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Febrile ulceronecrotic Mucha-Habermann disease is a rare and severe variant of pityriasis lichenoides, characterized by sudden onset of generalized ulceronecrotic papules that rapidly coalesce into ulcers associated with high fever. Systemic manifestations such as intravascular disseminated coagulation and pulmonary, cardiac, gastrointestinal, and central nervous system involvement are common. Treatment is based on oral corticosteroids, immunosuppressive drugs such as methotrexate, and general supportive treatment. The present case describes a stepwise approach to a patient with Mucha-Habermann disease with insufficient response to methotrexate.

Purpose of reviewPityriasis lichenoides (PL) is a spectrum of dermatological conditions involving polymorphous lesions. Natural history of the condition ranges from acute to chronic. Cases of PL following SARS-CoV-2 infection/vaccination have been reported, but not yet comprehensively reviewed. Hence, the objective of this article is to review and summarize cases of PL following SARS-CoV-2 infection/vaccination in order to guide clinicians in its diagnosis and management.Recent findingsPubMed, Embase, and Web of Science were searched for relevant articles. Thirteen articles, consisting of 14 cases of PL following SARS-CoV-2 infection/vaccination, were identified. Males represented 64.3% of cases, and the average age of those affected was 41.4 years. The majority of cases (N = 9, 64.3%) were following SARS-CoV-2 vaccination, the most commonly implicated being Pfizer-BioNTech (n = 8/10, 80%), while four (28.6) followed infection. The overall latency period ranged from 5 days to 1 month. Treatments varied greatly. However, at the time of follow-up, 12/14 patients (85.7%) had either marked improvement or complete resolution of lesions.SummaryThis review cannot determine causality. However, a temporal association was observed with the case reports, and one case of PL followed SARS-CoV-2 infection and recurred with subsequent vaccination, suggesting an association. Nevertheless, risk of developing PL following SARS-CoV-2 infection/vaccination is likely extremely low. There is also the possibility these cases are purely coincidental. Still, clinicians should be aware of this possible etiology when diagnosing a new or exacerbated case of PL. Finally, given that the majority of patients had marked improvement or complete resolution of lesions at the time of follow-up, clinicians should provide reassurance to their affected patients.

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. [ABSTRACT FROM AUTHOR]

Pityriasis lichenoides is an acute and/or chronic skin disease associated with recurrent erythematous papules that self-resolve. While its etiology is unknown, preceding viral infection may play a role. We present an atypical case of a 40-year-old woman with pityriasis lichenoides et varioliformis acuta as a complication of a COVID-19 infection.

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.

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.

Pityriasis lichenoides is an acute and/or chronic skin disease associated with recurrent erythematous papules that self-resolve. While its etiology is unknown, preceding viral infection may play a role. We present an atypical case of a 40-year-old woman with pityriasis lichenoides et varioliformis acuta as a complication of a COVID-19 infection. [ABSTRACT FROM AUTHOR]

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare, potentially fatal subtype of pityriasis lichenoides et varioliformis acuta (PLEVA). Herein, we present a rare case of a 14-year-old male without significant past medical history who was diagnosed with FUMHD without a clear inciting factor. He was effectively treated with systemic corticosteroids with complete resolution of symptoms. [ABSTRACT FROM AUTHOR]

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.

We observed ten children with a papular eruption with purpuric features during the SARS‐CoV‐2 pandemic in Northern Italy (May‐December 2020). Histological examination showed signs of SARS‐CoV‐2‐related dermatosis. Evidence of nucleocapsid viral proteins using SARS‐CoV‐2 (2019‐nCoV) nucleocapsid antibody revealed cuticular staining of the deep portion of the eccrine glands in all cases. [ABSTRACT FROM AUTHOR]

