HEMORRHAGIC BULLOUS HENOCH- SCHONLEIN PURPURA: A CASE REPORT Nirmala Ponnuthurai, Sabeera Begum, Lee Bang Rom Paediatric Dermatology Unit, Institute of Paediatric, Hospital Kuala Lumpur, Malaysia Abstract Henoch Schonlein purpura (HSP) is a vasculitis of small sized blood vessels. It is the most common acute systemic vasculitis in childhood. It mainly affects skin, gastrointestinal tract, joints and kidney. It results from Immunoglobulin A mediated inflammation. The characteristic skin findings are palpable purpuric lesions over the lower limbs and buttocks. The occurance of haemorrhagic bullae in children with HSP is rarely encountered. This case report describes a 12 year old boy with bullous haemorrhagic HSP treated at Institute Paediatric, Hospital Kuala Lumpur. Case Presentation A 12 year old boy presented with multiple purpuric rash over the bilateral lower limbs for 6 days which progressed to haemorrhagic bullous over 2 days duration. It was associated with bilateral ankle swelling and pain. He was otherwise asymptomatic with no urinary symptoms and no abdominal pain. There was also no history of taking any medication prior to developing the symptoms and had no history of fever, upper respiratory tract infection or animal/ insect bite. He has past history of HbH constant spring disease for which a bone marrow transplant was done in 2011 and he has been asymptomatic and well. On examination he was alert, pink. Temperature, 36.6; heart rate 74/min; respiratory rate 24/min; and blood pressure 93/67 mmhg. Skin examination revealed numerous petichiae as well as purplish palpable purpura. There were multiple tense hemorrhagic bullae with surrounding erythematous induration as well as some areas of central necrosis seen over bilateral lower limbs. The rash was symmetrical in distribution over the dorsal aspect of feet and extensor surface of the lower limbs up to the mid shin. At the arms and buttocks there were few purplish palpable purpura seen. There were similar symmetrical hemorrhagic vesicles seen at the dorsal aspect of hands bilaterally. Otherwise his palms, soles, trunk and face were spared. His other systemic examination was unremarkable. His abdomen was soft and non-tender with no organomegaly. Both the ankles were swollen and tender on active and passive movements. Laboratory investigations showed white blood cell count as 6.04 x 10 9 /L with normal differential count, haemoglobin of 13.4 g/dl and platelet count as 279 x 10 9 /L. His coagulation profile was normal as well as normal liver and renal function. Urine analysis showed blood 3+, protein 1+, nitrates negative and leucocyte negative. Urine sediment examination was negative
for casts and crystals with erythrocyte count of 22/npf. Diagnostic considerations included HSP, linear IgA dermatosis and pemphigus. He was started on oral prednisolone 1mg/kg/day and was also prescribed NSAIDS for pain relief. Potassium permanganate wash 1:10 000 was done daily over the lesions. Two 3 mm thickness skin biopsies were done at two different sites and sent for immunofluorescence staining and histopathological examination. The immunoflourecent staining showed perivascular IgA deposition seen at blood vessels whereas his histopathological examination revealed epidermis with mild spongiosis and focal intraepithelial neutrophil infiltration. There were no lichenoid reaction seen. The dermis showed marked perivascular neutrophil infiltration with evidence of fibrinoid necrosis and the deeper dermis and subcutaneous fat (medium sized artery) was normal. These findings of leukocytoclastic vasculitis were consistent with HSP. Distribution of rash
Morphology of Rash With continued treatment the clinical appearance of the lesions improved and he was discharged after 4 days. Oral prednisolone was stopped after 1 week. At 6 week follow up, the lesions showed healing with minimal residual hyper pigmentation. The patient s blood pressure and renal function were normal but proteinuria was persistent at 1 +. Follow up was arranged for monitoring his urine and renal function. Follow up Pictures
Histopathology of HSP Perivascular infiltration of eosinophils and neutrophils seen. The appendages and the large muscular artery appear normal H&E x400 H&E x100 Immunoflouresence for IgA: positive for endothelial wall Discussion HSP is a vasculitis of small sized blood vessels. The dominant features of HSP are cutaneous purpura (100%), arthritis (82%), abdominal pain (63%), GI bleeding (33%) and nephritis (40%). Palpable purpura is seen in almost 100% of patients with HSP and is considered to be one the main reason for hospital visits. The presence of atypical symptoms such as bullae and painful lesions in patients with suspected HSP can complicate diagnosis. Although bullous lesions are relatively common in adult-onset HSP (16-60% patients) they are very rare in pediatric patients (2%). The pathophysiology of haemorrhagic bullous HSP still remains unclear. Severe lesions are often noted at points of maximal pressure such as around the malleoli or dorsal aspect of foot. The matrix metalloproteinases secreted by polymorphoneuclear neutrophils on the dermal side of dermoepidermal junction have been found to be elevated in the blister fluid was postulated to migrate from the lesion of intensive vasculitis and cause degradation of collagen VII in basement membrane. Use of glucocorticoids in HSP remains controversial. Reported benefits of glucocorticoid therapy in HSP has included shortening the duration of abdominal pain, decreasing the risk of intussusception, decreasing HSP recurrence risk, and decreasing the risk of developing persistent renal disease. The use of systemic steroids has been described for patients with bullous HSP to reduce severity of HSP related bullae and its associated painful ulcers and necrosis.
Conclusion Haemorrhagic bullae are rare in children with HSP. The main stay of treatment remains symptomatic treatment and oral prednisolone therapy to reduce the severity of ulceration and necrosis in hemorrhagic bullous HSP. Though mostly recover there are some cases of reoccurrence seen. Further studies are needed enlighten the effects of steroids in the management of cutaneous manifestation of HSP. References [1] Saulsbury FT. Henoch Schonlein purpura in children. Report of 100 patients and review of the literature. Medicine 1999;78:395 409. [2] S. J. Park, J.H.Kim, T.S. Ha, and J. I. Shin, The role of corticosteroid in hemorrhagic bullous Henoch Schonlein purpura, Acta Paediatrica, vol. 100, no.7, pp. e3 e4,2001. [3] Joly P, Roujeau J-C, Benichou J, et al, A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med 2002;346:321 7. [4] Kobayashi T, Sakuraoka K, Iwamoto M, Kurihara S. A case of anaphylactoid purpura with multiple blister formation:possible pathophysiological role of gelatinase (MMP-9) Dermatology, vol 197,no.1,pp.62-64,1918.