Pityriasis Lichenoides Et Varioliformis Acuta Research Articles

Immunophenotyping and viral studies in pityriasis lichenoides et varioliformis acuta lesions.

The underlying pathogenesis of pityriasis lichenoides et varioliformis acuta (PLEVA) remains unclear, although immunologic injury and viral etiology have been suggested. To evaluate and expand the immunophenotype of PLEVA and to search for possible viral pathogens. Formalin-fixed, paraffin-embedded specimens of 20 patients with PLEVA and 9 patients with common inflammatory dermatoses (ID) were studied for immunophenotyping and for human herpesvirus (HHV) 1 and 2, cytomegalovirus (CMV), HHV-8, parvovirus B19, and Epstein-Barr virus (EBV) immunohistochemistry. The presence of HHV-6, HHV-7, and enteroviruses was assayed molecularly. The numbers of CD8+ T cells and T-cell intracellular antigen-1 (TIA-1)+ cells were statistically significantly higher in PLEVA compared to the ID group. Immunohistochemistry for human HHV-1 and HHV-2, CMV and HHV-8, parvovirus B19, and in situ hybridization for EBV were all negative. There was molecular evidence for HHV-7 in only one PLEVA case (5%). Molecular studies for HHV-6 and enterovirus involvement were negative in all the PLEVA specimens. The predominant T-cell infiltrate in PLEVA is dominated by CD8+ cells, and by increased numbers of TIA1+ cells, which may indicate a cytotoxic T-cell damage to the epidermis. Viral presence was not detected.

Paediatric onset lymphomatoid papulosis: results of a multicentre retrospective cohort study, on behalf of the EORTC Cutaneous Lymphoma Tumours Group (CLTG).

Lymphomatoid Papulosis (LyP) is a rare cutaneous T-cell lymphoproliferative disorder. Comprehensive data on LyP in the paediatric population is scarce. To characterize epidemiological, clinical, histopathological, and prognostic features of paediatric LyP. This was a retrospective, multicentre international cohort study including 87 cases of children and adolescents with LyP diagnosed between 1998 and 2022. Patients aged ≤ 18 years old at disease onset were included. Diagnosis was made in each centre based on clinical-pathological correlation. Eighty-seven patients from 12 centres were included. The mean age at onset was 7.0 years (range 3 months-18 years) with a male to female ratio of 2:1. The mean time between onset of first cutaneous lesions and diagnosis was 1.3 years (range 0-14 years). Initial misdiagnosis concerned 26.4% of patients. Initially, LyP was most often misdiagnosed as Pityriasis lichenoides et varioliformis acuta (PLEVA), insect bites, or mollusca contagiosa. Erythematous papules or papulonodules were the most frequent clinical presentation. Pruritus was specifically mentioned for 20.7% of patients. The main histological subtype was type A in 55.1% of the cases. If analysed, monoclonal TCR rearrangement was found in 76.5% of the skin biopsies. The overall survival rate was 100% with follow up at 5 years available for 33 patients and at 15 years for 8 patients. A development of associated haematological malignancy (HM) occurred in 9.6% of the cases (7/73), including four mycosis fungoides (MF) cases, one primary cutaneous anaplastic large cell lymphoma (pc-ALCL), one systemic ALCL and one case of acute myeloid leukaemia. If we compare incidence rates of cancer with the world 0-19 years old population from 2001-2010, we estimate a significantly higher risk of associated malignancy in general, occurring before the age of 19 years old with incidence rate ratio of 87.49 (CI 86.01-88.99). We report epidemiological data from a large international cohort of children and adolescents with LyP. Overall the prognosis of the disease is good, with excellent survival rates for all patients. Due to increased risk of associated HM, a long-term follow-up should be recommended for LyP patients.

Pityriasis lichenoides: assessment of 41 pediatric patients

ObjectivesThis study aims to evaluate the characteristics and treatment response of patients with pityriasis lichenoides seen in the last 43 years in a pediatric dermatology service. MethodsThis was a retrospective, analytical, longitudinal study of patients under 15 years of age. The medical records were reviewed and data were presented as frequencies, means and variances. Student's t-test, Mann-Whitney test, Fisher's exact test, Pearson/Yates chi-square test and multivariate logistic regression model were used, with p < 0.05 considered. Results41 patients were included, 32 (78.0%) with pityriasis lichenoides chronica (PLC), five (12.2%) with pityriasis lichenoides et varioliformis acuta (PLEVA) and four (9.8%) with clinical PLC without biopsy. The age range of school children and adolescents was 19 (46.3%) and 13 (31.7%) respectively and 27 (65.8%) were male. Two peaks of the highest frequency were observed between 2004 and 2006 (10 patients - 24.4%) and another between 2019 and 2021 (6 patients - 14.7%). There was remission in 71.9% (n = 23), with 56.6% (n = 17) of those who used antibiotic therapy and 80% (n = 4) of those who had phototherapy. The chance of remission was 13 times greater in patients with disease onset after 5 years of age. ConclusionsThe clinical form most commonly found was PLC mainly in school children and adolescents. The frequency peaks coincided with infectious outbreaks. The remission rate was satisfactory with antibiotic therapy, but higher with phototherapy. Remission was greater in patients with disease onset after 5 years of age.

