Treatment of Linear IgA Bullous Dermatosis in Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency
Linear IgA bullous dermatosis requires careful agent selection when standard options carry unacceptable risk. This protocol addresses patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency, dapsone hypersensitivity, severe anaemia, or organ failure — conditions that preclude routine first-line treatment.
Clinical scenario: In patients with glucose-6-phosphate dehydrogenase deficiency, dapsone and certain sulfonamides are associated with significant potential side effects. The same applies where dapsone hypersensitivity, severe anaemia, or organ failure is present. Each of these situations calls for an alternative management pathway.
Treatment approach (partial): When the standard pathway is not viable, the protocol considers systemic agents used in refractory disease — including immunosuppressive and biologic options. The full selection criteria, sequencing, and monitoring guidance are contained in the complete protocol.
References
Systemic corticosteroids may be a primary option when dapsone or sulfonamides cannot be used because of their significant potential side effects (for example, in G6PD deficiency), dapsone hypersensitivity, severe anaemia or organ failure.
The recommended dose is 2g/kg given over 3-5days in monthly intervals.
DOI: 10.1111/jdv.19880Digital
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