Acute generalized exanthematous pustulosis

Summary

Acute generalized exanthematous pustulosis (MONDO:0017384) is a disease and 4 clinical trials. A subtype of dermatitis — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: <1 / 1 000 000 (Israel) [Orphanet-validated]
• Phenotypes (HPO): 23
• Clinical trials: 4

Clinical features

Epidemiology

Prevalence records

2 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	<1 / 1 000 000	0.035	Israel	Validated
Annual incidence	1-9 / 1 000 000	0.3	Worldwide	Not yet validated

Signs & symptoms

Clinical features (HPO)

23 HPO clinical features (Orphanet curated; top 23 by frequency):

HPO ID	Term	Frequency
HP:0001974	Leukocytosis	Very frequent (80-99%)
HP:0200039	Pustule	Very frequent (80-99%)
HP:0000989	Pruritus	Frequent (30-79%)
HP:0001880	Eosinophilia	Frequent (30-79%)
HP:0001945	Fever	Frequent (30-79%)
HP:0003565	Elevated erythrocyte sedimentation rate	Frequent (30-79%)
HP:0011897	Neutrophilia	Frequent (30-79%)
HP:0032022	Eosinophilic dermal infiltration	Frequent (30-79%)
HP:0040189	Scaling skin	Frequent (30-79%)
HP:0000083	Renal insufficiency	Occasional (5-29%)
HP:0000282	Facial edema	Occasional (5-29%)
HP:0000979	Purpura	Occasional (5-29%)
HP:0001396	Cholestasis	Occasional (5-29%)
HP:0002094	Dyspnea	Occasional (5-29%)
HP:0002910	Elevated circulating hepatic transaminase concentration	Occasional (5-29%)
HP:0031236	Predominantly dermal neutrophilic infiltrate	Occasional (5-29%)
HP:0100792	Acantholysis	Occasional (5-29%)
HP:0100825	Cheilitis	Occasional (5-29%)
HP:0200037	Skin vesicle	Occasional (5-29%)
HP:0000509	Conjunctivitis	Very rare (<1-4%)
HP:0000953	Hyperpigmentation of the skin	Very rare (<1-4%)
HP:0001875	Decreased total neutrophil count	Very rare (<1-4%)
HP:0002716	Lymphadenopathy	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	acute generalized exanthematous pustulosis
Mondo ID	MONDO:0017384
MeSH	D056150
Orphanet	293173
NCIT	C112122
SNOMED CT	702617007
UMLS	C0877055
MedGen	209091
GARD	0021164
MedDRA	10048799
Is cancer (heuristic)	no

Also known as: AGEP · pustular drug eruption · toxic pustuloderma

Disease family

This is a subtype of dermatitis. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › integumentary system disorder › skin disorder › dermatitis › acute generalized exanthematous pustulosis

Related subtypes (32): spongiotic dermatitis, atopic eczema, psoriasis, contact dermatitis, urticaria, acneiform dermatitis, acrodermatitis, folliculitis, granuloma annulare, granulomatous dermatitis, lichen planus, neurodermatitis, neurotic excoriation, parapsoriasis, pityriasis rosea, seborrheic dermatitis, acanthosis nigricans, dermatosis papulosa nigra, lichen sclerosus et atrophicus, vitiligo, acne, porphyria cutanea tarda, dermatomyositis, hydroa vacciniforme, autoimmune bullous skin disease, cutaneous vasculitis, skin infection, intertrigo, lipodermatosclerosis, exfoliative dermatitis, radiodermatitis, food dermatitis

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 4.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	4

Top trials by phase / activity

NCT	Phase	Status	Title
NCT03659227	Not specified	RECRUITING	Drug Reactions Sampling (COLLECTIONTOXIDERMIES)
NCT01952275	Not specified	UNKNOWN	Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases
NCT02574988	Not specified	UNKNOWN	Severe Cutaneous Adverse Reactions in Thailand
NCT07428915	Not specified	COMPLETED	Evaluating Legit.Health Plus Support for Improving Diagnosis of Generalized Pustular Psoriasis and Other Skin Conditions Among Primary Care Physicians and Dermatologists