Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta with fatal outcome

Abstract

Ulceronecrotic Mucha-Habermann disease is a severe, febrile form of pityriasis lichenoides et varioliformis acuta. The condition may sometimes have a fatal outcome, especially in elderly patients. In this paper, we are reporting on a 60-year-old male patient who suffered from the eruption of erythematous, haemorrhagic, ulceronecrotic papules accompanied by high temperature. Erosions and ulceronecrotic papules covered over 80% of his body. Three weeks after his admittance to our hospital, clinical signs resembling ileus together with the leucocytosis occurred. The patient was transferred to the department of internal medicine and later to the surgery department. The fulminant course of the disease could not be stopped, and the patient died of severe intestinal and colon gangrene caused by the massive thrombosis of superior mesenteric artery. Up to date, only 23 cases of this severe form of the disease have been reported, and even with the early recognition, fulminant course may lead to death.

Febrile ulceronecrotic Mucha–Habermann disease associated with herpes simplex virus type 2

“…Fatalities have been reported in nine adults between 26 and 82 years of age. [10][11][12][13][14][15][16][17] Severe disease has occurred in paediatric cases, 2,18,19 but no deaths have been reported. Several cases have had milder disease lasting a number of weeks, in which the development of large ulceronecrotic lesions and fever distinguish the condition from typical PLEVA.…”

Febrile ulceronecrotic Mucha-Habermann disease

“…Systemic symptoms in the literature includes: high fever, neurological signs,5 asthenia, alteration of the clinical status, abdominal pain,5 6 diarrhoea,11 interstitial lung disease,12 cardiomyopathy,13 rheumatological manifestations,14 15 megaloblastic anaemia6 16 17 and death 18–22. The causes of death are due to: pneumonia,16 pulmonary thromboembolism,18 23 cardiac arrest,24 sepsis,20 22 23 25 hypovolemic shock21 and massive thrombosis of the superior mesenteric artery 23. The patient often reports of burning and pruritus.…”

Doença de Mucha-Habermann (variante febril úlcero-necrótica) com acometimento mucoso exuberante: relato de caso

“…[7][8][9][10] Levando-se em consideração o seu curso potencialmente grave, faz-se necessária monitoração rigorosa do paciente, para prevenção de desfechos fatais.…”