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Resistant pityriasis lichenoides et varioliformis acuta in a 3-year-old boy
Abstract
Pityriasis lichenoides et varioliformis acuta (PLEVA) represents the acute clinical subtype of pityriasis lichenoides (PL) and its occurrence is relatively common during childhood. Diagnosis and treatment may sometimes pose certain difficulties. We present the recalcitrant case of a 3-year-old boy with an asymptomatic polymorphic eruption consisting of multiple, scattered, 0.5 cm, round to ovoid, erythematous papules covered in places with a fine scale, vesicles and superficial erosions with thick hemorrhagic crusts. The correlation of the clinical features with the lesional histopathology favored the diagnosis of PLEVA. No first-line treatment scheme succeeded in controlling the eruption of new lesions. The only therapeutic approach that eventually managed to cease the disease evolution was the combination of prednisolone and methotrexate.
... A review article from 2019 on PLEVA in adults by Bellinato et al., suggested the use of methotrexate or erythromycin with or without topical corticosteroids as the first therapeutic option, but in our case, therapy with doxycycline and systemic corticosteroids resulted in a positive therapeutic response without the need for the use of methotrexate [13]. In refractory cases, in addition to methotrexate, successful therapy with acitretin, dapsone and cyclosporine is described in the literature [15,16]. ...
Introduction. Pityriasis lichenoides et varioliformis acuta is a rare inflammatory skin disease of unknown etiology and its diagnosis is sometimes established by eliminating diseases that are considered in the differential diagnosis. Given the lack of randomized clinical trials, recommendations for therapy remain based on case reports and case series. Case Report. We present a 63-year-old female patient with generalized skin lesions including, papules, papulonecrotic lesions, and atrophic scars accompanied by a subjective feeling of itching that occurred 2 months before admission. The histopathological findings showed a mixed perivascular inflammatory cellular infiltrate and capillary blood vessels with thickened walls in the superficial part of the dermis as signs of vasculitis. The infiltrate was dominated by lymphocytes, neutrophils were admixed, but there were no signs of cellular atypia, which supported the clinical diagnosis of pityriasis lichenoides et varioliformis acuta. Therapy with systemic corticosteroids and doxycycline was applied, which led to the resolution of lesions. Conclusion. The authors would like to bring to the readers? attention a rare skin disease, pityriasis lichenoides et varioliformis acuta, point to papulonecrotic tuberculids in differential diagnosis due to similar clinical presentation, remind them of the dilemmas that may arise in case of the described lymphocytic vasculitis based on the findings of histopathological analysis, and highlight the effectiveness of doxycycline and prednisone in the therapy.
... Combination of MTX with systemic steroids or cyclosporine and antibiotics has induced remission even in life-threatening cases. [39][40][41][42] ...
Vitiligo comprises of one of the commonest reasons for a dermatology consultation in the pediatric age group, worldwide. The incidence of childhood vitiligo varies from 1% to 8%, making it a significant pediatric condition. Various theories have been put forward to explain the occurrence of depigmentation in vitiligo, of which genetic factors play a predominant role in childhood vitiligo. The various modalities of treatment of childhood vitiligo are reviewed in this article. The management of vitiligo is extremely challenging, more so in the pediatric age group, as it can lead to significant psychologic trauma and dysregulation of social development in a child. The goals of management of childhood vitiligo should be aimed at addressing all these issues and achieving an optimum result out of the available modalities.
... 80 percent of pediatric PL cases were treated with erythromycin and two-thirds of these showed at least a partial response in study of Ersoy-Evans et al., Gunatheesan year old woman in whom lesions were rapidly resolved with azithromycin [18] . Lazaridou et al. reported a case of PLEVA resistant to clarithromycin [19] . We administered clarithromycin syrup to our case and we received a satisfactory result. ...
Pityriasis Iichenoides is a rare cutaneous disorder of clearly unknown etiology and speculated to be an infl ammatory response triggered by many infectious agents. We report a case of pityriasis lichenoides et varioliformis acuta (PLEVA) in a 7 year old boy. Lesions occurred abruptly aft er administration of combined diphtheria, tetanus, acellular pertussis and inactivated polio vaccine (DTaP/IPV). We want to emphasize that our case is the fi rst case report associated with this vaccine in literature and it is successfully treated with clarithromycin.
... Cornelison et al señalaron la eficacia del metotrexato en una serie de seis pacientes con PLEVA [44] . Posteriormente, se han publicado otros casos de remisiones completas con metotrexato administrado en dosis comprendidas entre 7,5-20 mg/kg/semana, aunque con frecuentes recaídas al finalizar el tratamiento [45][46][47] . ...
