Severe Febrile Mucha‐Habermann's Disease in Children: Case Report and Review of the Literature

Abstract

Mucha-Habermann disease, or pityriasis lichenoides et varioliformis acuta, is usually a benign, papulosquamous, cutaneous disorder. It has also been reported in a severe form with fever and systemic symptoms both in children and adults. We report a 12-year-old boy with the febrile, ulceronecrotic type. A review of similar cases in the literature shows a 16% frequency of acute necrotic lesions, as well as rare complications such as fever, superinfected lesions, bacteremia (most often with Staphylococcus aureus), and rheumatologic manifestations such as arthritis and scleroderma. There is no definitive treatment, but tetracycline, erythromycin, methotrexate, and ultraviolet light are used most frequently. The most common histologic feature is mononuclear perivascular infiltrates. Mucha-Habermann disease can mimic other common entities such as varicella and insect bites.

Febrile Ulceronecrotic Pityriasis lichenoides et varioliformis acuta

“…Drugs proposed are corticosteroids, antibiot ics, especially tetracyclines [5] and erythromycin [9J. Pho totherapy (UVB and PUVA) has been reported as success ful in the treatment of PLEVA [6,[10][11][12], More recently, good results have been achieved with methotrexate [6,7], All of the patients with severe ulceronecrotic Mucha Habermann disease reported in the literature healed after several months and different therapies.…”

Pitriyazis Likenoides et Varioliformis Akuta (PLEVA): Olgu sunumu

“…PLEVA'ya sebep olabilecek ajanlar arasında adenovirüsler, Ebstein-Barr Virus, Toxoplasma gondii, Parvovirus B19, Staphylococcus aureus ve Streptococcus pyogenes yer almaktadır (2). Literatürde çoğunlukla Staphylococcus aureus ile gelişen bakteriyemi tabloları bildirilmiştir (9). Bizim hastamızın kan kültüründe üreyen MSSA da PLEVA gelişiminden sorumlu olabilir.…”

Febrile Ulceronecrotic Mucha-Habermann Disease: Treatment with Infliximab and Intravenous Immunoglobulins and Review of the Literature

“…Children tend to have more favorable outcomes and no deaths have been reported in children. In adults, the mortality was attributed to pulmonary thromboembolism [8,34,] pneumonia [4,12,32], cardiac arrest [3], sepsis [9,18,23,28], hypovolemic shock [27], and massive thrombosis of the superior mesenteric artery [34]. The pathogenesis of PLEVA fulminans has not fully been elucidated, although multiple hypotheses exist.…”

“…A febrile ulceronecrotic severe variant of PLEVA, called febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is more destructive and associated to large coalescent skin necrosis with a rapid progression [1]. 48 cases of PLEVA fulminans have been reported to date in the literature with 9 lethal forms (20%) (table 1) [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44]. Oral, genital and conjunctival mucosa can be affected [2,6,9,12,13].…”

“…Oral, genital and conjunctival mucosa can be affected [2,6,9,12,13]. Systemic manifestations are present like high fever, myalgia, arthralgia, gastrointestinal and central nervous system symptoms, interstitial pneumonitis, lymphocytic myocarditis, megaloblastic anemia, pancytopenia, and diffuse intravascular coagulation [1,3,4,8,9,12]. FUMHD mainly occurs in children and young adults in the second and third decades of life, with a predominance of male and a mean age of 27 years.…”