Objective Pityriasis ichenoides consists of Pityriasis Lichenoides et Varioliformis Acuta and Pityriasis Lichenoides Chronica. It might resolve spontaneously and without important sequelae such as lymphoma. This study aims to evaluate the demographic and clinical pictures of both types of pityriasis lichenoides. Methods This is a clinical descriptive study on a long-term basis, in which patients with a clinical picture of pityriasis lichenoides were collected during the period between 2012 and 2020. Any triggering factors before the onset of the disease were noted and biopsies for histopathological evaluation were carried out. Results Fifty-six patients with pityriasis lichenoides were evaluated; no obvious triggering factors were elucidated in both types. The acute form consisted of 16 (80%) males and 4 (20%) females. Their age range was from 5 to30 years with a median of around 17years. All presented with acute generalized erythematous necrotic lesions with burning and itching, simulating chicken pox that left hyperpigmentation after recovery. The chronic form consisted of 36 cases. Their age range was from 3 to 12 years with a median of 7 years; 31 (86%) were males and 5 (13.8%) were females. All cases had a slow course of progression over months and years. Fourteen patients (38.8%) were seen with generalized non-itchy erythematous papules with mica-like scales whereas 24 (66.66%) presented with hypopigmented lesions. Conclusion Both acute and chronic forms are self-limiting conditions that stay for weeks to years and remit spontaneously. They are considered as an immune reaction to specific triggering factors such as an infection. [ABSTRACT FROM AUTHOR]

Copyright of Turkish Journal of Dermatology / Turk Dermatoloji Dergisis is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Competing Interests: None disclosed.

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. [ABSTRACT FROM AUTHOR]

We presented 6 years-old boy who admitted to our clinic with a pre-diagnosis of cutaneous mastocytosis. The child had red-brown colored, diffuse maculopapular eruptions which were more pronounced on his trunk without any pruritus. The other findings on physical examination were normal. We performed punch biopsy from lesions on his skin to reveal the diagnosis. Histopathological examination of biopsy material was consistent with Pityriasis Likenoides et Varilioformis Acuta (PLEVA). He resolved without treatment in the follow-up period. In this report, we examined the clinical symptoms, diagnostic work-up and treatment of PLEVA and discussed the differential diagnosis of PLEVA with mastocytosis. [ABSTRACT FROM AUTHOR]

ABSTRACT 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. [ABSTRACT FROM AUTHOR]

Copyright of Turkish Journal of Dermatology / Turk Dermatoloji Dergisis is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract: Introduction: Abrupt onset of papular lesions, with ulcero necrotic evolution could refers to many dermatitis. Febrile ulceronecrotic Mucha-Habermann disease is an unusual severe form of pitiriasis lichenoides and varioliform acuta (PLEVA) characterized with ulceronecrotic eruption potentially associated with high fever and systemic symptoms leading to death. Lymphomatoid papulosis is a recurrent papulonodular eruption with an initial presentation close to PLEVA. While this disorder usually has a bening course, about 10% of the patients develop lymphoproliferative disorders such as CD30+ lymphoma. Exegesis: The authors reported an acute form of PLEVA in a young man hospitalized in internal medicine unit with fever and ulcero-necrotic papulo-vesicular lesions. Treatment with tetracycline was successful. They also report a case of lymphomatoïd pustulosis that occurred in a 34 years old woman with an extensive nodulo-pustular eruption characterized by central necrosis and ulceration. Histopathological examination revealed CD30 lymphocytic infiltration leading to diagnosis. Conclusion: Theses two dermotosis, uncommun and clinically similar, are both characterized with an unpredictable evolution that to be known by internist. [Copyright &y& Elsevier]

Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica represent 2 ends of a disease spectrum of unknown etiology. Herein we describe 2 cases of pityriasis lichenoides et varioliformis acuta, in which human herpesvirus 7 DNA was detected in skin samples by polymerase chain reaction methodology, an association not previously described. This report may support the involvement of viral infection in the etiopathogeny of this disease., (Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)

Objective: Pityriasis lichenoides et varioliformis acuta (PLEVA) is characterized by 2-3 mm erythematous papules that became vesiculopustular with hemorrhagic necrosis. Methode&Material: A 45-year-old lady, a known case of parathyroid adenoma, presented with a generalized itchy skin rash starting four days after radiocontrast iodide injection. Result: There are some reports of provocation of PLEVA by tegafur and astemizole. Conclusion: We report for the first time the triggering of PLEVA by radiocontrast iodide injection. [ABSTRACT FROM AUTHOR]