Resumen
La pitiriasis liquenoide, antes conocida como parapsoriasis en «gotas», constituye un conjunto de dermatosis inflamatorias que reúne, por un lado, una forma crónica o pitiriasis liquenoide crónica y, por otro, una forma aguda o pitiriasis liquenoide y varioliforme aguda, con varias formas intermedias. Desde el punto de vista clínico, la pitiriasis liquenoide crónica se manifiesta por una erupción de elementos papuloescamosos, que evoluciona por episodios y remisiones durante varios meses. En la forma aguda, se observan lesiones necróticas que en su evolución dejan una cicatriz varioliforme. La fisiopatología es desconocida. El diagnóstico se basa en el cuadro clínico así como en la histología y en el estudio inmunohistoquímico. El principal diagnóstico diferencial es la papulosis linfomatoide, por sus similitudes clínicas y, a veces, histológicas. Son varias las modalidades terapéuticas disponibles, aunque serían necesarios grandes estudios aleatorizados. El tratamiento de primera línea recurre a los antibióticos, esencialmente a las ciclinas, asociados a dermocorticoides o tacrolimús tópico. Como segunda opción, se propone la PUVAterapia o la fototerapia con ultravioletas B. La corticoterapia oral y el metotrexato se reservan para las formas graves y recidivantes.
... Combination of MTX with systemic steroids or cyclosporine and antibiotics has induced remission even in life-threatening cases. [39][40][41][42] ...
... 73,74 MTX has also been used effectively for other conditions such as pityriasis lichenoides et varioliformis acuta and dermatomyositis. 50,71,73,75 ...
... In our case, we treated the patient with the combination of erythromycin and dapsone. [10,11] We did keep an eye on hemoglobin level during the course of treatment. The combination of these two safe drugs in a pediatric age group had not been tried till now. ...
Immunosuppressants are required for an array of pediatric dermatoses. Certain dermatological conditions require long-term use of immunomodulators such as methotrexate and mycophenolate mofetil. All immunosuppressants have varying toxicity profiles and long-term use of these in the pediatric age group may lead to significant adverse effects. This concise review focuses on the immunosuppressants commonly used for dermatoses in children and gives a comprehensive list of the various indications for use in each and associated adverse effects on long-term use. An English language search was done on studies published till date on immunosuppressant use in pediatric dermatoses. A documented review was prepared, analyzed, and presented in a narrative fashion to highlight the important pediatric indications for the use of the respective immunosuppressant, with special emphasis on the adverse effects on prolonged usage.
Pityriasis lichenoides et varioliformis acuta (PLEVA), also known as Mucha-Habermann disease, is an uncommon cutaneous inflammatory disease that mostly affects children and young adults. It usually presents as an acute eruption of inflammatory macules, papules or papulovesicles, which quickly develop haemorrhagic or necrotic crusts. The most severe presentation of the disease is febrile ulceronecrotic Mucha-Habermann disease (FUMHD), which can be potentially life-threatening. This is the reason why we should recognise and start the right treatment of the disease in time.
Pityriasis lichenoides et varioliformis acuta (PLEVA), tudi Mucha-Habermannova bolezen, je redka kožna vnetna bolezen, ki prizadene predvsem otroke in mlade odrasle. Navadno se kaže kot akutni izbruh eritematoskvamoznih makul, papul ali papulovezikul, ki hitro razvijejo hemoragične ali celo nekrotične kruste. Najhujša oblika bolezni, febrilna ulceronekrotična Mucha-Habermannova bolezen (FUMHD), je potencialno življenje ogrožajoče stanje, zato je prav, da bolezen prepoznamo ter pravočasno in ustrezno pristopimo k zdravljenju.
Pityriasis lichenoides (PL) is a skin condition of unclear etiology that occurs not uncommonly in childhood. It is often classified into the acute form, pityriasis lichenoides et varioliformis acuta (PLEVA), and the chronic form, pityriasis lichenoides chronica (PLC). We performed a comprehensive review of the English-language literature using the PubMed database of all cases of childhood PL reported from 1962 to 2014 and summarized the epidemiology, clinical features, treatment options, and prognosis of this condition in children. The proposed etiologies are discussed, including its association with infectious agents, medications, and immunizations and evidence for PL as a lymphoproliferative disorder. We found an average age of PL onset of 6.5 years, with a slight (61%) male predominance. We also found that PLEVA and PLC tend to occur with equal frequency and that, in many cases, there is clinical and histopathologic overlap between the two phenotypes. When systemic therapy is indicated, we propose that oral erythromycin and narrowband ultraviolet B phototherapy should be first-line treatment options for children with PL since they have been shown to be effective and well tolerated. In most cases, PL follows a benign course with no greater risk of cutaneous T-cell lymphoma, although given the rare case reports of transformation, long-term follow-up of these patients is recommended.
