han analizado los pacientes tratados con PUVA, incluyendo tratamiento tópico y sistémico durante 14 años. Todos estos pacientes recibieron un régimen de terapia PUVA estándar. Resultados. Durante el período de 1982 a 1996 se analizaron 877 pacientes. Un total de 41 dermatosis recibieron el tratamiento entre las que destacamos 341 casos de psoriasis y 71 casos de linfomas cutáneos de células T. El trabajo tiene como finalidad describir las características de los pacientes tratados con terapia PUVA durante estos años y comparar los resultados con los procedentes de otros ámbitos. Conclusiones. Aunque la terapia PUVA está ampliamente distribuida en un gran número de países para el tratamiento de diferentes enfermedades cutáneas, existen pocos estudios que indiquen las características de estos pacientes y las variaciones en los parámetros de PUVA dependiendo de las diferentes enfermedades

Background. Photochemotherapy with 8-methoxypsoralen and long-wavelength UV-A (PUVA) has been extensively used for the treatment of various skin diseases since its approval in 1982 by the US Food and Drug Administration. Methods. A retrospective study was performed of patients treated with PUVA, including topical and systemic treatment, over a period of 14 years. All patients were treated using a standard PUVA therapy regimen. Results. A total of 877 patients were analyzed for the period 1982 to 1996. Forty-one skin diseases were treated, including 341 cases of psoriasis and 71 cutaneous T-cell lymphomas. The aim of the study was to describe the characteristics of the patients treated with PUVA therapy during that period and compare the results with those observed in other regions. Conclusions. Although PUVA therapy is widely used in a large number of countries for the treatment of various skin diseases, few studies have described the characteristics of the patients and the differences in the parameters of PUVA according to the disease

Early-stage mycosis fungoides, parapsoriasis en plaque, and pregnancy.

BACKGROUND: Non-Hodgkin's lymphoma (NHL) coincident with pregnancy is rare, and the literature regarding mycosis fungoides (MF), the most common primary cutaneous NHL, and pregnancy is strikingly sparse. The effect of pregnancy on MF, or on parapsoriasis en plaque (PPP), and the effect of these diseases on pregnancy, are still unknown. OBJECTIVE: To study the effect of pregnancy on MF and PPP and the impact of these diseases on pregnancy. METHODS: The files of the MF and PPP patients seen during the past 12 years in our department were reviewed to search for patients who had been pregnant during the course of their disease. RESULTS: Nine women who met the study criteria were identified, seven with early-stage MF and two with PPP. A total of 12 pregnancies was recorded: nine in patients with MF and three in patients with PPP. In none of the patients was there any indication that pregnancy changed the course of MF or PPP. Of the 12 pregnancies, 11 were normal; one was naturally aborted. Two of the patients were treated with topical steroids during pregnancy. One patient was treated with narrow-band ultraviolet-B combined with topical steroids. The rest preferred to avoid any therapy. CONCLUSIONS: Pregnancy appeared to have no impact on the course of early MF or PPP, and no adverse effect was noted on pregnancy. Further studies are needed to clarify the interplay between pregnancy and MF or PPP.

[The excimer laser in dermatology and esthetic medicine]. / Der Excimer-Laser in der Dermatologie und ästhetischen Medizin.

First reports about the use of the excimer laser in dermatology date back to 1997. It is seen as an improvement on conventional phototherapy and photochemotherapy because of the lower cumulative UV-dose involved, the shorter time frame required for treatment and the option of targeting individual lesions without affecting the surrounding healthy skin. In addition to the indications of psoriasis vulgaris, vitiligo and atopic eczema (for which there is now FDA approval in the US), the spectrum of possible uses for the excimer laser is growing rapidly, especially in the field of light-sensitive dermatoses. Case studies so far have ranged from post-operative hypopigmentation to acne vulgaris and from alopecia areata to parapsoriasis en plaque. The foremost priorities in the future will be to evaluate reproducible therapeutic regimens with realistic prospects of success in large-scale studies; assess potential iatrogenic risks in treatment; develop pathogenetic models for the mechanism of action; and define therapeutic approaches to new indications. This paper summarizes the publications to date and discusses our observations and experiences.

[Large nodular lymphomatoid papulosis associated with parakeratosis variegata]. / Grossknotige lymphomatoide Papulose in Assoziation mit einer Parakeratosis variegata.

A 63 year old man suffering from lymphomatoid papulosis in association with parakeratosis variegata over a period of 20 years is presented. This case is unusual in respect to long-standing history, the rare combination of the two entities, and the extraordinary size of the nodules of lymphomatoid papulosis. Extracorporal photopheresis has induced partial remission lasting up to nine months so far.

[Small-plaque parapsoriasis: case report]. / Parapsorijaza malih plakova: prikaz slucaja.

