Severe cutaneous adverse drug reactions

87 Pages·2017·1.22 MB·English

Severe cutaneous adverse
drug reactions

Maja Mockenhaupt
Dokumentationszentrum schwerer Hautreaktionen (dZh)
Department of Dermatology
Medical Center – University of Freiburg, Germany
International Registry of Severe Cutaneous Adverse

Reactions (SCAR) to Drugs and Collection
of Biological Samples

SCAR:
- Toxic epidermal necrolysis (TEN)
and recently
GBFDE
- Stevens-Johnson syndrome (SJS)
- Hypersensitivity syndrome (HSS) / Drug reaction with
eosinophilia and systemic symptoms (DRESS)
- Acute generalized exanthematous pustulosis (AGEP)
International Registry of Severe Cutaneous Adverse
Reactions (SCAR) to Drugs and Collection
of Biological Samples
Aims:
- to build an International Registry of SCAR for continuous
surveillance of new drugs
- to organize a centralized collection of biological samples
for immunologic and genetic investigations
- to constitute a cohort of ca. 300 patients in order to study the
outcome, prognostic factors, sequelae and impact on quality
of life
International Registry of Severe Cutaneous Adverse
Reactions (SCAR) to Drugs and Collection
of Biological Samples
Aims:
- to build an International Registry of SCAR for continuous
surveillance of new drugs
- to organize a centralized collection of biological samples
for immunologic and genetic investigations
- to constitute a cohort of ca. 300 patients in order to study the
outcome, prognostic factors, sequelae and impact on quality
of life
Requirements
•  Initiation and organization of a network
•  Definition of clinical entities (phenotypes)
•  Systematic case ascertainment
•  Standardized case validation
•  Professional data management and
statistical analysis
Stevens-Johnson syndrome (SJS) /
Toxic epidermal necrolysis (TEN)
•  in the early phase confluent
macules and flat atypical targets
•  dark colour with necrosis and
detachment of the epidermis
•  hemorrhagic erosions of mucous
membranes
SJS/TEN
Consensus definition

t
n
e (Bastuji-Garin et al, 1993)
m
h
c •  <10%: SJS
a
t
e
d

n
i
k
•  10-30%: SJS/TEN-
s

f
o overlap

t
n
u

o
m
A
•  >30%: TEN
Diagnostics - SJS/TEN
•  check for positive Nikolsky‘s sign
•  Tzanck-test or better cryostat diagnostic
•  conventional skin biopsy and - if needed -

immunofluorescence test
→
specific laboratory parameters
do not exist !
SJS/TEN – Nikolsky‘s sign
Progression - SJS/TEN