A near fatal case of the dapsone hypersensitivity syndrome in a patient with urticarial vasculitis

Abstract

Dapsone (4,4'-diaminodiphenyl sulphone) is used for a variety of dermatological conditions including immunobullous diseases and urticarial vasculitis. Side-effects are common and include lethargy, headaches, methaemoglobinaemia and haemolysis. Severe adverse effects are rare but the dapsone hypersensitivity syndrome is well recognized. Symptoms include fever, haemolytic anaemia, lymphocytosis and hepatitis. We report a near fatal case of the dapsone hypersensitivity syndrome in a patient with urticarial vasculitis. This diagnosis should be remembered in any patient who becomes unwell whilst taking dapsone.

Dapsone Hypersensitivity Syndrome-related Lung Injury without Eosinophilia in the Bronchoalveolar Lavage Fluid

“…Dapsone hypersensitivity syndrome (DHS) was first described in the 1930's as involving several types of adverse reactions caused by the drug, including skin rashes, fever and eosinophilia (2). DHS occurs in 0.5% to 3% of dapsone-treated patients, with the time of onset from several weeks up to six months after starting therapy (1,(3)(4)(5). DHS is characterized by a hypersensitivity response to dapsone and presents with clinical manifestations, such as fever and skin eruptions.…”

A case report of life threatening Dapsone Hypersensitivity Syndrome

“…7 It can develop several weeks to as long as 6 months after the treatment initiation and the reported incidence range 0.5-3%. 8 Anaemia, oral erosions, conjunctivitis, splenomegaly, atypical lymphocytosis and rise of liver enzymes are the other corroborative findings. All these features need not necessarily be present.…”

Dapsone as a Glucocorticoid-Sparing Agent in Maintenance-Phase Pemphigus Vulgaris

“…However, frequent adverse effects include hemolytic anemia and methemoglobinemia, which may necessitate stopping treatment. Other, rare, adverse effects include blood dyscrasias, exfoliative dermatitis, hepatic damage (which may be dose dependent), 16 mood or other mental changes, peripheral neuritis, and "sulfone syndrome," which has been reported to occur in less than 0.5% to 2% of cases. 17 None of the patients in the present series had to discontinue dapsone therapy owing to an adverse reaction.…”