Pityriasis Lichenoides Et Varioliformis Acuta Research Articles

Role of skin biopsy in papulosquamous lesions—a comparative study

Dermatologists make their clinical diagnosis mostly through skin reaction patterns. This study was done to assess the correlation between the clinical diagnoses of papulosquamous lesions made at dermatological clinics and the diagnosis of these lesions at biopsy. Our study lends insight into the list of most common to least common lesions of papulosquamous lesions. This is a cross-sectional study conducted at a dermatological clinic, on those patients who consulted to a dermatologist. This presumptive investigation on papulosquamous lesion was implied on 83 patients as lichen planus, lichen nitidus, lichen striata, lichenoides dermatitis, psoriasis, pityriasis rubra pilaris, pityriasis rosea, pityriasis lichenoides, pityriasis lichenoides et varioliformis acuta (PLEVA) and Reiter’s disease and then the biopsy was performed on these patients for the confirmation of the clinical diagnosis to assess that diagnosis given by biopsy in these patients matched to the clinical diagnosis and whether it showed any deviation from the clinical diagnosis made by dermatologist. The histopathology report confirmed the clinical diagnosis in 67.5 % and gave the diagnosis in 32.5 % of the total number of cases, which means that 32.5 % cases were those in which the diagnosis provided by the biopsy did not match the diagnosis provided by the clinical diagnosis. The total number of cases was 83: lichen planus occurred most frequently, i.e. 32.5 %, biopsy confirmed diagnosis in 21 patients and gave correct diagnosis in six patients, followed by psoriasis 24.1 %. In the case of psoriasis, seven patients were misdiagnosed at clinics, and in these patients, biopsy gave the correct diagnosis of psoriasis. The frequency of pityriasis rubra pilaris and lichenoides dermatitis was the same, 14.5 %. About six patients with pityriasis rubra pilaris and six cases with lichenoides dermatitis were given wrong diagnosis at clinics, and the correct diagnosis was provided at biopsy; PLEVA was diagnosed in 6 % of the cases and pityriasis rosea in 3.6 %. Reiter’s disease occurred in about 2.4 % of the cases and lichen nitidus and lichen striata in 1.2 % of the cases. Biopsy gave the correct diagnosis of one patient with pityriasis rubra pilaris, one with Reiter’s disease and one with PLEVA. This research investigation, postulated and enquired on the basis of histopathology reports, confirmed the clinical hypothesis in 67.5 % of cases and gave the diagnosis in 32.5 % of the total number of cases. These results verified the aforementioned hypothesis that the patients who are diagnosed with the papulosquamous lesion must undergo biopsy for the confirmation of the disease.

Methotrexate Treatment in Children with Febrile Ulceronecrotic Mucha-Habermann Disease: Case Report and Literature Review.

Febrile Ulceronecrotic Mucha-Habermann disease is a rare and potentially fatal variant of pityriasis lichenoides et varioliformis acuta and is characterized by high fever, constitutional symptoms, and acute oncet of ulceronecrotic lesions. We present an 11-year-old male with Febrile Ulceronecrotic Mucha-Habermann disease who was cured with methotrexate and review the use of methotrexate for this disorder in the pediatric age group with the relevant literature.

Association between HLA‐B*13:01 and DIHS/DRESS due to dapson in a Japanese patients of pityriasis lichenoides et varioliformis acuta (PLEVA)

