A Rare Presentation of Bullous Henoch-Schoenlein Purpura Complicated by Shock: A Case Report

Abstract

Henoch-Schoenlein purpura (HSP) is an immunoglobulin A mediated vasculitis and mainly affects skin, joints, gastrointestinal tract, and kidneys. Typically, skin lesions present as erythematous maculopapular, petechiae, and purpuric rash and atypically as a bullous lesion. A 6-year-old female presented with bullous lesions over her hands and legs, oligoarthritis, abdominal pain complicated with hypotensive shock. The patient was managed with fluid resuscitation, corticosteroids, and antibiotics which offered significant improvement. Bullous IgA vasculitis complicated by non-hemorrhagic hypovolemic shock is rare and may pose diagnostic challenges. Early recognition and timely management are essential to prevent morbidity.

Introduction

Henoch-Schoenlein purpura (HSP) is an IgA mediated vasculitis and is one of the most common systemic vasculitis in children between 2 and 10 years with annual incidence of 10 to 20 cases/100 000 children. ¹

It presents as a tetrad of purpura with predominance in lower extremities, oligoarthritis, abdominal pain, and renal involvement. ² Skin lesions usually presents as erythematous macules, papules, patches, and purpura. ³ Rarely, 2% of pediatric HSP presents as a bullous rash. ² However the coexistence of bullous lesions complicated by non-hemorrhagic hypovolemic shock is rarely found in children.

We report a rare case of pediatric HSP presenting with a bullous lesion and complicated by shock.

Case Report

Case Presentation

A previously healthy 6-year-old female presented with a 5-day history of palpable rash over both lower limbs, associated with abdominal pain and swelling of ankle and knee joints. She also had decreased oral intake and multiple episodes of non-bilious vomiting. There was history of decreased urine output for 1 day prior to presentation.

There was no history of fever, recent infection, drug intake, vaccination, or prior hospitalization. There was a history of unintentional weight loss of 5 kg over the preceding 4 months.

Examination

On examination, the child was dehydrated. Her blood pressure was 80/40 mm Hg (below fifth BP centile) clinically indicating the child was in hypotensive shock. Other vital findings were appropriate for her age. On anthropometry, weight for age was 13 kg (<−3 SD), height for age was 107 cm (−2 to −1 SD), and BMI for age was 11.3 kg/m² (<−3 SD) indicating severe undernutrition. Systemic examination findings were insignificant.

Cutaneous examination revealed multiple palpable purpuric lesions distributed over the bilateral lower limbs, buttocks, and hands. The lesions were pruritic but non-tender. Additionally, erythematous plaques with overlying clustered vesicles and bullae were noted over both hands and legs.

Musculoskeletal examination revealed swelling and tenderness of bilateral ankle and knee joints consistent with oligoarthritis (Figures 1 and 2).

Figure 1.

Bullous lesion in dorsum of right hand.

Figure 2.

Lesions of buttock.

Investigations

Complete blood count, renal function tests, serum electrolytes, complement levels (C3, C4), and urinalysis were within normal limits. ANA was performed as part of vasculitis evaluation and was negative. Abdominal ultrasonography revealed mild ascites without evidence of other abnormality. Bullous fluid aspiration was done and culture was sterile. Skin swabs and blood cultures were sterile (Table 1).

Table 1.

Investigation Summary.

Investigation	Result	Remarks
Complete blood count	Within normal limits	No cytopenia or leukocytosis
Renal function tests	Within normal limits	No evidence of renal impairment
Serum electrolytes	Within normal limits	No dyselectrolytemia
Complement levels (C3, C4)	Within normal limits	No hypocomplementemia
Urinalysis	Normal	No hematuria or proteinuria
ANA	Negative	Performed as part of vasculitis workup
Abdominal ultrasonography	Mild ascites	No other abnormality detected
Bullous fluid culture	Sterile	No bacterial growth
Skin swab culture	Sterile	No bacterial growth
Blood culture	Sterile	No bacteremia

Abbreviation: ANA, antinuclear antibody.

Management

Based on the presence of palpable purpura, oligoarthritis, abdominal pain, oliguria, and characteristic bullous lesions, a diagnosis of bullous HSP was considered.

The child was suspected to be in hypovolemic shock and was managed with fluid bolus at 20 ml/kg. Further, IV Clindamycin 30 mg/kg/day and IV Ceftriaxone 100 mg/kg/day were administered in view of extensive bullous lesion with suspected secondary infection. Then, treatment was started with oral Prednisolone at 2 mg/kg/day for 2 weeks and then tapered. Dermatology consultation was made and her bullae was aspirated, culture of which was sterile. Her lesions were managed with topical fusidic acid and betamethasone 2 times a day for 2 weeks. Oliguria improved following fluid resuscitation, and urine output normalized during hospital stay. There was improvement in rash, joint pain, and abdominal pain after 7 days. The patient is currently under weekly follow up with urinalysis and serum creatinine.

Follow Up

At 4 week follow up, the child remained asymptomatic with normal blood pressure and normal urinalysis report. She continues to be monitored weekly with urinalysis reports for next 1 month.

Discussion

Henoch-Schonlein purpura (HSP), or IgA vasculitis is the most common systemic vasculitis in children. It is characterized by tetrad of clinical manifestations, which includes palpable purpura, abdominal pain, joint pain, and renal disease. ⁴ While, HSP is a common systemic manifestation, its initial presentation as bullous skin lesions with coexisting hypovolemic shock and severe malnutrition can be a diagnostic challenge.