Pityriasis Lichenoides ET Varioliformis Acuta (PLEVA) As a Possible Differential Diagnosis for Monkeypox during the 2022 Outbreak in Non-Endemic Countries

In May 2022 several cases of monkeypox transmitted among humans in the UK, the USA and Europe were detected. The images of monkeypox skin lesions that were widely published in the media consisted exclusively of deeply seated vesicles and pustules on brown and black skin from endemic outbreaks in Africa. However, as cases accumulated in non-endemic countries, it has become clear that the clinical presentation of the current outbreak outside of Africa shows a very different picture, and that at least some of these cases might mimic pityriasis lichenoides et varioliformis acuta (PLEVA). It is a rare dermatologic condition of unknown aetiology, which has been described also as a reaction to vaccines. Currently, the World Health Organisation recommended laboratory tool for diagnosing monkeypox is the real time polymerase chain reaction test. Antigen and antibodies detection methods are not considered useful due to cross-reactivity between different orthopoxviruses. In this paper, I present the classic dermatologic criteria used for distinguishing between monkeypox and pityriasis lichenoides et varioliformis acuta as described in the literature. Further, as a hypothesis, I suggest considering pityriasis lichenoides et varioliformis acuta, a non- infectious skin reaction associated with extrinsic factors, including vaccines (also Covid-19 vaccines) as a possible differential diagnosis for monkeypox cases in the non-endemic countries.

A Clinicopathological Study on Interface Dermatitis

Background: Interface dermatitis (ID), also known as lichenoid tissue reaction, is a form of skin reaction characterized by an inflammatory infiltration that appears to cover the dermo-epidermal junction under inexpensively analysis. A wide range of inflammatory skin disorders demonstrate interface alteration with significant overlap of histological characteristics. Aim of the Study: The purpose of this study was to link interface dermatitis clinicopathologically. Methods: This cross-sectional descriptive type of study was conducted in the department of dermatology and venereology, TMSS Medical College, Bogura, Bangladesh, from January 2021 to December 2022. The study included 200 patients aged 5 to 75. Skin biopsies were obtained from clinically confirmed cases of lichenoid skin lesions and sent for histological investigation. The correlation was then performed with clinical diagnosis. All acquired data was analysed using descriptive statistics in SPSS 11.5. Results: Out of 200 cases evaluated, the most common type of ID was Lichen simplex chronicus (95, 47.5%), with Lichen planus (LP) and its variants coming in second (84, 42%). LP-like keratosis (15, 7.5%), Inflammatory verrucous epidermal nevus (ILVEN) 2 cases, Pityriasis lichenoides et varioliformisacuta (PLE-VA) 2 cases, and Prurigosimplex (PS) 2 cases were the least prevalent. Clinicopathological concordance was seen in 84 (42%) of the lichen planus patients and discordance in 116 (58%) of the cases. Conclusion: In our investigation, the most consistent histological results were basement membrane degenerations such as lymphocytic infiltrates along the dermo-epidermal interface. Interface dermatitis refers to a group of conditions that share clinical and histological characteristics. As a result, comprehensive histological studies are required to identify distinct features of various kinds of interface dermatitis.

What is pityriasis lichenoides?

Pityriasis lichenoides (PL) is an uncommon skin rash. PL has two main forms: Pityriasis lichenoides et varioliformis acuta (PLEVA): this "acute" (fast) form comes on quickly. Pityriasis lichenoides chronica (PLC): this "chronic" (long) form often develops slowly and lasts longer.

Dermoscopy as a diagnostic aid in pityriasis lichenoides etvarioliformis acuta.

