ACUTE ACUTE ACUTE ACUTE ACUTE ACUTE ACUTE ACUTE GENERALIZED GENERALIZED GENERALIZED GENERALIZED GENERALIZED GENERALIZED GENERALIZED GENERALIZED

EXANTHEMATHOUS EXANTHEMATHOUS EXANTHEMATHOUS EXANTHEMATHOUS EXANTHEMATHOUS EXANTHEMATHOUS EXANTHEMATHOUS EXANTHEMATHOUS EXANTHEMATHOUS EXANTHEMATHOUS EXANTHEMATHOUS EXANTHEMATHOUS EXANTHEMATHOUS EXANTHEMATHOUS EXANTHEMATHOUS EXANTHEMATHOUS PUSTULOSISPUSTULOSISPUSTULOSISPUSTULOSISPUSTULOSISPUSTULOSISPUSTULOSISPUSTULOSIS

CHULEEPORN KONGMEESOOK, M.D.CHULEEPORN KONGMEESOOK, M.D.CHULEEPORN KONGMEESOOK, M.D.CHULEEPORN KONGMEESOOK, M.D.CHULEEPORN KONGMEESOOK, M.D.CHULEEPORN KONGMEESOOK, M.D.CHULEEPORN KONGMEESOOK, M.D.CHULEEPORN KONGMEESOOK, M.D.

OutlineOutline

•• EpidemiologyEpidemiology

•• Clinical featuresClinical features

•• DiagnosisDiagnosis•• DiagnosisDiagnosis

•• TreatmentTreatment

EpidemiologyEpidemiology

•• UnreliableUnreliable

•• 11--5 cases per million per year5 cases per million per year

Alexis Alexis SidoroffSidoroff ChemChem ImmunolImmunol Allergy 2012;97:139Allergy 2012;97:139––148148

Clinical FeaturesClinical Features

•• Numerous, small nonNumerous, small non--follicular, follicular, intraepidermalintraepidermal or or subcornealsubcorneal pustules pustules

(<5 mm) on an (<5 mm) on an erythematouserythematous backgroundbackground

•• Typical Typical histopathologicalhistopathological changeschanges

•• Fever (> 38 Fever (> 38 °°C)C)•• Fever (> 38 Fever (> 38 °°C)C)

•• Blood Blood neutrophilneutrophil counts > 7 counts > 7 ×× 101099/L/L

•• Acute evolution with spontaneous Acute evolution with spontaneous resolution of pustules < 15 daysresolution of pustules < 15 days

MarijnMarijn M. M. SpeeckaertSpeeckaert et al et al Eur J Dermatol 2010; 20 : 425Eur J Dermatol 2010; 20 : 425--3333

Alexis Alexis SidoroffSidoroff ChemChem ImmunolImmunol Allergy 2012;97:139Allergy 2012;97:139––148148

MajaMaja MockenhauptMockenhaupt JDDG 2009 ;7:142JDDG 2009 ;7:142––162162

www.regiscar.uniwww.regiscar.uni--freiburg.de/diseases/agepfreiburg.de/diseases/agep

Differential diagnosisDifferential diagnosis

•• PustularPustular psoriasispsoriasis

•• SubcornealSubcorneal pustularpustular dermatosisdermatosis

•• PustularPustular vasculitisvasculitis•• PustularPustular vasculitisvasculitis

Alexis Alexis SidoroffSidoroff et al et al J J CutanCutan PatholPathol 2001; 28:1132001; 28:113––119119

PustularPustular psoriasispsoriasis

www.dermis.netwww.dermis.net

SubcornealSubcorneal pustularpustular dermatosisdermatosis

www.uptodate.comwww.uptodate.com

PustularPustular vasculitisvasculitis

YoshihideYoshihide Asano et al Asano et al ActaActa DermDerm VenereolVenereol 2010;90:4202010;90:420--421421

HistopathologyHistopathology

MarijnMarijn M. M. SpeeckaertSpeeckaert et al et al Eur J Dermatol Eur J Dermatol 20102010;;2020::425425--3333

EtiologyEtiology

•• Drugs Drugs ((90 90 %%))

•• InfectionsInfections

•• Other factors (spider bite, mercury, Other factors (spider bite, mercury, chemotherapy, herbal medicationschemotherapy, herbal medications))chemotherapy, herbal medicationschemotherapy, herbal medications))

Alexis Alexis SidoroffSidoroff ChemChem ImmunolImmunol Allergy Allergy 20122012;;9797::139139––148148SuranSuran L Fernando Australasian Journal of Dermatology L Fernando Australasian Journal of Dermatology 20122012; ; 5353: : 8787––9292

MarijnMarijn M. M. SpeeckaertSpeeckaert et al et al Eur J Dermatol Eur J Dermatol 20102010;;2020::425425--3333SimaSima Halevy Halevy CurrCurr OpinOpin Allergy Allergy ClinClin ImmunolImmunol 20092009;;99::322322––328328

Alexis Alexis SidoroffSidoroff et al British Journal of Dermatology 2007;157: 989et al British Journal of Dermatology 2007;157: 989––996996

Alexis Alexis SidoroffSidoroff et al British Journal of Dermatology 2007;157: 989et al British Journal of Dermatology 2007;157: 989––996996

Alexis Alexis SidoroffSidoroff et al British Journal of Dermatology 2007;157: 989et al British Journal of Dermatology 2007;157: 989––996996

Alexis Alexis SidoroffSidoroff et al British Journal of Dermatology 2007;157: 989et al British Journal of Dermatology 2007;157: 989––996996

Alexis Alexis SidoroffSidoroff et al et al J J CutanCutan PatholPathol 2001; 28:1132001; 28:113––119119

Min Min JeeJee ChoiChoi et al Ann et al Ann DermatolDermatol 20102010;;2222::163163--169169

DiagnosisDiagnosis

•• Not standardized diagnostic toolsNot standardized diagnostic tools

Alexis Alexis SidoroffSidoroff ChemChem ImmunolImmunol Allergy 2012;97:139Allergy 2012;97:139––148148SuranSuran L Fernando Australasian Journal of Dermatology 2012; 53: 87L Fernando Australasian Journal of Dermatology 2012; 53: 87––9292

TreatmentTreatment

•• SelfSelf--limited diseaselimited disease

•• Discontinue causative drugDiscontinue causative drug

•• Systemic corticosteroid not necessarySystemic corticosteroid not necessary

•• Antibiotics if indicateAntibiotics if indicate

SuranSuran L Fernando Australasian Journal of Dermatology 2012; 53: 87L Fernando Australasian Journal of Dermatology 2012; 53: 87––9292Alexis Alexis SidoroffSidoroff et al et al J J CutanCutan PatholPathol 2001; 28:1132001; 28:113––119119

THANK YOUTHANK YOUTHANK YOUTHANK YOUTHANK YOUTHANK YOUTHANK YOUTHANK YOU

PathophysiologyPathophysiology

MarijnMarijn M. M. SpeeckaertSpeeckaert et al et al Eur J Dermatol Eur J Dermatol 20102010; ; 20 20 : : 425425--3333

MajaMaja MockenhauptMockenhaupt JDDG JDDG 2009 2009 ;;77::142142––162162

MajaMaja MockenhauptMockenhaupt JDDG 2009 ;7:142JDDG 2009 ;7:142––162162

MarijnMarijn M. M. SpeeckaertSpeeckaert et al et al Eur J Dermatol Eur J Dermatol 20102010;;2020::425425--3333

MarijnMarijn M. M. SpeeckaertSpeeckaert et al et al Eur J Dermatol Eur J Dermatol 20102010;;2020::425425--3333