Observation A 38-year old Japanese woman visited our dermatological department on June 24, 2013, with a three-day history of erythematous lesions on the trunk and extremities. She had been treated orally with 50 mg of diaminodiphenyl sulfone (dapson) daily for pityriasis lichenoides et varioliformis acuta (PLEVA) for 38 days. Physical examination on admission revealed fever (38.4°C), bilateral cervical lymphadenopathy, facial edema with erythema, and round erythematous and purpuric plaques on the trunk and extremities. Erythematous targetoid lesions were also found. Blood examination revealed an increased white blood cell count (13.4 ~ 109/l: normal 3.5-9 ~ 109/l), and mild liver dysfunction (alanine aminotransferase; 40 IU/l : normal 5-25 IU/l). A skin biopsy obtained from the patient’s abdomen revealed acanthosis, hydropic and vacuolar degeneration of epidermal basal cells and upper dermal infiltration consisting of lymphocytes and neutrophils. No extensive epidermal necrosis was found. A lymphocyte transformation test (LTT) for dapson was positive (stimulation index (SI) = 5.3, cut-off for LTT, SI = 1.8). These findings fulfilled the criteria for drug-induced hypersensitivity syndrome (DIHS)/drug reaction with eosinophilia and systemic symptoms (DRESS), and dapson therapy was discontinued. The patient was started on a course of systemic prednisolone (1.0 mg/kg daily). The dose was tapered over the next 70 days in line with the improvement of clinical symptoms. Both human herpesvirus-6 and cytomegalovirus reactivation had not been seen during the course. Human leukocyte antigen (HLA)-B*13:01 and HLA-C*03:04 were identified in this patient.

Clinical Remission of Primary Aggressive CD8+Cutaneous T-Cell Lymphoma After Pralatrexate Infusion

Report of a Case A white male patient in his 60s developed pruritic and painful plaques over his upper body. His medical history included Wegner granulomatosis, which was successfully treated with cyclophosphamide in 2006. He took no medications daily and denied any drug allergies. A drug-related cutaneous eruption was considered at the initial evaluation, and sequential courses of corticosteroids and antibiotics for secondary impetiginizationwere prescribed. Biopsy specimens were sent for hematoxylin-eosin staining and direct immunofluorescence. Hematoxylin-eosin staining revealed mild epidermal spongiosis with focal vacuolar alteration along the dermoepidermal junction, dyskeratotic cells, focal exocytosis of lymphocytes, extravasated erythrocytes and superficial polymorphous infiltrate, parakeratosis, and intracorneal neutrophils. Immunostains showed a CD3+ population with a 1:1 ratio of CD4 and CD8+ cells. Results from direct immunofluorescence were negative. Overall, findings favored an acute interface inflammatory process. The eruption progressed overmonths into erosions and ulcers with tumors (Figure 1) involvingoralmucosabut sparingpalms and soles. Repeated biopsies for hematoxylin-eosin staining and direct immunofluorescence ruled out a pemphigus-related disorder. The patient also appeared clinically septic and complained of malaise, anorexia, and severe pain in the skin. Hewas admitted for sepsis at a local hospital, underwent fluid resuscitation, and received intravenous antibiotics. Repeated biopsies showed an upper dermal bandlike CD8-predominant lymphocytic infiltrate associated with papillarydermal fibrosis and focal epidermotropismwithatypia. Immunostains showedan inverted ratioofCD4toCD8(1:1-2),withmild decreases in CD7 and CD3 levels, and few epidermotropic CD30+ cells.CD56andgranzymeBstainedmononuclearcells,but therewas no CD57 staining. Insufficient tissue precluded T-cell rearrangement studies. Although our differential included MuchaHabermann disease, clinicopathologic correlation favored primary aggressiveCD8+cutaneousT-cell lymphoma(CTCL)—specifically, the patient did not develop fever until months later; cutaneous lesions didnot resolvespontaneously; several tumorsdevelopedontheskin; andarthritis, hemoptysis, orgastrointestinal tractbleedingwereabsent; however, oral mucosa was affected. Findings from flowcytometry showednoevidenceof lymphoproliferative disorder, and a positron emission tomography– computer tomography (PET/CT) scan demonstrated skin involvement without nodal or visceral involvement.

Pityriasis lichenoides chronica: opis przypadków – rola czynników infekcyjnych?

ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Chiriac A, Suditu G, Mihaila D, et al. Pityriasis lichenoides chronica: case reports – the role of infectious agents?. Dermatology Review/Przegląd Dermatologiczny. 2014;101(3):192-196. doi:10.5114/dr.2014.43809. APA Chiriac, A., Suditu, G., Mihaila, D., Chirana, A., Chiriac, A., & Foia, L. et al. (2014). Pityriasis lichenoides chronica: case reports – the role of infectious agents?. Dermatology Review/Przegląd Dermatologiczny, 101(3), 192-196. https://doi.org/10.5114/dr.2014.43809 Chicago Chiriac, Anca, Gabriela Suditu, Doina Mihaila, Alice Chirana, Anca E. Chiriac, Liliana Foia, and Caius Solovan et al. 2014. "Pityriasis lichenoides chronica: case reports – the role of infectious agents?". Dermatology Review/Przegląd Dermatologiczny 101 (3): 192-196. doi:10.5114/dr.2014.43809. Harvard Chiriac, A., Suditu, G., Mihaila, D., Chirana, A., Chiriac, A., Foia, L., Solovan, C., and Brzeziński, P. (2014). Pityriasis lichenoides chronica: case reports – the role of infectious agents?. Dermatology Review/Przegląd Dermatologiczny, 101(3), pp.192-196. https://doi.org/10.5114/dr.2014.43809 MLA Chiriac, Anca et al. "Pityriasis lichenoides chronica: case reports – the role of infectious agents?." Dermatology Review/Przegląd Dermatologiczny, vol. 101, no. 3, 2014, pp. 192-196. doi:10.5114/dr.2014.43809. Vancouver Chiriac A, Suditu G, Mihaila D, Chirana A, Chiriac A, Foia L et al. Pityriasis lichenoides chronica: case reports – the role of infectious agents?. Dermatology Review/Przegląd Dermatologiczny. 2014;101(3):192-196. doi:10.5114/dr.2014.43809.

Epstein–Barr virus‐associated T/natural killer‐cell lymphomas in the elderly: The first consensus meeting in Kofu 2013

From a clinicopathological conference on nine elderly patients with Epstein-Barr virus (EBV)-associated T/natural killer (NK)-cell lymphoma, we have addressed the patients' backgrounds, clinical manifestations, histopathological findings, cytogenesis, complications and prognoses. Among these elderly patients (>65years old), seven patients had extranodal NK/T-cell lymphoma, nasal type (ENKL) with an NK-cell phenotype, and two patients had EBV(+) T-cell lymphomas or lymphoproliferative disorders (LPD) with cutaneous lesions mimicking pityriasis lichenoides et varioliformis acuta (PLEVA) or hydroa vacciniforme (HV). No patients had a previous episode of EBV-related symptoms such as infectious mononucleosis, chronic active EBV infection, HV or hypersensitivity to mosquito bites. Elderly patients with ENKL may show the centroblastoid variant. EBV(+) CD8(+) CD56(+/-) lymphocytes may be responsible for the development of PLEVA or HV-like cutaneous lesions in the elderly.

Steroid unresponsive case of ulcerative mucha-habermann disease (febrile ulcernecrotic mucha-habermann disease) treated with methotrexate.

A 20 year old male presented with fever associated with eruption of papules, plaques and vesiculobullous lesions on the chest, back, extremities, palms, soles, and genital mucosa of 20 days duration. Histopathological examination revealed epidermal clefts, edema and vacuolar degeneration of keratinocytes, basal cell degeneration, and dermal perivascular lymphocytic infiltrate. On the basis of clinical features and histology, a diagnosis of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) was made. Treatment with doxycycline (100 mg BD for 4 weeks) and oral prednisolone 60 mg/day tapered to 25 mg in 4 weeks led to initial response that was followed by a relapse on tapering steroid. Addition of methotrexate (7.5 mg increased to 15 mg in 2 weeks) led to a dramatic response.