Diagnosis of pityriasis lichenoides et varioliformis acuta (PLEVA) is based on the characteristic pattern of lesions in different stages of development, ranging from erythematous maculopapules to papules with a crusted and/or necrotic centre. However, it may raise the differential diagnosis with other entities. It is therefore not uncommon to have to perform skin biopsies to reach a diagnosis, including in infants. In this study, we report the cases of three patients with PLEVA, highlighting the correlations between the clinical, dermoscopic and histological features. Observation of the dermatoscopic findings described, such as punctate or glomerular vessels and erythematous globules surrounding a homogeneous orange or crusty central area, may allow for a rapid diagnosis, avoiding the need for invasive techniques.

Pityriasis Lichenoides; A Case Series With Clinical And Histological Evaluation In A Sample Of Sixteen Iraqi Patients In Najaf

Background: Pityriasis lichenoides is an uncommon papulosquamous disorder of an unknown etiology. The clinical spectrum consists of an acute ulcerative variant, pityriasis lichenoides et varioliformis acuta (PLEVA), and a chronic, non-ulcerative variant, pityriasis lichenoides chronica (PLC). A third, much more rare and aggressive form, febrile ulceronecrotic Mucha–Habermann disease (FUMHD), also occurs. Pityriasis Lichenoides is often benign, with a chronic course and with eventual spontaneous resolution over months to years. Objectives: The current study aims to evaluate the pityriasis lichenoides among patients attending Al-Sader medical city, Najaf, in the last five years, clinically and histologically. Patients and method: This is a case series performed retrospectively over the period from January 2017 through November 2021, in Najaf. Sixteen patients with clinical and histological PL were evaluated according to their medical records and online archives. A histopathological revision of their paraffin-embedded sections was also performed and statistically analyzed. Results: Sixteen patients with pityriasis lichenoides were evaluated; PLC was more common than PLEVA (14:2). PL mainly affects people in the second decade of life (37.5%). The age at the time of diagnosis ranged from 5 to 41 years, with a mean ±SD of 20.1± 9.9 years, which was higher for PLC (21.4±9.7) years than for PLEVA (11±7) years. Females outnumber males in PLC (9:5) as the only two reported cases of PLEVA were males. The skin phototype III was double that of type IV (11:5). More urban patients than rural patients were presented. The cases peak was in 2020 (31.25%), followed by 2021 (18.75). However, the clinical and histopathological features were classic and comparable to the published literature. Conclusion: PL is a disease predominant among young adults; more females were affected by PLC, while males were predominantly affected by PLEVA. More patients, who were more urban than rural, with skin phototype III were affected.

Pityriasis lichenoides presented with skin rash mimicking Urticaria: A case report.

Acute urticaria is urticaria with or without angioedema that is present for less than six weeks, while chronic urticaria is present for more than six weeks. Pityriasis lichenoides (PL) is a benign cutaneous inflammatory disease of unknown etiology. Acute PL typically resolves within a few weeks, while chronic PL lasts several months. The skin rash of PL may resemble the rash of other conditions, so the distinction is essential and depends on history and physical examination and is confirmed by skin biopsy. A 64-year-old gentleman presented with seven days history of generalized itchy skin (hives). Individual lesions last 24-48 hours and do not leave pigmentation or scarring. No systemic involvement. No specific triggers, with two previous similar episodes 30 years and 20 years ago. Levocetirizine, 5 mg tablet, was prescribed, and he was instructed to increase the dose to 4 tablets daily if needed. On reassessing the patient after ten days, he did not respond well. The rash was different from the initial one, with individual lesions lasting for five days or more, so he was referred to a dermatologist for a skin biopsy. Basic investigations were normal. Performing skin biopsy is needed to exclude other pathologies. Skin biopsy showed pathological changes of lichenoid dermatitis compatible with pityriasis lichenoides et varioliformis acuta (PLEVA). He has been treated with azithromycin 250 mg daily for three weeks with rapid and complete resolution without scaring. Urticarial rash may mimic the skin rash of other conditions. Detailed serial history and physical examination are warranted to exclude other diagnoses. Skin biopsy is needed when diagnosing conditions other than urticaria are suspected.

Febrile Ulceronecrotic Mucha-Habermann Disease: A Case Report and Review of Literature in the Paediatric Population

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare fulminant variant of pityriasis lichenoides et varioliformis acuta (PLEVA) that is characterized by a large ulceronecrotic appearance with high fever and a variety of systemic symptoms. We report here a case of FUMHD in a 17-year-old male Chinese patient who was treated successfully with a combination therapy of methotrexate, methylprednisolone, and intravenous immunoglobulin. In addition, a literature review was conducted to summarize the key characteristics of paediatric FUMHD cases.

