Clinical presentation
- Spreading, red, blistering and scaly annular plaques
- Blister, erode and crust over time
- Migrates with new lesions appearing as old ones heal
- May leave post-inflammatory hyperpigmentation
- Mucosal involvement: painful stomatitis and glossitis, angular cheilitis, vaginitis, urethritis
- Nail involvement: onycholysis
Causes
- Glucagonoma (primary cause)
- Pseudoglucagonoma syndrome: liver disease, inflammatory bowel disease
Histology
- Classic napolitan slice appearance: from the surface to the depth, the epidermis has three different staining characteristics: parakeratotic hyperkeratosis that can progress to homogenous eosinophilic necrosis, a layer of pale vacuolated intermediate keratinocytes, relative preservation of the lower layers
Diagnosis
- Glucagon levels >500
- Malignancy screening mandatory
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