Acute generalised exanthematous pustulosis
From IDWiki
Background
- A form of severe cutaneous adverse reaction
- Also known as a pustular drug eruption or toxic pustuloderma
- Typically triggered by medications
- Antibiotics (most common trigger by far): beta-lactams (most common antibiotic trigger), macrolides, fluoroquinolones, tetracyclines, sulfonamides
- Other antimicrobials
- NSAIDs
- Proton pump inhibitors
- Long list of other medications
- Possibly also infections, spider bites, allergens, herbal medications, UV therapy
- Can also be idiopathic, without identified trigger
Clinical Manifestations
- Onset typically within 10 days of starting drug, resolving within 2 weeks of drug discontinuation
- Characteristic rash is diffuse, erythematous, with many pinpoint pustules on an erythematous base
- Lesions typically pinpoint, but can coalesce
- Rash is pruritic or burning
- Typically starts in face or intertriginous areas, then spreads centrally
- Can occasionally involve palms and soles but involvement of oral mucosa is rare
- Followed by desquamation in a collarette pattern over 1-2 weeks
- Can also have systemic symptoms, including subjective or objective fevers
- Typical changes on bloodwork include leukocytosis with neutrophilia, elevated CRP, and occasional mild eosinophilia
EuroSCAR Criteria
| Category | Criterion | Value | Score |
|---|---|---|---|
| Morphology | Pustules | Typical | +2 |
| Compatible | +1 | ||
| Insufficient | 0 | ||
| Erythema | Typical | +2 | |
| Compatible | +1 | ||
| Insufficient | 0 | ||
| Distribution/pattern | Typical | +2 | |
| Compatible | +1 | ||
| Insufficient | 0 | ||
| Post-pustular desquamation | Yes | +1 | |
| No/insufficient | 0 | ||
| Course | Mucosal involvement | Yes | -2 |
| No | 0 | ||
| Acute onset (≤10 days) | Yes | 0 | |
| No | -2 | ||
| Resolution (≤15 days) | Yes | 0 | |
| No | -4 | ||
| Fever (≥38°C) | Yes | +1 | |
| No | 0 | ||
| Neutrophils (≥7000) | Yes | +1 | |
| No | 0 | ||
| Histology | Other disease | -10 | |
| Not representative/no histology | 0 | ||
| Exocytosis of neutrophils | +1 | ||
| Subcorneal and/or intraepidermal nonspongiform or NOS pustule(s) with papillary edema or subcorneal and/or intraepidermal spongiform or NOS pustule(s) without papillary edema | +2 | ||
| Spongiform subcorneal and/or intraepidermal pustule(s) with papillary edema | +3 | ||
Interpretation
| Score | Interpretation |
|---|---|
| ≤0 | no AGEP |
| 1-4 | possible AGEP |
| 5-7 | probable AGEP |
| 8-12 | definite AGEP |
Further Reading
- Acute generalized exanthematous pustulosis: European expert consensus for diagnosis and management. J Eur Acad Dermatol Venereol. 2024;38(1):2073-2081. doi: 10.1111/jdv.20232
