Drug-Induced Bullous Sweet Syndrome with Multiple Autoimmune Features

Lund, Jared J.; Stratman, Erik J.; Jose, D. Amilan; Xia, Ling; Wilson, Deborah A.; Moizuddin, Mohammed

doi:10.4061/2010/176749

Cited by 11 publications

(16 citation statements)

References 24 publications

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“…Atypical SS subtypes include ulcerative, papular and nodular, and bullous. The latter is a rare variant (> 60 reported cases) in which neutrophilic bullae predominate in a distribution similar to that of classic SS . Dorsal hand involvement in bullous SS is an uncommon but shared feature seen also in atypical pyoderma gangrenosum (PG) and neutrophilic dermatosis of the dorsal hands (NDDH), thereby making these entities difficult to distinguish clinically.…”

Section: Discussionmentioning

confidence: 99%

“…Standard treatment is oral corticosteroids, which leads to resolution of most cases. Antineutrophilic treatments, including potassium iodide, colchicine, indomethacin, clofazimine, doxycycline, metronidazole and dapsone, can be used in steroid‐refractory cases …”

Section: Discussionmentioning

confidence: 99%

“…The presence of atypical features, including ulcers, haemorrhage and dorsal hand involvement, may lead to consideration of other aetiologies, such as atypical PG and NDDH . Diagnosis of the bullous SS variant is therefore aided by recognition of the atypical clinical presentation, together with the corresponding histopathological features and elevated levels of inflammatory markers on laboratory examination …”

Section: Discussionmentioning

confidence: 99%

“…The latter is a rare variant (> 60 reported cases) in which neutrophilic bullae predominate in a distribution similar to that of classic SS. [2][3][4][5] Dorsal hand involvement in bullous SS is an uncommon but shared feature seen also in atypical pyoderma gangrenosum (PG) and neutrophilic dermatosis of the dorsal hands (NDDH), [1][2][3][4][5][6] thereby making these entities difficult to distinguish clinically. Nevertheless, from a histopathological perspective, both NDDH and atypical PG tend to demonstrate vasculitis, which is typically absent in SS, although leucocytoclasia may be present.…”

Section: Discussionmentioning

confidence: 99%

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Erythema and bullae on the face and hands of a farm worker

2018

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Erythema and bullae on the face and hands of a farm worker

2018

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“…This rash was also in the setting of a S. pyogenes bacteremia which could have resulted in the skin rash. The differential diagnoses for the cutaneous rash were hydralazine associated with early Sweet’s syndrome as described with carbamazepine in the literature [4], cutaneous vasculitis [5], erythroderma, streptococcal-induced cutaneous eruption from hematogenous spread, or an unrelated cause such as a contact dermatitis or early cellulitis.…”

Section: Discussionmentioning

confidence: 99%

Hydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis presenting with a vasculitic syndrome, acute nephritis and a puzzling skin rash: a case report

et al. 2013

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IntroductionAnti-neutrophil cytoplasmic antibody-associated vasculitis has been associated with many drugs and it is a relatively rare side effect of the antihypertensive drug hydralazine. The diagnosis and management of patients who have anti-neutrophil cytoplasmic antibody-associated vasculitis may be challenging because of its relative infrequency, variability of clinical expression and changing nomenclature. The spectrum of anti-neutrophil cytoplasmic antibody-associated vasculitis is wide and can be fatal. This case documents a 62-year-old woman who presented with hydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis with a puzzling cutaneous rash.Case presentationWe report a rare case of hydralazine-induced anti-neutrophil cytoplasmic antibody-associated vasculitis in a 62-year-old Caucasian woman who presented with a vasculitic syndrome with a sore throat, mouth ulcers and otalgia after several months of constitutional symptoms. She then proceeded to develop a rash over her right lower limb. Clinically, the rash had features to suggest Sweet’s syndrome, but also had some appearances consistent with embolic phenomena and did not have the appearance of palpable purpure usually associated with cutaneous vasculitis. Differential diagnoses were hydralazine-associated Sweet’s syndrome, streptococcal-induced cutaneous eruption or an unrelated contact dermatitis. A midstream urine sample detected glomerular blood cells in the setting of anti-neutrophil cytoplasmic antibody-positive renal vasculitis and Streptococcus pyogenes bacteremia. A renal biopsy revealed a pauci-immune, focally necrotizing glomerulonephritis with small crescents. Her skin biopsy revealed a heavy neutrophil infiltrate involving the full thickness of the dermis with no evidence of a leucocytoclastic vasculitis, but was non-specific. She was initially commenced on intravenous lincomycin for her bloodstream infection and subsequently commenced on immunosuppression after cessation of hydralazine. The patient was subsequently discharged from hospital after a rapid clinical improvement.ConclusionHydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis is a rare adverse effect and can present with a severe vasculitic syndrome with multiple organ involvement. Features of this association include the presence of high titres of anti-myeloperoxidase-anti-neutrophil cytoplasmic antibody with multi-antigenicity, positive anti-histone antibodies and the lack of immunoglobulin and complement deposition histopathogically. A rash that is characteristic of Sweet’s syndrome has also been described as an association. Prompt cessation of hydralazine may be sufficient to reverse disease activity but immunosuppression may be needed for definite treatment.

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