A case report of febrile ulceronecrotic Mucha-Habermann disease.

Background: Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe variant of pityriasis lichenoides et varioliformis acuta, characterized by a rapid onset of painful, necrotic skin lesions and systemic symptoms. The diagnosis of FUMHD is complex, hinging on the clinical presentation, histopathological findings, and exclusion of other severe dermatoses. The key diagnostic criteria include sudden development of ulceronecrotic papules and plaques, fever, and evidence of systemic disease. Due to the rarity of FUMHD, there is no consensus on optimal treatment, reflecting a significant gap in the dermatological practice. Case Description: This report details a multimodal approach tailored to our 13-year-old patient, incorporating systemic corticosteroids, immunosuppressive therapy, and intensive supportive care. The strategy was designed to address the acute and aggressive nature of the disease while mitigating potential systemic complications. The report emphasizes on the intricate, multi-layered care required to manage FUMHD, illustrating the challenges and considerations in treating this complex condition. It underscored the necessity of a personalized, comprehensive care plan that extends beyond medical intervention to include psychological and social support. The outcome of our patient was encouraging, with a marked reduction in cutaneous manifestations and improvement in systemic symptoms. Conclusions: It was found that prevention and care of skin injuries and complications, as well as the protection of patient's mental state during the development of the disease, are very important. Therefore, early diagnosis, prompt treatment, close monitoring of infection indicators, and specialized care are essential to improve the prognosis of patients with FUMHD.

Rapid recovery in a child with febrile ulceronecrotic Mucha-Habermann disease following intravenous immunoglobulin administration.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a lymphocyte-mediated inflammatory skin disorder, is considered a severe variant of pityriasis lichenoides et varioliformis acuta that can lead to a fatal outcome if not managed in a timely fashion. Children with FUMHD can have systemic complications involving various organs. The scarcity of reported cases and the absence of well-designed studies or randomized clinical trials to evaluate different therapeutic modalities pose a major challenge in treating this potentially life-threatening disorder. We report a five-year-old child with FUMHD and seizures treated unsuccessfully with a combination of systemic steroids, methotrexate, dapsone, and oral erythromycin, who improved rapidly and achieved disease control with just a single infusion of low-dose intravenous immunoglobulin.

Febrile ulceronecrotic Mucha-Habermann disease during pregnancy: A case report.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe form of 'pityriasis lichenoides et varioliformis acuta', with a progressive and potentially fatal course. To the best of our knowledge, there has been no reported case of FUMDH during pregnancy before. Due to life-threatening nature of the disease and the lack of evidence-based treatment, management of FUMHD in pregnancy is a therapeutic challenge. Additionally, some of the drugs that are effective in the treatment are contraindicated in pregnancy. Herein, we report a 27-year-old woman diagnosed with FUMHD in her 19th week of pregnancy and treated with ceftriaxone and erythromycin.

A Diagnostically Challenging Case of De Novo Febrile Ulceronecrotic Mucha-Habermann Disease with Fatal Pulmonary Involvement: A Case Report.

The febrile ulceronecrotic Mucha-Habermann disease is a rare and potentially lethal variant of pityriasis lichenoides et varioliformis acuta (PLEVA). It is characterized by a sudden onset of ulceronecrotic skin lesions associated with high fever and systemic symptoms. Herein, we report a 23-year-old male, not known to have any medical illnesses, presented with a month-long history of persistent fever of unknown origin associated with a sudden onset of progressive diffuse necrotic ulcers and widespread papulosquamous lesions. Pan CT showed enlarged lymph nodes in the cervix, chest, and abdomen. Unfortunately, a skin biopsy was done late, showing features consistent with PLEVA. Few days after admission, despite being on intravenous methylprednisolone, our patient rapidly deteriorated by showing severe acute respiratory symptoms and consequently died. In spite of the continuous addition of new case reports to the literature, no definite diagnostic criteria have been established, leading to late or missed cases, and an optimum treatment is still waiting.

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.

Febrile Ulceronecrotic Mucha-Habermann Disease: A Case Report and a Systematic Review.