Acute varioliform parapsoriasis (Mucha–Habermann disease)

Acute varioliform parapsoriasis (Mucha–Habermann disease)

Mortality risk factors in febrile ulceronecrotic Mucha- Habermann disease: A systematic review of therapeutic outcomes and complications.

Febrile Ulceronecrotic Mucha- Habermann Disease (FUMHD) is a variant of Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA). Although rare, the condition may progress to involve serious complications and even lead to fatal outcomes if diagnosis and appropriate treatment is delayed. A PubMed search following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRIMSA) guidelines was performed to find cases of FUMHD from the earliest records to October 2021. Treatments, complications, and patient outcomes were extracted from the literature and summarized, while a review of quality was also performed. A total of 63 publications with 68 patients were found. Successful treatment modalities for FUMHD included antibiotics, antivirals, systemic steroids, Methotrexate (MTX), cyclophosphamide, Cyclosporine (CYA), Intravenous Immunoglobulins (IVIG), pentoxifylline, and ultraviolet B phototherapy. Out of 68 patients, 55 patients had their condition fully resolved and 13 cases were fatal. Increased age, systemic involvement, and monoclonal T-cell receptor rearrangement were associated with worst prognosis, but mucosal involvement did not affect mortality risk. Overall, the publications had low risk of bias, but most lacked adequate follow-up periods. FUMHD is a diagnostic and therapeutic challenge due to the lack of clearly defined diagnostic criteria and optimum treatment. Further studies with larger patient populations and longer follow-up periods may lead to refinement of diagnostic criteria, establish an optimum treatment regimen, and better estimate the likelihood of recurrence.

Type D lymphomatoid papulosis with pityriasis lichenoides et varioliformis acuta-like features in a child with parvovirus infection: a controversial diagnosis in the spectrum of lymphoid proliferations: case report and literature review

BackgroundLymphomatoid papulosis (LyP) is a rare condition in pediatrics; LyP histological type D has been reported in only 7 children. The differential diagnosis of LyP in the spectrum of lymphoid proliferation remains controversial.Case presentationA 6-year-old boy presented to Emergency Department with a 3-week history of an erythematous papulo-vesicular itchy eruption over the submandibular regions, trunk and extremities. History, symptoms and laboratory tests were unremarkable. SARS-CoV-2 antigen was negative. The clinical suspicion of pityriasis lichenoides et varioliformis acuta (PLEVA) was posed, and topical steroids were introduced. One week after, he returned with an extensive painful scaly papulo-erythematous rash, with some ulcerated and necrotic lesions, and fever; therefore the child was hospitalized. Biochemical results were within reference limits, except for high level of C-reactive protein, aspartate aminotransferase, alanine transaminase and bilirubin. Due to a persistently high fever, systemic corticosteroid treatment was administered, with a good clinical response and an improvement of the skin lesions. Anti-PVB-19 Immunoglobulin M was detected. Elevated levels of IL-6, IL-10 and IFN-γ were also recorded. Five days post-admission, most of the lesions had cleared, and the child was discharged. Methotrexate was started, with a positive response. At skin biopsy a “PLEVA-like” pattern was apparent, with a dense, wedge shaped lymphoid infiltrate featuring epidermotropism and morphologically comprising pleomorphic and blastic cells. The pattern of infiltration was highlighted by immunohistochemical stains, which prove the process to feature a CD8+/CD30 + phenotype, the latter being intense on larger cells, with antigenic loss. Polymerase chain reaction for T-cell receptor gamma (TCRG) chain clonality assessment documented a monoclonal peak. A diagnosis of LyP type D was favored.ConclusionThe reported case encompasses most of the critical features of two separated entities—PLEVA and LyP—thus providing further support to the concept of them representing declinations within a sole spectrum of disease. Studying the role of infectious agents as trigger potential in lymphoproliferative cutaneous disorders and detecting novel markers of disease, such as cytokines, could have a crucial impact on pathogenic disease mechanisms and perspective therapies.

Mucha-Habermann Disease: The Quandary Continues

Pityriasis lichenoides et varioliformis acuta (PLEVA) is an uncommon skin disorder, clinically characterized by an acute onset of a polymorphic eruption. The disease has an unpredictable course, the pathogenesis is not fully understood, and many treatments have been proposed while self-resolution may occur. Hence, We report a puzzling case of PLEVA in its early stage of transition to a febrile ulceronecrotic Mucha-Habermann disease.

A Fatal Case of Febrile Ulceronecrotic Mucha-Habermann Disease in a 10-Year-Old Boy.