© 2015 Wiley Periodicals, Inc.
Methotrexate is a cytotoxic antimetabolite agent and a folic acid antagonist. Except for its use in oncology and rheumatology, it is widely used in dermatology. The most important indications include severe forms of psoriasis, but also a wide range of autoimmune diseases and dermatoses with different etiology and pathophysiology such as: bullous pemphigoid, dermatomyositis, pityriasis rubra pilaris, sarcoidosis, T-cell lymphomas, Behcet’s disease, adult atopic eczema, scleroderma, Reiter’s syndrome and many others. For dermatological indications methotrexate is usually taken in low oral doses, 5-25 mg once a week. In certain diseases it can be applied to the lesion itself, or in the form of local preparations. Considering the fact that numerous drugs affect various metabolic phases of methotrexate and may increase its toxicity, it is of utmost importance to consider other prescribed drugs, especially certain antibiotics, nonsteroidal antiinflammatory drugs, antiepileptic drugs, retinoids, proton pump inhibitors and so on. If the selection of patients is correct, if drugs are taken regularly and laboratory monitoring is included, methotrexate is a truly efficient and safe drug that can be taken for months or years if necessary.
Eosinophils are often present in the inflammatory infiltrate of an interface dermatitis, but the diagnostic specificity of eosinophils in interface dermatitis has not been formally evaluated. We retrospectively identified 97 examples of interface dermatitis with clinically confirmed diagnoses, including lupus erythematosus (LE), lichen planus, pityriasis lichenoides (PL), graft-vs.-host disease (GVHD), dermatomyositis (DM) and drug reaction. Diagnoses were clinically confirmed by at least two dermatologists. Slides were reviewed in a blinded fashion by at least two dermatopathologists. The average eosinophil count per 10 ×200 (×20 objective) fields was lowest for PL (0.2), DM (0.3), GVHD (0.4), and LE (0.5) [defined as Group 1] and was higher for lichen planus, drug reactions, erythema multiforme (major and minor) and viral exanthems [defined as Group 2]. Distinction between Group 1 and Group 2 was maximized using an eosinophil count cutoff of 1.1. In conclusion, eosinophils are usually rare to absent in PL, DM, most forms of LE and GVHD. While final interpretation requires a composite assessment of all features, our results suggest that the presence of even a single eosinophil within nine or ten ×20 fields argues against a diagnosis of PL, DM or LE.
Sharon VR, Konia TH, Barr KL, Fung MA. Assessment of the ‘no eosinophils' rule: are eosinophils truly absent in pityriasis lichenoides, connective tissue disease, and graft-vs.-host disease?
Juvenile idiopathic arthritis (JIA) is the most common diagnosis in children and adolescents with rheumatic disorders. In many children and adolescents, JIA is successfully treated with non-steroidal anti-inflammatory drugs (NSAID) and physiotherapy. However, in a significant number of cases the disease is resistant to this therapy, and treatment with "second line" disease-modifying antirheumatic drugs (DMARDs) is required. Methotrexate (MTX) is frequently referred to as "first-choice second-line agent" for the treatment of JIA. To increase drug safety, the Working Groups for Children and Adolescents with Rheumatic Diseases in Germany (AGKJR) and Pediatric Rheumatology Austria have initiated the formulation of evidence-based recommendations. Evidence is based on consensus expert meetings, a MEDLINE search with the key words "Methotrexate" and "juvenile arthritis" limited to age 0-18 years, standard textbooks and review articles, data from the central registry of the German Research Center for Rheumatic Diseases (Deutsches Rheumaforschungszentrum Berlin DRFZ), experience with MTX in adults with rheumatoid arthritis (RA), and recommendations of the German Society of Rheumatology (DGRh). Based on these data, evidence and recommendations are graded, and evidence-based recommendations for the use of MTX in children and adolescents with rheumatic disease are presented.