Febrile Ulceronecrotic Mucha-Habermann Disease in a Young Boy: A Case Report and Review of the Literature

© 2014 The Authors. doi: 10.2340/00015555-1791 Journal Compilation © 2014 Acta Dermato-Venereologica. ISSN 0001-5555 Pityriasis lichenoides et varioriformis acuta (PLEVA), also known as Mucha-Habermann disease, is an uncommon, idiopathic, acquired dermatosis characterised by erythematous and scaly papules that are often accompanied by haemorrhagic and papulonecrotic lesions. The patient’s general health is usually not affected. Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare variant of PLEVA that was first described by Degos et al. in 1966 (1). FUMHD is more destructive and is associated with high fever, systemic symptoms and the development of large coalescent skin necroses which may lead to death. Here we report a young boy with FUMHD successfully treated with systemic corticosteroid.

The relevance of recognizing clinical and morphologic features of pityriasis lichenoides: clinicopathological study of 29 cases

Background:Pityriasis lichenoides (PL) is a lymphoproliferative disease of unknown origin; its diagnosis is based on clinical characteristics and confirmed by histology.Objectives:To describe clinical and histological features of PL in 29 pediatric patients.Materials and Methods:Retrospective descriptive study of children (patients less than 15 years old) diagnosed with PL between 1986 and 2010 at a Reference Service in Pediatric Dermatology from South Brazil.Results:Twenty-nine PL cases were found by chart review in 24 years. Mean age of diagnosis was 8 years (22 to 178 months) and a mean time of diagnosis was 13.8 months (1 to 120 months). Twenty cases (69%) were male. Seasonal correlation was found with colder months in 62% of cases (p<0.01). Clinical diagnosis was pityriasis lichenoides chronica (PLC) in 25 cases, and pityriasis lichenoides et varioliformis acuta (PLEVA) in four. Itching was the main reported symptom occurring in 13 (45%). Fourteen cases had been histologically evaluated. In six, microscopic findings were consistent with PLC, in four consistent with PLEVA, and four biopsies exhibited mixed characteristics of both forms. Concordance between clinical and histological diagnosis was seen in most cases.Conclusion:PL occurs in children and young adults, more commonly in males, and during cold months. PLC was the more frequent clinicohistologic form, and necrotic lesions characterized PLEVA. Associating clinical and histological findings is important for differentiating between PLC and PLEVA diagnosis.

Febrile Ulceronecrotic Mucha-Habermann Disease (Pityriasis Lichenoides et Varioliformis Acuta Fulminans) Associated With Parvovirus Infection

Febrile ulceronecrotic Mucha-Habermann disease is a rare fulminant variant of pityriasis lichenoides et varioliformis acuta, characterized by a rapidly progressive course with predominant ulceronecrotic lesions associated with fever and systemic manifestations. It carries a great morbidity and is potentially fatal. The exact pathogenesis is not clear, and it has been proposed to be the result of hypersensitivity reaction to an infection. We report a patient with febrile ulceronecrotic Mucha-Habermann disease in a 12-year-old boy in whom the condition was most likely precipitated by parvovirus infection, and he showed a favorable response to a combination of prednisolone with narrow band ultraviolet B (NB-UVB) phototherapy.

Mucha-Habermann disease

A case of Mucha-Habermann disease (MHD), possibly associated with macrophage activation syndrome (MAS), is reported. The purpose of this paper was to describe the rare MHD (also known as pityriasis lichenoides et varioliformis acuta – PLEVA) in a 28-year-old male, who presented with generalized ulceronecrotic lesions on the skin and mucosae, gastrointestinal involvement, and heart andliver failure, associated with continuous high fever. The patient might have progressed to MAS and eventually died. The MHD is rare, potentially fatal and has severe systemic complications. The importance of early diagnosis and aggressive treatment is emphasized.

