Acute generalized exanthematous pustulosis
Synonyms: AGEP · Pustular drug eruption · Toxic pustuloderma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- All ages
- Status
- active
A rare hypersensitivity reaction characterized by the rapid development of numerous, nonfollicular, sterile, pinhead-sized pustules on an erythematous base, predominantly occurring on the trunk, intertriginous and flexural areas, with rare, mostly oral, mucosal involvement. Fever, peripheral blood leukocytosis, and mild eosinophilia are accompanying features. Systemic involvement, with hepatic, renal or pulmonary dysfunction, occasionally occurs. Onset usually occurs 1-12 days after administration of the causal medication and is most frequently associated with beta‐lactam antibiotics, macrolides (including pristinamycin and clindamycin), diltiazem, terbinafine, (hydroxy‐)chloroquine but many other medications have also been implicated. Histology reveals spongiform, subcorneal and/or intraepidermal, pustules but this pattern is not specific (same in pustular psoriasis).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acute generalized exanthematous pustulosis | AGEP · Pustular drug eruption · Toxic pustuloderma |
| fr | Pustulose exanthématique aiguë généralisée | AGEP · PEAG |
| de | Akute generalisierte exanthematische Pustulose | AGEP · Arzneimittelexanthem, pustulöses · Pustuloderm, toxisches |
| es | Pustulosis exantemática generalizada aguda | AGEP · Erupción pustulosa por fármacos · Pustuloderma tóxico |
| it | Pustolosi esantematosa generalizzata acuta | AGEP · Eruzione pustolosa da farmaci · Pustoloderma tossico |
| nl | Acute gegeneraliseerde exanthemateuze pustulose | AGEP · Pustuleuze eruptie door medicatie · Toxische pustulodermie |
| pt | Pustulose exantemática generalizada aguda | Erupção pustular por fármaco · PGEA · Pustulodermia tóxica |
| pl | Ostra uogólniona osutka krostkowa | AGEP |
| cs | Akutní generalizovaná exantematózní pustulóza | AGEP · Pustulózní poléková erupce · Toxická pustuloderma |
| uk | Гострий генералізований екзантематозний пустульоз | AGEP · Гнійничкові медикаментозні висипання · Токсична пустульодермія |
| zh | 急性泛发性发疹性脓疱病 | 脓疱性药疹 |
| tr | Akut jeneralize ekzantematöz püstüloz | AGEP · Toksik püstüloderma · Püstüler ilaç erupsiyonu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L27.0 | NTBT | attributed | validated |
| ICD-11 | EH67.0 | E | specific | validated |
| MONDO | 0017384 | E | validated | |
| MeSH | D056150 | E | validated | |
| UMLS | C0877055 | E | validated | |
| MedDRA | 10048799 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:293815Toxic dermatosis
rare skin diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.3 / 100 000 | Worldwide | Value and class | Not yet validated | 11168761[PMID]_ORPHANET |
| Annual incidence | <1 / 1 000 000 | 0.035 / 100 000 | Israel | Value and class | Validated | 18669133[PMID] |
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
23 annotations · validated 2023-04-13| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001974 | Increased total leukocyte count | |
| HP:0200039 | Pustule | |
| Frequent (79-30%) | ||
| HP:0003565 | Elevated erythrocyte sedimentation rate | |
| HP:0032022 | Eosinophilic dermal infiltration | |
| HP:0001945 | Fever | |
| HP:0001880 | Increased total eosinophil count | |
| HP:0011897 | Neutrophilia | |
| HP:0000989 | Pruritus | |
| HP:0040189 | Scaling skin | |
| Occasional (29-5%) | ||
| HP:0100792 | Acantholysis | |
| HP:0100825 | Cheilitis | |
| HP:0001396 | Cholestasis | |
| HP:0002094 | Dyspnea | |
| HP:0002910 | Elevated circulating hepatic transaminase concentration | |
| HP:0000282 | Facial edema | |
| HP:0031236 | Predominantly dermal neutrophilic infiltrate | |
| HP:0000979 | Purpura | |
| HP:0000083 | Renal insufficiency | |
| HP:0200037 | Skin vesicle | |
| Very rare (<4-1%) | ||
| HP:0000509 | Conjunctivitis | |
| HP:0001875 | Decreased total neutrophil count | |
| HP:0000953 | Hyperpigmentation of the skin | |
| HP:0002716 | Lymphadenopathy | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 23855377[PMID]_28084022[PMID]_34409363[PMID]_32926975[PMID]_35187690[PMID]_35054090[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/293173
- This record as JSON: /data/d/293173.json