Henoch schönlein purpura: It's not just for kids

LEARNING OBJECTIVE #1: Be able to recognize key differences between adult and pediatric Henoch Schönlein Purpura. LEARNING OBJECTIVE #2: Be able to identify possible inciting events for adult Henoch Schönlein Purpura. CASE: Henoch Schönlein Purpura (HSP), otherwise known as IgA vasculitis, is a wellrecognized, self-limited disease process in the pediatric population characterized by nonthrombocytopenic purpura, tenosynovitis, gastrointestinal angina, and rarely renal insufficiency. Over the past 20 years, HSP has become increasingly recognized in the adult population and has been strongly associated with gastrointestinal and/or pulmonary malignancies. Unfortunately, the clinical manifestations and natural progression of the disease in the adult population are poorly understood and do not mirror that of the pediatric population. In our case, we have a patient who presented with cutaneous manifestations that progressed to end-stage renal disease despite immunosuppressive therapy, consistent with previous case reports of the aggressive nature of adult-onset HSP. A 61-year-old woman with a past medical history of poorly controlled diabetes (hemoglobin A1c of 11 %) and nicotine dependence presented to the Emergency Department with a 1 week history of a painful, erythematous rash over her bilateral lower extremities initially starting on her tibia and spreading up her legs to include her thighs, lower back, forearms, and face. She had an upper respiratory infection approximately 3 weeks prior, which had completely resolved upon presentation. She denied any abdominal pain, melena, hematochezia, or hematuria. Physical examination revealed an afebrile Caucasian woman who was in obvious pain. Diffuse palpable purpura was noted over her bilateral lower extremities and forearms, along with petechiae over her low back and buttocks with small pustules starting to appear on her face. Other systemic examination was normal. Initial laboratory evaluation was non-revealing including a complete blood count, complete metabolic profile, and urinalysis. C-reactive protein was mildly elevated with a normal erythrocyte sedimentation rate. Work-up for infectious and autoimmune etiologies was negative. Skin biopsy revealed strong IgA deposition in superficial blood vessels suggestive of an IgA predominant vasculitis. Without systemic manifestations, the patient was started on Prednisone 60 mg daily for cutaneous small vessel vasculitis without specific subtype. Over the next 2 weeks, her cutaneous lesions continued to progress, and she developed clawing of her left ring and small finger concerning for mononeuritis multiplex. Concurrently, she developed acute renal failure and worsening of her inflammatory markers. Repeat urinalysis revealed significant hematuria with nephrotic range proteinuria and granular casts. Renal biopsy revealed a proliferative, necrotizing, and crescentic glomerulonephritis with IgA-dominant deposits consistent with HSP. Further work-up to identify respiratory or gastrointestinal malignancy was negative. She was initiated on cyclophosphamide in addition to prednisone with continued progression of her renal and cutaneous manifestations. The decision was made to trial Plasma Exchange Therapy. Unfortunately, she did not regain renal function and was started on hemodialysis with possible future consideration for renal transplant. With aggressive wound care, her cutaneous lesions gradually improved. DISCUSSION: This case illustrates that the presentation of HSP differs significantly with age. Adult patients have a much more aggressive and severe course with over half developing renal insufficiency versus five to fifteen percent in pediatric patients. Likewise, common manifestations in children, such as tenosynovitis and gastrointestinal angina, are frequently absent in the adult population. Adult-onset HSP is also commonly associated with upper respiratory and gastrointestinal cancers; although, gastrointestinal and respiratory infection can still be the inciting event.