Febrile Ulceronecrotic Mucha-Habermann Disease Research Articles

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.

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.

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.

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.

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 (>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.

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.

Ulcerative nodules on the entire body

Ulcerative nodules on the entire body

CASE REPORT OF MUCHA-HABERMANN DISEASE

Pityriasis lichenoides et varioliformis acuta (PLEVA), also known as a Mucha-Habermann disease, is an uncommon, idiopathic, and acquired dermatosis. The disease is characterized by erythematous, scaly, papules, and polymorphic lesions which often progresses to hemorrhagic necrosis and heals with varioliform scarring. A febrile ulceronecrotic variant of PLEVA, also termed pityriasis lichenoides (PL) with ulceronecrosis and hyperthermia (PLUH) or febrile ulceronecrotic Mucha-Habermann disease (FUMHD), is a severe variant of PLEVA. The disease is characterized by the acute onset of large, more destructive, coalescent papules, leading to ulceronecrotic skin lesions associated with high fever and other systemic symptoms. In spite of the presence of multiple treatment modalities with variable success rate, the disease has poor prognosis. Here, we report the case of a 17-year-old male patient who presented with typical features of FUMHD and responded well to systemic administration of corticosteroid therapy.

A Fatal Case of Febrile Ulceronecrotic Mucha-Habermann Disease which Presenting as Toxic Epidermal Necrolysis.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a severe form of pityriasis lichenoides et varioliformis acuta, is an inflammatory dermatosis of unknown etiology manifested by ulcerative and necrotic lesions accompanied by systemic manifestations. The mortality rate of FUMHD is about 15%. It is reported here a case of FUMHD presenting as toxic epidermal necrolysis that resulted in multiple organ failure and death.

Phototherapy for Pityriasis Lichenoides in the Pediatric Population: A Review of the Published Literature.

Pityriasis lichenoides (PL) is a dermatologic disorder that manifests in either the acute (pityriasis lichenoides et varioliformis acuta) or the chronic form (pityriasis lichenoides chronica, also known as parapsoriasis chronica). Traditional first-line therapy consists of corticosteroids or antibiotics; however, these treatments are often accompanied with multiple side effects and may be ineffective. The goal of this study was to review the use of phototherapy for treating PL in the pediatric population. We performed a systematic review of the literature in the National Library of Medicine's PubMed database and the SCOPUS database discussing phototherapy for treatment of PL in the pediatric population. The following search terms were used: 'pityriasis lichenoides', 'pityriasis lichenoides chronica', 'pityriasis lichenoides et varioliformis acuta', and 'febrile ulceronecrotic Mucha-Habermann disease'. The systematic search and screening of articles resulted in 14 articles including a total of 64 patients with PL treated with phototherapy. Three different modalities were utilized, with five studies using broadband ultraviolet B (BB-UVB) radiation, nine studies utilizing narrowband UVB (NB-UVB), and two studies employing psoralen with ultraviolet A (PUVA) therapy. Overall, the use of BB-UVB had an initial clearance rate of 89.6% with 23.1% recurrence, whereas NB-UVB cleared 73% of the lesions with no recurrence, and PUVA therapy initially cleared 83% of the lesions with 60% recurrence. The side-effect profiles were similar and revealed limited toxicity. Phototherapy shows promising results and a favorable side-effect profile in the treatment of PL. Ultimately, large randomized controlled trials are needed to determine optimal treatments.

Febrile ulceronecrotic Mucha-Habermann disease: proposed diagnostic criteria and therapeutic evaluation.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare severe variant of pityriasis lichenoides et varioliformis acuta characterized clinically by aggressive ulceronecrotic skin lesions associated with high fever and histologically by features typical of pityriasis lichenoides et varioliformis acuta. Despite the continuous addition of new case reports, no definite diagnostic criteria have been established, and an optimum treatment is still waiting. Herein, we review the different aspects of this rare entity, including pathogenesis, clinical and histopathological features, differential diagnosis, course, prognosis, and outcome. Different diagnostic and therapeutic challenges associated with FUMHD are also evaluated and discussed. We propose two sets of diagnostic criteria to define the disease more precisely and to avoid missing cases. The first comprises constant clinical and histopathological features that are always present in every case, the combination of which is necessary for diagnosis. The second set includes variable features that may be present in some cases and to which any emerging finding could be added. Although different therapeutic options have been used, there is no optimum therapy for FUMHD, and the disease still represents a therapeutic challenge.

Febrile Ulceronecrotic Mucha-Habermann Disease: Two Cases with Excellent Response to Methotrexate.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a severe form of pityriasis lichenoides et varioliformis acuta (PLEVA), featuring large, ulcerative, necrotic skin plaques, high fever, and other systemic symptoms, is a rare disorder of unknown etiology. No randomized controlled trials have established treatment guidelines and multiple modalities are often employed, making it difficult to assess the efficacy of any single agent. We report two cases of this condition in which treatment with methotrexate plus antibiotic treatment for superinfection led to rapid improvement.

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.

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.

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 (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.

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.

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.