Bullous diseases and disorders — Clinical Case (Wikimedia Commons)

Diagnosis: Bullous diseases and disorders

Watercolour drawing of a hand with a bullous eruption, which appeared at intervals on different parts of the body. [Further detail on case available on source catalogue, see link below] Keywords: God. Clinical photograph sourced from Wikimedia Commons (CC BY 4.0). Attribution: Godart, Thomas.

Clinical Presentation

Varies by specific disease: tense bullae on erythematous skin in bullous pemphigoid, flaccid bullae and erosions in pemphigus vulgaris, grouped vesicles on extensor surfaces in dermatitis herpetiformis.

Clinical History

Age of onset varies: pemphigus typically 40-60 years, bullous pemphigoid >60 years. Medication history important (drug-induced pemphigoid). Associated conditions: celiac disease with dermatitis herpetiformis.

Treatment

First-line: systemic corticosteroids. Steroid-sparing: dapsone (DH), mycophenolate, azathioprine, rituximab (refractory pemphigus). Topical: potent corticosteroids for limited BP.

Differential Diagnosis

Bullous pemphigoid, Pemphigus vulgaris, Dermatitis herpetiformis, Linear IgA disease, Epidermolysis bullosa acquisita

Key Learnings

Biopsy TWO sites: lesional for H&E and perilesional for DIF. ELISA for desmoglein 1/3 (pemphigus) and BP180/BP230 (pemphigoid) aids monitoring.

Tags: bullous, autoimmune, pemphigoid, vesiculobullous