According to EULAR criteria, diagnosis requires purpura or petechiae with lower limb predominance plus 1 of 4 features (abdominal pain, IgA histopathology, arthritis/arthralgia, or renal involvement). ⁵ Our patient fulfilled these criteria with palpable purpura, abdominal pain, and arthritis involving ankle and knee joints, supporting the diagnosis for HSP despite atypical bullous presentation and shock. Most of the available literature are limited to case series and case reports of bullous HSP showing the rarity of its presentation.^3,6,7

Etiological factors like preceding upper respiratory infection, drugs, vaccinations, and malignancies are said to have a role in HSP and subsequent risk of the disease is also said to be higher in children with preceding allergic conditions such as asthma. ⁸ However, in our patient there was no such past history suggesting the disease may have other unknown trigger.

The exact pathogenesis of IgA vasculitis is not known however, genetic factors, disrupted mucosal immunity, and immune complexes with abnormal IgA or IgA antibodies are said to be involved in pathogenesis. ⁹ This leads to subsequent disruption of small blood vessels of GI tract, kidney, joints, and skin.

IgA vasculitis nephritis has a similar pathogenesis to IgA nephropathy which involves a series of processes and is triggered by infections in a genetically predisposed individuals. ¹⁰ The key event is the formation of galactose-deficient IgA1 (Gd-IgA1), leading to circulating immune complex formation and deposition in small vessels and the renal mesangium, causing mesangial proliferation, cytokine release, and complement activation (mainly via alternative and lectin pathways), resulting in inflammation and vascular injury, and further amplified by Toll-like receptor activation and anti-endothelial cell antibodies. ¹⁰

Watanabe and Tsukano reported that blistering lesions in HSP showed epidermal vesicles with neutrophil infiltration, leukocytoclastic vasculitis, and IgA deposits. ¹¹ Kobayashi et al suggested that neutrophil-derived MMP-9 may contribute to blister formation by degrading type VII collagen in the basement membrane. ¹² This might be cause for bullae formation in our case.

The presence of bullous lesions in HSP might be misleading with other causes like toxic epidermal necrolysis, erythema multiform, pemphigus, bullous impetigo, dermatitis herpetiformis, and staphylococcal-scalded skin syndrome. ⁶ Thus, the fulfillment of the criteria for HSP along with all the essential lab investigations pointed our diagnosis for HSP.

Bullous lesion in HSP has a predilection to spread over other body parts including face and result in ulcer, necrosis, and scarring. While the exact cause of HSP remains unknown the formation of bullous lesion is likely to be due to pressure factors. ¹ In majority of reported cases of HSP, bullous lesions were present in the sites of classic HSP lesions like lower extremities.^3,7 In our case, bullous lesions involved those regions of skin where classic purpuric rashes first appeared supporting the finding of the available literature.

Hypovolemic shock is not a direct manifestation of IgA vasculitis and it may occur secondary to persistent vomiting and poor oral intake. Many available literatures suggested gastrointestinal hemorrhage, sepsis, or renal involvement as a probable cause. The rapid improvement in Blood Pressure of the patient following fluid bolus suggests that the likely case of shock is fluid loss secondary to vomiting, poor oral intake, and other ongoing systemic inflammation in the body including gastrointestinal (GI) tract. Presence of mild ascites in abdominal ultrasonography supports our finding of third space fluid loss which might be secondary to systemic inflammation in the body.

Malnutrition can occur due to various underlying systemic diseases. ¹³ In our case the child was suffering from preexisting malnutrition as evidenced by the history of significant weight loss over 4 months which is earlier than the onset of IgA vasculitis. Therefore it is unlikely to be a manifestation of HSP. However, acute gastrointestinal presentations like vomiting and decreased oral intake during illness may have worsened the nutritional deficit. Although causes like inflammatory bowel disease can be associated with IgA vasculitis, evaluation for these condition was not possible in our resource limited setting.

Management of IgA vasculitis is primarily supportive, including adequate hydration, pain relief, and nutrition.^1,14 Corticosteroids are used in cases with significant joint involvement and severe abdominal pain or other complications. ¹ A randomized controlled trial has demonstrated that early corticosteroid therapy can reduce the duration and severity of joint pain and abdominal pain but it does not prevent renal involvement. ¹⁵

In our patient, prednisolone was started in view of persistent joint and abdominal pain despite use of NSAIDs following which rapid clinical improvement was noted over 1 week. Topical corticosteroids and antibiotics facilitated resolution of the bullous lesions with secondary infection. Adequate fluid resuscitation corrected hypotension and restored urine output, highlighting the importance of early recognition and supportive management in complicated presentations.

Conclusion

HSP may present atypically as bullous lesions which might be diagnostically challenging. The presence of typical features of IgA vasculitis may help in diagnosis and timely intervention. This case highlights the importance of recognizing atypical presentations and considering IgA vasculitis in children presenting with bullous lesion, even in the presence of complications such as dehydration and shock secondary to vomiting and poor oral intake. IgA vasculitis must be managed focusing on symptomatic relief, use of steroids appropriately and restoring overall nutrition and growth of the child and follow up with urinalysis and renal function to look for HSPN.