INTRODUCTION: Small plaque parapsoriasis is a relatively rare, chronic, idiopathic dermatosis, most often seen in middle age people. This disease shows a definite male predominance of approximately 3-4: 1. It is characterized by presence of round or oval erythematous, slightly scaly plaques on the limbs and trunk, which histologically reveal mild eczematous changes. CASE REPORT: A male patient, 61 years of age, was admitted to the Clinic of Dermatovenereology in Novi Sad due to long persisting erythematous patches on his upper and lower limbs. Plaques were of oval and round shape, pretty well marginated. They were of light red colour, covered with fine scales with a slightly wrinkled surface. He complained of itching. Laboratory findings showed no abnormalities. Histopathologic examination of the skin specimen revealed epidermal atrophy, focal parakeratosis, perivascular dermal infiltrate of mononuclear cells with exocytosis in the epidermis. This finding was compatible with the clinical diagnosis. After treatment with topical corticosteroid cream combined with whole body exposure to sunlight irradiation, vast majority of skin lesions regressed. DISCUSSION: The clinical course of small plaque parapsoriasis is very long. The plaques are remarkably stubborn, responding to treatment with steroid creams or to natural or artificial sunlight, but usually reappearing promptly when treatment is discontinued. The patches increase in number for a time, and then remain relatively constant for a long time. A small minority of cases clears entirely. Recent studies provided evidence of monoclonality and immunophenotypic abnormalities. Rearrangement of T-cell receptor genes was demonstrated by using PCR method. Detection of monoclonal T-cell populations in skin lesions, as a characteristic of lymphoproliferative diseases, forced some authors to include this dermatosis into a group of abortive cutaneous T-cell lymphomas. CONCLUSION: This case deserves a long and probably life-long clinical and histological assessment, especially due to new knowledge about the possible nature of this disease.

Balneophototherapy in small plaque parapsoriasis--four case reports.

Four patients suffering from small plaque parapsoriasis were treated successfully with balneophototherapy. Within 4 weeks salt-water baths and UV irradiation resulted in clinical clearing of more than 90% of lesions with a duration of total clinical response between 8 and 12 weeks without further maintenance treatment.

Tratamiento de Linfoma T cutáneo ( Micosis fungoide ) con Interferon Alfa intralesional / Cutaneous T-cell Lymphoma (Mycosis fungoides): treatment with Interferon Alpha intralesional

Se presenta el caso de un paciente de 63 años de edad, portador de Linfoma T (Micosis Fungoide) de dos años de evolución, en todos sus estadíos (máculas, parches, placas y tumores); quien luego de haber recibido todas las opciones terapéuticas (algunas, por diversas razones, sólo parcialmente) sin resultado, inicia Interferon alfa intralesional, en dosis de 1.000.000 de U. por centímetro cuadrado de superficie cutánea que provocó remisión completa de las lesiones en seis meses, sin recaída en nueve meses de control. Presentamos una nueva modalidad terapéutica, en corto lapso de tiempo muy efectiva para una enfermedad de curso inexorable, en la que todos los resultados terapéuticos son muy poco efectivos.

Tratamiento de Linfoma T cutáneo ( Micosis fungoide ) con Interferon Alfa intralesional / Cutaneous T-cell Lymphoma (Mycosis fungoides): treatment with Interferon Alpha intralesional

Se presenta el caso de un paciente de 63 años de edad, portador de Linfoma T (Micosis Fungoide) de dos años de evolución, en todos sus estadíos (máculas, parches, placas y tumores); quien luego de haber recibido todas las opciones terapéuticas (algunas, por diversas razones, sólo parcialmente) sin resultado, inicia Interferon alfa intralesional, en dosis de 1.000.000 de U. por centímetro cuadrado de superficie cutánea que provocó remisión completa de las lesiones en seis meses, sin recaída en nueve meses de control. Presentamos una nueva modalidad terapéutica, en corto lapso de tiempo muy efectiva para una enfermedad de curso inexorable, en la que todos los resultados terapéuticos son muy poco efectivos.(AU)

Pityriasis lichenoides and lymphomatoid papulosis.

The clinical features, histopathology, immunopathology, and management of pityriasis lichenoides and lymphomatoid papulosis are discussed, with particular emphasis on the pediatric aspects of these conditions. The difficulties in logically separating pityriasis lichenoides into an acute (pityriasis lichenoides et varioliformis acuta) and a chronic (pityriasis lichenoides chronical) form are addressed. The development of lymphoreticular malignancy in patients with lymphomatoid papulosis has been well documented, but pityriasis lichenoides has characteristically been regarded as a benign condition. However, recent reports of the development of large plaque parapsoriasis in patients with pityriasis lichenoides have led to a reconsideration. Some of these patients were in the pediatric age group. Although there are significant clinical, histopathological, and immunopathological differences between pityriasis lichenoides and lymphomatoid papulosis, the demonstration of similar clonal T cell receptor gene rearrangements and the confirmation of the potentially premalignant nature of both suggests that there may indeed be an interrelationship between these two controversial entities. Close follow-up of patients with both of these conditions is recommended, with observation being discontinued only when the patient has been free of lesions for several years.