The characteristics and treatments of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) are not well-understood. We reported a FUMHD case, and searched Medline, Embase, Pubmed, Scopus, and Web of Science from inception to June 16, 2021, to perform a systematic review to synthesize its characteristics and treatments. Seventy-eight reports, including 84 people were eligible. Most of them were male (62/83, 74.7%), with high fever state (50/80, 62.5% had a high fever of 39°C or above), and with more positive skin bacterial cultures (31/41, 75.6%). Adults were associated with a higher risk of death (OR = 12.976, 95% CI: 1.049, 160.504, p = 0.046), but not positive blood bacterial cultures (p = 0.102). Systematic corticosteroids combination with other immunosuppressants (methotrexate or cyclosporine) were associated with significantly more effective cases (26/31 = 83.9%, χ2 = 4.065, p = 0.044). Furthermore, no significant differences between the low-dose and high-dose systematic corticosteroid groups were detected in treatment validation (p > 0.05). Overall, FUMHD was associated with male patients, high fever, and positive skin bacterial cultures. Early combination therapy with lower doses of corticosteroids and methotrexate or cyclosporine may be an optimal choice for the treatment of FUMHD.

A Diagnostically Challenging Case of Febrile Ulceronecrotic Mucha-Habermann Disease in an Adult Female Successfully Treated with Methotrexate and Cyclosporine.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by intermittent pyrexia, acute onset of generalized ulceronecrotic lesions, and histopathology suggestive of PLEVA. Prompt diagnosis and treatment are necessary to halt the progression of this potentially fatal disease; however, the widely variable presentation of FUMHD in addition to its rarity poses a diagnostic challenge. We report the case of a previously healthy 43-year-old woman who presented to the emergency department with 1 month of generalized rash and intermittent fevers. Her only reported new exposure were elective intravenous vitamin infusions received at a medi-spa 1 week prior to onset of lesions. Initial evaluations were inconclusive, and confluent ulceronecrotic, hemorrhagic lesions appeared on approximately 90% of her body despite steroid, antibiotic, and cyclosporine therapy. Repeat histopathology was consistent with PLEVA, and in the context of her clinical presentation she was diagnosed with FUMHD. The patient rapidly attained remission with methotrexate therapy but sustained residual scarring.

A case of febrile ulceronecrotic Mucha-Habermann disease with comorbidities.

Febrile ulceronecrotic Mucha-Habermann disease is a very rare and severe variant of pityriasis lichenoides et varioliformis acuta. Adult cases are difficult to diagnose as in the early course they can mimic erythema multiforme or lymphomatoid papulosis. We report a case of a 38-year-old woman who presented with 90% body surface area involvement, fever, diarrhea, malaise and associated comorbidities. She was treated with systemic steroids and methotrexate but suffered a fatal outcome. So far, a total of 65 cases are reported in the literature.

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.

Pitiriasis liquenoide crónica en un adulto mayor: reporte de un caso / Pityriasis lichenoides in an older adult: report of a case

La pitiriasis liquenoide crónica (PLC) es una enfermedad benigna, que se caracteriza por tener un curso gradual, con aparición de múltiples pápulas con escamas adherentes de predominio en tronco y extremidades proximales. Con el objetivo de describir la presentación clínica, discutir el diagnóstico, diagnósticos diferenciales, tratamiento y revisar la literatura se presenta el caso de un hombre de 88 años que presentó múltiples lesiones papuloescamosas en extremidades inferiores. Se realizó biopsia cutánea que confirmó el diagnóstico de PLC. El paciente recibió tratamiento con emulsión hidratante, clobetasol tópico y claritromicina con resolución completa de las lesiones. La pitiriasis liquenoide crónica es un trastorno inflamatorio poco frecuente, de etiología desconocida que ocurre más comúnmente en adultos jóvenes y niños. Si bien la clínica es sugerente, se requiere biopsia cutánea para su confirmación diagnóstica. Es un trastorno benigno, a menudo asintomático y autolimitado, por lo que se debe valorar su tratamiento paciente a paciente. Principalmente se utilizan corticoides tópicos y antibióticos orales (tetraciclinas y eritromicina). Se ha vinculado en escasas publicaciones con el desarrollo de linfoma cutáneo y como síndromes paraneoplásicos, por lo que se sugiere realizar seguimiento.

