Bullous erythema nodosum leprosum (bullous type 2 reaction)

Abstract

A 35-year-old man suffering from lepromatous leprosy with recurrent erythema nodosum leprosum (ENL) (bullous type 2 reaction) was admitted for acute exacerbation of the reaction. He had received regular WHO multibacillary multidrug therapy (WHO-MB-MDT) and corticosteroids for 11 months. The dose of corticosteroids could not be tapered below 20 mg/day due to sudden, frequent, and severe relapses of ENL. In hospital, he suddenly developed hypotension for which he was moved to the intensive care unit and managed. On the third day, he developed multiple, flaccid and tense bullae, some of which were hemorrhagic, over the face, trunk, and proximal extremities (Fig. 1). Some of the ENL lesions showed a central bluish hue. Investigations revealed leukocytosis, thrombocytopenia, and hypokalemia. Blood culture yielded coagulase-negative Staphylococcus aureus. Slit skin smear and smear from the blister fluid showed numerous, fragmented, acid-fast bacilli (AFB) in clusters and many neutrophils (Fig. 2). Skin biopsy revealed a subepidermal bulla that consisted of neutrophils and macrophage granulomas in the dermis together with perivascular neutrophilic infiltration (Fig. 3); however, there were no features suggestive of vasculitis. Fite's stain demonstrated fragmented AFB. Both direct and indirect immunofluorescence studies did not show features of autoimmune bullous disorders; however, moderately strong discontinuous fibrinogen deposits were seen along the basement membrane and around the blood vessels. There were no deposits of immunoglobulin G (IgG) and C3. He was treated with antimicrobials, potassium chloride, and hydrocortisone, 100 mg intravenously, twice daily, which was subsequently switched to oral prednisolone. He responded well and was discharged. Upon lowering the dose of corticosteroids, he again developed severe ENL with fewer bullous lesions. Figure Figure 1 Open in figure viewerPowerPoint Multiple, tense, hemorrhagic bullae over the trunk Figure 2Open in figure viewerPowerPoint Smear from the blister fluid showing numerous, fragmented, acid-fast bacilli (AFB) and neutrophils (Ziehl–Neelsen stain, × 200) Figure 3Open in figure viewerPowerPoint Photomicrograph of the skin biopsy showing subepidermal bulla consisting of neutrophils and macrophage granulomas in the dermis together with perivascular neutrophilic infiltration (hematoxylin and eosin, × 200)