: Pityriasis lichenoides (PL) is a cutaneous disease of unknown origin, with an autoinvolutive course, that can occur in pediatric patients. Traditionally, acute and chronic variants have been described, but other special forms of presentation have been reported. We reviewed the clinical records and histopathologic specimens of all pediatric patients diagnosed with PL in our hospital from 1980 to 1995 to assess the clinicopathologic features of this disorder in our environment. Twenty-two of the 118 cases reviewed were pediatric patients less than 15 years old (12 males and 10 females, 18.6% of all patients). Their ages ranged from 3 to 15 years, with a mean of 9.3 years. Most of the patients (72%) had the chronic variant of the disease, while the remainder had an acute course. One patient suffered from acute ulceronecrotic PL. Systemic treatments prescribed were erythromycin in eight patients, PUVA in five patients, and methotrexate in one patient. Three patients had a prolonged course with more than two episodes. Acute and chronic PL are polar extremes, but individual cases cannot be classified only on the basis of histopathologic data, since coexistence of lesions in different stages of evolution can lead to sampling bias. Acute ulceronecrotic forms and the presence of a variable degree of cellular atypia in the infiltrate are liable to cause differential diagnostic problems with lymphomatoid papulosis (LP), which cannot be completely resolved on the basis of T-cell receptor clonal rearrangement detection.
Pityriasis lichenoides is usually classified into an acute and a chronic form. From a review of 89 cases of the disease seen since 1974 it seems that a more realistic classification into three main groups, according to the distribution of pityriasis lichenoides lesions, could be made, namely, a diffuse, a central, and a peripheral form, each characterized by a different clinical course. Conversely, no correlations were detected in our series between the severity of skin lesions and their distribution or the overall course of the disease. None of our cases suggests the possible evolution of pityriasis lichenoides into lymphomatoid papulosis. Although no infectious causative agent has been identified, a viral origin seems likely in some cases. Most patients responded favorably to UVB irradiation. Our conclusions are (1) that pityriasis lichenoides is probably a clinical disorder with a diverse etiology and (2) that its classification by distribution seems more useful than its subdivision into an acute and a chronic form.
To investigate the efficacy of folinic acid in reducing the side effects associated with methotrexate (MTX) therapy in children with juvenile idiopathic arthritis (JIA) and to determine whether folate supplementation may reduce the benefit of MTX administration.
This was a retrospective, non-controlled study. Inclusion criteria were: 1) diagnosis of JIA according to the Durban 1997 criteria; 2) treatment with low to intermediate doses of MTX (10-20 mg/m2/week) as the sole second-line agent for at least 6 mos.; and 3) supplementation with folinic acid (2.5-7.5 mg) in a single weekly dose 24 hrs after MTX administration. All patients were started on folinic acid only after the development of a side effect. Exclusion criteria were: treatment with higher doses of MTX (> 20 mg/m2/week). The outcomes investigated were: hepatotoxicity (liver transaminase increase), gastrointestinal toxicity, disease flare, and clinical remission. The number of episodes per patient-year of MTX treatment of each outcome before and after folinic acid supplementation was compared by the Wilcoxon matched pairs test.
A total of 43 children with JIA were included in the study. The mean duration of treatment before and after folinic acid supplementation was 1.1 years and 1.8 years, respectively. After the start of folinic acid supplementation, the mean number of episodes per patient-year of hepatotoxicity and gastrointestinal toxicity decreased from 2.30 to 0.32 (p < 0.001) and from 1.09 to 0.29 (p = 0.002), respectively. The mean number of disease flares and clinical remissions per patient-year did not change significantly.
In our JIA patients, folinic acid supplementation resulted in a significant reduction in the most common side effects of MTX, without affecting the clinical efficacy of the drug.
Methotrexate (MTX) has transformed the outlook for children with juvenile idiopathic arthritis (JIA). Most of the evidence from uncontrolled clinical trials suggests that MTX is an effective agent for treating active JIA. Data from controlled clinical trials suggests that MTX has statistically significant effects on patient centred disability measures in JIA patients with active arthritis. Although we would like a much larger study directed evidence base for our use of the drug, the studies that have been done are sound and have been followed by a change in clinical expectations and advice that speak of qualitative evidence from clinical practice, confirming the scientifically acquired data. Randomised controlled multicentre trials using sufficient numbers of patients, including functional assessment and quality of life measures, are needed to confirm the long term efficacy and safety of MTX in JIA.
Febrile ulceronecrotic Mucha-Habermann's disease is an unusual severe form of pityriasis lichenoides et varioliformis acuta characterized by abrupt onset of ulceronecrotic eruption associated with a high fever and systemic symptoms. To our knowledge, 19 cases of this disease have been reported in the literature, and 4 of them were fatal. We report the case of a 12-year-old boy with this disorder who had abdominal pain, hypoproteinemia, and anemia. Although these associated symptoms are considered life-threatening factors according to reported cases, our patient was successfully treated with methylprednisolone semipulse and subsequent methotrexate therapies. From a review of the literature and the present case, we propose that when patients have these systemic symptoms, therapeutic choices include methotrexate, high-dose corticosteroids, and 4,4-diamino-diphenyl-sulfone, which may depress early development of this disease.