Doença de Mucha-Habermann

A case of Mucha-Habermann disease (MHD), possibly associated with macrophage activation syndrome (MAS), is reported. The purpose of this paper was to describe the rare MHD (also known as pityriasis lichenoides et varioliformis acuta – PLEVA) in a 28-year-old male, who presented with generalized ulceronecrotic lesions on the skin and mucosae, gastrointestinal involvement, and heart andliver failure, associated with continuous high fever. The patient might have progressed to MAS and eventually died. The MHD is rare, potentially fatal and has severe systemic complications. The importance of early diagnosis and aggressive treatment is emphasized.

Varicella Precipitating Febrile Ulceronecrotic Mucha‐Habermann Disease

We report a 9-year-old boy with skin lesions clinically and histologically compatible with pityriasis lichenoides et varioliformis acuta that evolved to the severe variant febrile ulceronecrotic Mucha-Habermann disease and finally to pityriasis lichenoides chronica. Varicella-zoster virus (VZV) was isolated in culture medium from the skin lesions and serum serology was positive for VZV. This is the first time that a virus has been isolated in culture in this condition.

Pentoxifylline and Cyclosporine in the Treatment of Febrile Ulceronecrotic Mucha‐Habermann Disease

We describe two pediatric cases of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) with response to pentoxifylline and cyclosporine therapies. Based on our review of the literature, we are presenting the first case of FUMHD treated with pentoxifylline and the third case of FUMHD successfully treated with cyclosporine. These agents may be of therapeutic benefit in the treatment of FUMHD, in part by suppressing tumor necrosis factor-alpha, which we believe may mediate the disease process.

Phototherapy for pityriasis lichenoides: our experience

Pityriasis lichenoides is a benign disease that includes a continuous spectrum with two polar ends: pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC). Although its benign and self-limited character, treatment is required, both for itch relief and for cosmetic issues. The present study is a retrospective analysis of 13 patients (11 PLC and 2 PLEVA) treated in our institution with psoralen plus ultraviolet A (PUVA) or ultraviolet A combined with ultraviolet B (UVA/UVB) during the period 1998–2011. In the PUVA group, complete response was achieved in five patients and partial response in two. Total cumulative UVA dose was 84.4 J/cm2. One patient quit therapy without therapeutic response. In the UVA/UVB group, complete response was achieved in two patients and partial response in an equal number of patients. One patient did not reach a significant improvement. Total cumulative doses were: 26.1 J/cm2 for UVA and 3.62 J/cm2 for UVB. There were no acute side effects in either therapeutic group. In the present study, PUVA phototherapy was preferred for patients with more widespread or long-evolving disease, while UVA/UVB was selected for patients who presented more recent disease or contraindications for PUVA therapy. Regardless of the absence of clinical guidelines, both therapeutic options proved to be successful, ascertaining phototherapy as an effective and safe option for pityriasis lichenoides patients.

Varicella‐Zoster Virus As a Possible Cause of Pityriasis Lichenoides et Varioliformis Acuta

The etiologic agents for pityriasis lichenoides et varioliformis acuta (PLEVA) are largely unknown, although it has been suggested that foreign antigens such as infectious agents are the pathogenic mechanism. We present a case suggesting a possible relationship between varicella-zoster virus and PLEVA.

