Mucha‐Habermann disease: a pediatric case report and proposal of a risk score

Abstract

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare inflammatory dermatological disease. A case of a 13-year-old boy with FUMHD possibly triggered by mycoplasma infection is presented. Based on FUMHD cases identified in a MEDLINE literature search, demographic, treatment, and outcome data were analyzed. An FUMHD mortality risk score is proposed based on the likelihood ratios of risk factors for a fatal outcome. Our FUMHD case had marked leukopenia and thrombocytopenia at admission. He recovered without systemic immunosuppressive treatment. Literature review revealed 119 FUMHD cases. Overall lethality was 14/119 (12%, CI 6-17%), and lethality in children was lower (1/54, 2%, CI 0-6%) compared to adults (13/65, 20%, CI 11-31%). Risk factors for a fatal outcome (likelihood ratio; P) were sepsis (24.97, P < 0.001), adult vs. pediatric patient age (11.19; P = 0.001), systemic involvement (19.97, P < 0.001), and mucosal involvement (4.58; P = 0.032). The proposed FUMHD mortality risk score = Age/ 10 + 4 + 4 (if systemic involvement) + 1 (if mucosal involvement) was discriminative (sensitivity 93%, specificity 77%). In FUMHD, immune-suppressive treatment intensity should be balanced against the mortality risk, as infectious complications are a frequent cause of death.

Mortality risk factors in febrile ulceronecrotic Mucha- Habermann disease: a systematic review of therapeutic outcomes and complications

“…Blohm et al suggested a mortality risk score based on increased age, systemic involvement, and mucosal involvement increasing the mortality risk. 11 Although increased age and systemic involvement is related to increased risk of mortality in this systematic review, mucosal involvement did not appear to be associated with mortality risk and was proportionally reported in the resolved and fatal group.…”

“…A potential trigger was identified in 17.6% (12/68) of FUMHD cases and were most commonly infectious in etiology including mycoplasma, 11 Varicella Zoster Virus (VZV), 33 parvovirus B19, 45 Epstein- Barr Virus (EBV), 30 , 51 , 66 Herpes Simplex Virus 2 (HSV-2), 56 Cytomegalovirus (CMV), 57 and adenovirus type II. 24 Other potential triggers reported include a spider bite, 38 measles vaccine, 67 and nonfebrile enteritis.…”

“…As a result, the mortality risk score suggested by Blohm et al may already incorporate TCR rearrangement, as age is a factor in the calculation. 11 However, it seems that the degree of monoclonal TCR rearrangement is more important than patient age as a 26-year-old patient succumbed to the disease, 12 while another 24- year-old patient with only mild monoclonal TCR rearrangement was able to recover. 56 …”

“… 6 Blohm et al proposed a calculation for mortality risk that may help guide healthcare providers on indications for more aggressive treatment of patients who are refractory to initial modalities. 11 …”

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

“…Since the disease discovery by Degos et al in 1966, there have been approximately 121 reported cases in the literature, including this case. The overall mortality rate is 12.4%; most deaths occurred in adults [2, 3] . Fever and acute progressive generalized eruption of necrotic papules that rapidly evolve to form necrotic plaques and ulcers that may reach up to a few centimeters in diameter are classic for this disease.…”