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) represents a fulminant and potentially lethal variant of pityriasis lichenoides. Only 24 cases have been described so far. We report a 9-year-old boy who initially presented with classical pityriasis lichenoides et varioliformis acuta (PLEVA) following a mild enteritis. Three weeks later, his skin lesions started to ulcerate progressively, involving > 90% of his body surface, accompanied by high fever, normal C-reactive protein, but highly elevated serum levels of tumour necrosis factor (TNF)-alpha. Methotrexate 10 mg m(-2) weekly was required to halt disease progression, while oral steroids (initial dose 2.8 mg kg(-1) daily) alone proved insufficient. Sequential histology revealed progressively dense perivascular and intramural lymphocytic inflammation as well as keratinocyte necrosis. Our case demonstrates the clinical and histological continuum between 'classical' PLEVA and FUMHD and points to the potentially pathogenic significance of TNF-alpha. We hypothesize that in future cases, treatment with TNF-alpha antagonists might represent a reasonable alternative to high-dose immunosuppressive therapy.
Pityriasis lichenoides represents a unique group of inflammatory skin disorders that include pityriasis lichenoides et varioliformis acuta (PLEVA), febrile ulceronecrotic Mucha-Habermann disease (a subtype of PLEVA), and pityriasis lichenoides chronica. The history, epidemiology, clinical features, pathophysiology, and treatment of this group of conditions are reviewed in this manuscript. Learning objective: At the completion of this learning activity, participants should be familiar with the clinical manifestations, histopathological findings, proposed mechanisms for pathogenesis, methods of treatment, and potential outcomes of pityriasis lichenoides and its subtypes.
Pityriasis lichenoides (PL) occurs in all age groups, although predominantly in younger individuals.
We sought to study the clinical features of PL in children followed up at our institution.
The records of 124 children who were given the diagnosis of PL at our institution between 1993 and 2003 were retrospectively reviewed.
PL chronica (PLC) was recorded in 37% of the cases, PL et varioliformis acuta (PLEVA) in 57.3%, and clinical features of both disorders were seen simultaneously in the remaining. The median age of onset was 60 months (range: 6-180 months), although the median age of onset of PLEVA (median: 60 months) was significantly younger than that of PLC (median: 72 months) (P = .03). The age distribution showed peaks at 2 to 3 years (24.8%) and 5 to 7 years (32%). A history of infection or drug intake preceded the skin manifestations in 30% and 11.2% of patients with PLC and PLEVA, respectively. The disease began most commonly during winter (35%) or fall (30%). The median duration was 20 months (range: 3-132 months) in patients with PLC and 18 months (range: 4-108 months) in patients with PLEVA. Involvement was diffuse in 74.2% of the patients, peripheral in 20.2%, and central in the remainder. The disease was recurrent in 77% of the patients (n = 80). Of the patients, 59% had pruritus, whereas 32% reported no symptoms; the remainder had fever, arthralgia, or both. Erythromycin estolate or ethylsuccinate was administered to 79.7% of the affected children; 66.6% of these showed at least a partial response.
The analyzed data were collected retrospectively and biopsies were not performed in all patients.
PL is not an uncommon disease in childhood, with age peaks in the preschool and early school-age years. It is usually recurrent, and shows a seasonal variation with onset most often in the fall or winter. In childhood PL, erythromycin is an effective initial treatment choice.
The purpose of this review is to educate the reader about two cutaneous lymphoproliferative diseases in childhood: pityriasis lichenoides and cutaneous T-cell lymphoma. Pityriasis lichenoides has traditionally been divided into acute and chronic subtypes. The two forms of the disease, however, are best thought of as two ends of a benign lymphoproliferative spectrum. Cutaneous T-cell lymphoma is a rare but underrecognized cutaneous malignancy in children. Early stage disease and hypopigmented presentation are characteristic of pediatric cutaneous T-cell lymphoma. The optimal investigation and treatment plans are still controversial.
This article will summarize recent articles on pityriasis lichenoides and pediatric cutaneous T-cell lymphoma, including recent findings from an international registry of pediatric cutaneous T-cell lymphoma.
After reading this review, the reader should be able to recognize the clinical presentation of pityriasis lichenoides, to understand the overlap between its acute and chronic forms, and to recognize its relationship with cutaneous T-cell lymphoma. In addition, the reader will appreciate the challenges in diagnosing and treating pediatric cutaneous T-cell lymphoma.
Pityriasis lichenoides and its
- Bowers S Warshaw
- Em
Bowers S, Warshaw EM. Pityriasis lichenoides and its