A Fatal Case of Febrile Ulceronecrotic Mucha-Habermann Disease in a 10-Year-Old Boy.

Wolf's Isotopic Response Seen as a Rare Occurrence of Pityriasis Lichenoides et Varioliformis Acuta (PLEVA) Lesions Over Healed Lesions of Tinea Corporis.

Wolf's Isotopic Response Seen as a Rare Occurrence of Pityriasis Lichenoides et Varioliformis Acuta (PLEVA) Lesions Over Healed Lesions of Tinea Corporis.

Febrile Ulceronecrotic Mucha-Habermann Disease: A Rare Case Report and Review of Cases Treated With Oral Cyclosporine

We present a case of febrile ulceronecrotic Mucha-Habermann disease who presented with widespread erythematous crusted papules, which rapidly progressed to ulceronecrotic lesions accompanied by fever. The serologies showed high titers of cytomegalovirus IgG (&gt;1:3,200) and IgM (1:800). The histopathological study showed epidermal necrosis. The treatment was begun with systemic steroids and roxithromycin to which the patient did not respond. After switching the treatment to methotrexate, the patient was further complicated by getting hepatitis. Low dose cyclosporine resolved the situation within 2 weeks. In patients for whom methotrexate is contra-indicated or ineffective, cyclosporine can suppress T-lymphocyte hyperresponsiveness and resolve this disease.

Methotrexate for pityriasis lichenoides et varioliformis acuta (Mucha-Habermann disease) and pityriasis lichenoides chronica: A retrospective case series of 33 patients with an emphasis on outcomes

To the Editor: Pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC) are rare papulosquamous disorders.1 While PLEVA/PLC can relapse and remit, persistent cases may warrant therapy.1 Methotrexate (MTX) has been investigated previously in 13 pediatric PLEVA/PLC patients (100% response rate), 6 PLEVA patients (100% complete resolution), and 4 PLEVA patients (0% response rate).1-4 We evaluated MTX for PLEVA/PLC in 33 patients.

Diagnostic Value of Plasmacytoid Dendritic Cells in Differentiating Pityriasis Lichenoides et Varioliformis Acuta From Lymphomatoid Papulosis.

Pityriasis lichenoides et varioliformis acuta (PLEVA) and lymphomatoid papulosis (LyP) can often demonstrate clinical and histopathologic overlap. A recent study demonstrated significant plasmacytoid dendritic cell (pDC) recruitment in lesions of PLEVA, whereas another study reported minimal pDC recruitment in lesions of LyP. To confirm the possible diagnostic value of pDCs in differentiating PLEVA and LyP, we compared the presence and distribution of pDCs and myxovirus protein A (MxA) expression (an indirect assessment of pDC activity). In total, 19 cases of PLEVA (16 patients) and 14 cases of LyP (11 patients) were examined using immunohistochemical stains for anti-blood-derived dendritic cell antigen-2 and MxA. Individual semiquantitative scoring systems were used to assess the immunohistochemical results, and a Mann-Whitney test with a subsequent 2-tailed P test was performed for statistical analysis. No statistically significant difference in the number of pDCs in both groups was found. However, most PLEVA cases (84%) demonstrated intense and diffuse MxA expression, whereas LyP cases (71%) demonstrated weak patchy staining (P < 0.007). Our study suggests that although additional studies may be needed to determine whether pDCs are more relevant to the pathogenesis of PLEVA or LyP, pDC activity through MxA staining may play a role in differentiating PLEVA from LyP and may serve as a platform for additional studies.

Pityriasis lichenoides: a clinical and pathological case series of 49 patients with an emphasis on follow-up.

The classification of pityriasis lichenoides (PL) into pityriasis lichenoides et varioliformis acuta (PLEVA), PL chronica (PLC) and febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is based on both clinical and chronological features. In this retrospective monocentric study, we aimed to investigate the relevance of the classification in routine practice. We enrolled 49 patients (25 female, 24 male; median age 41 years). The lesions were papular in 76% of patients, necrotic in 12% and mixed in 12%. We found three histological patterns: 'classic' (65%), 'lymphomatoid' (13%) and 'mild' (22%). The 'lymphomatoid' pattern was associated with necrotic presentation and the 'mild' pattern with papular lesions (P = 0.01). Among the 27 patients with follow-up, 18% had relapses and 44% had chronic disease. One patient had mycosis fungoides. Neither clinical nor histological findings were correlated with disease progression, and are a reflection of the intensity of epidermal injury rather than of the disease course. The term 'pityriasis lichenoides' should be preferred to the classic PLEVA/PLC/FUMHD classification.