Mucha-Habermann disease: a pediatric case report and proposal of a risk score
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Mucha-Habermann disease: a pediatric case report and proposal of a risk score. / Blohm, Martin Ernst; Ebenebe, Chinedu Ulrich; Rau, Cornelius; Escherich, Carolin; Johannsen, Jessika; Escherich, Gabriele; Driemeyer, Joenna; Nagel, Philipp Daniel; Kobbe, Robin; Lütgehetmann, Marc; Lennartz, Maximilian; Booken, Nina; Schneider, Stefan Werner; Singer, Dominique.
In: INT J DERMATOL, Vol. 61, No. 4, 04.2022, p. 401-409.Research output: SCORING: Contribution to journal › SCORING: Review article › Research
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TY - JOUR
T1 - Mucha-Habermann disease: a pediatric case report and proposal of a risk score
AU - Blohm, Martin Ernst
AU - Ebenebe, Chinedu Ulrich
AU - Rau, Cornelius
AU - Escherich, Carolin
AU - Johannsen, Jessika
AU - Escherich, Gabriele
AU - Driemeyer, Joenna
AU - Nagel, Philipp Daniel
AU - Kobbe, Robin
AU - Lütgehetmann, Marc
AU - Lennartz, Maximilian
AU - Booken, Nina
AU - Schneider, Stefan Werner
AU - Singer, Dominique
N1 - © 2021 The Authors. International Journal of Dermatology published by Wiley Periodicals LLC on behalf of the International Society of Dermatology.
PY - 2022/4
Y1 - 2022/4
N2 - 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.
AB - 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.
U2 - 10.1111/ijd.15770
DO - 10.1111/ijd.15770
M3 - SCORING: Review article
C2 - 34287852
VL - 61
SP - 401
EP - 409
JO - INT J DERMATOL
JF - INT J DERMATOL
SN - 0011-9059
IS - 4
ER -
