Febrile ulceronecrotic Mucha-Habermann disease - a case and treatment review.

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.

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.

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.

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.

A case of febrile ulceronecrotic Mucha-Habermann disease responsive to systemic corticosteroids.

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.

Role of streptococcal infection in the etiopathogenesis of pityriasis lichenoides chronica and the therapeutic efficacy of azithromycin: a randomized controlled trial.

The exact aetiology of pityriasis lichenoides chronica (PLC) remains unknown. While phototherapy is the most investigated therapeutic modality, azithromycin has been used scarcely. The aim of this study is to evaluate the therapeutic efficacy of azithromycin in the treatment of PLC compared to NB-UVB and evaluating the presence of streptococcal infection as a possible etiological factor in PLC patients. The study was designed as a randomised controlled trial. Twenty-four patients with PLC were randomly allocated into either azithromycin (n = 13, standard dose every 10 days) or NB-UVB (n = 11, thrice weekly) groups. End of study (EOS) was either complete clearance of lesions or a maximum of 8 weeks. Therapeutic efficacy was defined as percent reduction in lesions and was calculated for the rash as a whole, erythematous papules alone, and hypopigmented lesions alone and graded into complete, very-good, good, poor or no response. Anti-streptolysin O titre (ASOT), anti-deoxyribonuclease B titre (anti-DNaseB) and throat culture were evaluated at day 0. No significant difference existed between both groups as regards therapeutic efficacy. At EOS, NB-UVB achieved significantly more percent reduction in the extent of hypopigmented lesions and consequently in the rash as a whole (p = 0.001, p = 0.034, respectively). The extent of the rash as a whole was significantly less in the NB-UVB at EOS (p = 0.029, respectively). The effect of NB-UVB on hypopigmented lesions appeared early at week 4 of treatment. Only two patients, one from each group, relapsed during the 3 month follow-up. Evidence of recent streptococcal infection was present in 79% of the cases, mainly in the form of elevated ASOT (94.7%). It was significantly more encountered in young children (< 13 years) (p = 0.03) and was associated with more extent of erythematous papules and consequently with more extent of the rash as a whole (p = 0.05 and p = 0.01, respectively). It did not affect outcome of therapy at EOS. Azithromycin did not show more favorable response in patients with recent streptococcal infection. Therapeutic efficacy of azithromycin is comparable to NB-UVB in treatment of PLC; however, NB-UVB is superior in management of hypopigmented lesions. It is highly suggested that PLC could be a post streptococcal immune mediated disorder.Registration number: ClinicalTrials.gov, NCT03831269.

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.

BACKGROUND: Lymphomatoid 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 PRESENTATION: A 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. CONCLUSION: The 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.

Pityriasis lichenoides acuta (PLEVA) pemphigoides: A rare bullous variant of PLEVA.

Although the clinical presentations of patients with pityriasis lichenoides et varioliformis acuta (PLEVA) may vary, bullae are not usually part of the clinical spectrum. To date, only two other cases of a bullous variant of PLEVA with evidence of autoantibodies against hemidesmosomal antigens have been reported. The term PLEVA pemphigoides was suggested for this unique clinical, pathological and serological combination of both PLEVA and bullous pemphigoid.

Oral erythromycin in pityriasis lichenoides chronica and pityriasis lichenoides et varioliformis acuta.

Pityriasis lichenoides (PL) is an uncommon cutaneous disorder. Oral erythromycin is proposed to be effective in treating the disease. Here, we reported 16 pediatric patients with PL and systematically reviewed published literatures on erythromycin treatment response in pediatric PL patients, to observe the different treatment response to erythromycin in the pityriasis lichenoides chronica (PLC) and the pityriasis lichenoides et varioliformis acuta (PLEVA) groups. Sixteen patients, 8 with PLC and 8 with PLEVA, were treated with erythromycin. In the PLC group, 25% (n = 2) patients responded to erythromycin, while in the PLEVA group, 87.5% (n = 7) patients responded to erythromycin. The response rate was higher in the PLEVA group than the PLC group (P =.05). No side effect was reported in the 16 patients. A total of 34 children including 16 from our studies were included for further descriptive analysis, in which 12 had PLC and 22 had PLEVA. In the PLC group, 41.7% (n = 5) of patients responded to erythromycin while in the PLEVA group, 90.9 % (n = 20) of patients responded. The response rate was higher in the PLEVA group than the PLC group (P = .004). In conclusion, erythromycin is effective and safe in the treatment of children with PL, and erythromycin was more effective in patients with PLEVA than PLC.