Chronic lichenoid pityriasis (PLC) is a benign disorder, characterized by a gradual course with the appearance of multiple squamous papules with adherent scales predominating in the trunk and proximal extremities. With the objective to describe its clinical presentation, diagnosis, differential diagnosis, treatment and review literature, we present an 88-yearold male with multiple lesions in the lower extremities of one year of evolution, with papules and adherent scales. A skin biopsy was performed that confirmed the diagnosis of PLC. The patient received treatment with moisturizing emulsion, clobetasol topical and clarithromycin with complete resolution of the lesions. Chronic lichenoid pityriasis is a rare inflammatory disease of unknown etiology that occurs most commonly in young adults and children. Although the clinic is suggestive, a skin biopsy is required for diagnostic confirmation. It is a benign disorder, often asymptomatic and self-limiting, so its patientto- patient treatment should be assessed. Topical corticosteroids and oral antibiotics (tetracyclines and erythromycin) are used. It has been linked in few publications with the development of cutaneous lymphoma and as paraneoplastic syndromes, so it is suggested to follow up.

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 Ulceronecotic Pityriasis Lichenoides et Varioliformis Acuta Successfully Treated with Oral Cyclosporin / 대한피부과학회지

Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta (PLEVA), or febrile ulcerative Mucha-Haberman disease (FUMHD) is very rare, but potentially lethal variants of PLEVA. This subtype is characterized by rapidly progressive ulceronecrotic lesions and systemic manifestations, such as high fever, gastrointestinal, neurological, cardiologic and pulmonary involvement, and rheumatologic manifestations. Several treatments with variable response have been challenged, such as systemic steroid, antibiotics, methotrexate, dapsone, cyclosporine and ultraviolet therapy. But there is no standard therapy for FUMHD to date. We report a 59-year-old woman with FUMHD, who recurred after discontinuation of systemic steroid, but successfully treated with oral cyclosporine.

A Case of Febrile Ulceronecrotic Mucha-Habermann's Disease in an Infant / 대한피부과학회지

Febrile ulceronecrotic Mucha-Habermann's disease (FUMHD) is an unusual severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by abrupt onset of widespread ulceronecrotic cutaneous eruption associated with high fever and systemic symptoms. In most cases, a mild typical form of PLEVA was usually present prior to the acute fulminating course. We report a case of FUMHD in a 6-month-old infant, who presented widespread hemorrhagic papules and coalescent thickly crusted ulcerations, high fever, liver dysfunction, and diarrhea with histopathologic findings suggestive of PLEVA.

Febrile Ulceronecrotic Pityriasis Lichenoides et Varioliformis Acuta in a child / 대한피부과학회지

Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta (PLEVA) is a very rare cutaneous disorder clinically characterized by the acute onset of erythematous papules, vesicles and large coalescent ulceronecrotic lesions on the whole body accompanied by high fever and severe constitutional symptoms. In general, ulceronecrotic lesion is preceded by a typical rash of PLEVA. Histopathologically, it shows widespread epidermal necrosis and fibrinoid necrosis of blood vessel walls in addition to the superficial and deep perivascular lymphocytic infiltrations. Several regimens including systemic corticosteroid, antibiotics, methotrexate or ultraviolet therapy have been challenged in this disorder . We report a typical case of febrile ulceronecrotic PLEVA in a 10-year-old child who showed a dramatic response to methotrexate.

A Case of Febrile Ulceronecrotic Pityriasis Lichenoides et Varioliformis Acuta / 대한피부과학회지

Febrile ulceronecrotic PLEVA is an unusually severe from of PLEVA, characterized by the sudden onset of diffuse ulceronecrotic eruption aasociated with high fever. A mild eruption precede the acute fulminating course, We observed a 13-year-old boy presenting the form of febrile ulceronecrotic PLEVA. The histologic features were those of PLEVA. He received the systemic corticosteroids with aupportive care. To recognize this disorder is important because of a possibly fatal outcome, So close attention and vigorous therapy are necessary.