Pityriasis Lichenoides et Varioliformis Acuta With Numerous CD30+ Cells

Pityriasis lichenoides comprises a clinicopathologic spectrum of cutaneous inflammatory disorders, with the 2 most common variants being pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica. The aim of the study was to describe 13 cases of a unique PLEVA variant characterized in the conspicuous CD30 component and thus mimicking lymphomatoid papulosis (LyP), a condition currently classified in the spectrum of CD30 lymphoproliferative disorders. The cohort included 10 female and 3 male patients whose ages at diagnosis ranged from 7 to 89 years (mean 41 y; median 39 y). The clinical manifestation was that of PLEVA, with small erythematous macules quickly evolving into necrotic papules. No waxing and waning was seen on follow-up in any of the cases. Histopathologically, typical features of PLEVA were present, but an unusual finding was occurrence of a considerable number of CD30 small lymphocytes as detected immunohistochemically. Over half of the cases also displayed a large number of CD8 cells and showed coexpression of CD8 and CD30 in the intraepidermal and dermal component of the infiltrate. Of the 11 cases of PLEVA studied for T-cell receptor gene rearrangement, 6 evidenced a monoclonal T-cell population, and 5 were polyclonal. Parvovirus B19 (PVB19) DNA was identified in 4 of 10 cases investigated, and positive serology was observed for PVB19 in 2 patients, altogether suggesting that PVB19 is pathogenetically linked to PLEVA at least in a subset of cases. The presence of CD30 lymphocytes and CD8 lymphocytes would be consistent with an inflammatory antiviral response, as CD30, even atypically appearing lymphoid cells have been identified in some viral skin diseases. The main significance of the PLEVA variant is, however, its potential confusion with LyP or some cytotoxic lymphomas. Admittedly, the CD30 PLEVA variant described herein and LyP show considerable overlap if one takes into account all known variations of the 2 conditions recognized in recent years, thus suggesting that LyP and PLEVA may be much more biologically closely related entities than currently thought or can even occur on a clinicopathologic spectrum.

Childhood Pityriasis Lichenoides and Oral Erythromycin

Pityriasis lichenoides (PL) is not uncommon in childhood, but current knowledge about the efficacy of oral erythromycin therapy for its treatment in children is limited. To investigate the role of oral erythromycin therapy in the treatment of PL in children, the records of 24 children with PL who had been started on oral erythromycin treatment at our institution between 2005 and 2008 were retrospectively reviewed. The study included 24 patients (14 male, 10 female) with a median age of 7 years (range 2-14) of whom 15 (62.5%) had PL chronica (PLC), six (25%) PL et varioliformis acuta (PLEVA), and three (2.5%) PLEVA-PLC overlap. History of upper respiratory tract infection was reported in 33% (n = 8) of the patients. History of drug intake and vaccination was noted in 20% (n = 5) and 20% (n = 5), respectively. The disease began during spring (30%, n = 7) or fall (30%, n = 7) in the majority of patients. The median duration of the disease was 11 months (range 1-48 months). Fifteen (68.2%) patients had more than 100 lesions. Distribution was diffuse in 82% (n = 18) of the cases and peripheral in the remainder (n = 6). Oral erythromycin was started at a dosage of 30 to 50 mg/kg per day in three to four divided dosages for 1 to 4 months. Good response was recorded in 64% and 73% of patients in the first and second months of therapy, respectively. Response rate rose to 83% in the third month. In those for whom follow-up data were available (n = 16), relapse was recorded in 12.5% (n = 3). Oral erythromycin may be an effective and well-tolerated treatment option for PL in children and should be continued for at least 3 months.

Febrile Ulceronecrotic Mucha‐Habermann Disease with Central Nervous System Vasculitis

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA). We report a case of FUMHD in an 11-year-old boy who subsequently developed neurologic symptoms and was found to have necrotizing vasculitis on brain biopsy. Over 5 years of follow-up, he had one biopsy-proven recurrence of his skin lesions and continued rehabilitation and treatment for residual neurologic complications. This case provides histological evidence of central nervous system vasculitis associated with FUMHD. Our patient's disease was eventually controlled with cyclophosphamide.

Febrile ulceronecrotic Mucha-Habermann disease: A case with systemic symptoms managed with subcutaneous methotrexate

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare, idiopathic, acquired dermatosis that can affect all ages and ethnic groups. We present a 10-year-old patient with FUMHD associated with arthritis and chronic fatigue, managed with methotrexate. Through our literature review, we also explore treatment protocols for a disease for which internationally standardized management is yet to be formulated.