Stronglyloides hyperinfection syndrome complicating corticosteroid therapy for pemphigus vulgaris 

We present the case of a 60 year old Vietnamese gentleman with an 8 month history of pemphigus vulgaris, characterised by cutaneous and oral blisters and erosions. Haematoxylin &Eosin of a characteristic lesion demonstrated suprabasal acantholysis and immunoflourescence showed epidermal intercellular IgG deposition. This was controlled with high dose prednisone (150mg daily) and azathioprine (150mg daily) prior to admission.
Fever, vomiting and abdominal pain prompted hospitalization. Blood tests demonstrated a normal eosinophil count, but high C-reactive protein (146mg/L). A chest radiograph revealed non-specific opacification and induced sputum demonstrated pneumocystis jirovecii with silver staining also identifying strongyloides, serology was later confirmative. He was treated with co-trimoxazole, oral albendazole and subcutaneous ivermectin and required mechanical ventilation due to respiratory failure. Corticosteroid doses were weaned to stress doses in the context of life-threatening infection but a flare of his pemphigus vulgaris prompted their up-titration, in addition to intravenous immunoglobulin therapy.
Strongyloides stercoralis is a parasitic nematode. Amongst the immunocompetant, strongyloidiasis is largely asymptomatic or presents with eosiniophilia or mild gastrointestinal symptoms. Stronglyloides hyperinfection syndrome is a life-threatening condition characterised by accelerated autoinfection. In disseminated infection, mortality usually results from secondary gram-negative bacteraemia. Most commonly seen in patients iatrogenically immunosuppressed with corticosteroids1, it has also been reported with other immunosuppressive agents and in patients with haematological malignancies, organ transplantation, HTLV-1 and HIV infection2 3.
Screening for asymptomatic infective conditions prior to initiating immunosuppressive regimes is essential. We advocate baseline testing for strongyloides stercoralis in all at-risk patients with re-evaluation after relevant foreign travel.
Geri G, Rabbat A, Mayaux J, et al. Strongyloides stercoralis hyperinfection syndrome: a case series and a review of the literature. Infection 2015;43(6):691-8. doi: 10.1007/s15010-015-0799-1 [published Online First: 2015/05/27]
Pichard DC, Hensley JR, Williams E, et al. Rapid development of migratory, linear, and serpiginous lesions in association with immunosuppression. Journal of the American Academy of Dermatology 2014;70(6):1130-4. doi: 10.1016/j.jaad.2013.11.036 [published Online First: 2014/05/17]
Mejia R, Nutman TB. Screening, prevention, and treatment for hyperinfection syndrome and disseminated infections caused by Strongyloides stercoralis. Current opinion in infectious diseases 2012;25(4):458-63. doi: 10.1097/QCO.0b013e3283551dbd [published Online First: 2012/06/14]

Dr. Charlotte Thomas

Skin & Cancer Foundation Australia/ St Vincent's Hospital Sydney