Publish in this journal
Journal Information
Vol. 17. Issue 9.
Pages 549-551 (November 2021)
Visits
...
Vol. 17. Issue 9.
Pages 549-551 (November 2021)
Case Report
Full text access
Hemorrhagic bullous IgA vasculitis (Schönlein-Henoch purpura), does it have a worse prognosis?
Vasculitis IgA (púrpura de Schönlein-Henoch) hemorrágico-ampollosa, ¿tiene peor pronóstico?
Visits
...
Julia Alonso de la Hoza,
Corresponding author
nereida000@msn.com

Corresponding author.
, Carmen Eugenia Martínez Antequerab, Beatriz Fernández Mansoa, Lucía Llorente Otonesa, Jaime de Inocencio Arocenab,c
a Servicio de Pediatría, Hospital Universitario de Fuenlabrada, Fuenlabrada, Madrid, Spain
b Unidad de Reumatología Pediátrica, Hospital Universitario 12 de Octubre, Madrid, Spain
c Departamento de Salud Pública y Materno-Infantil, Universidad Complutense de Madrid, Madrid, Spain
Article information
Abstract
Full Text
Bibliography
Download PDF
Statistics
Figures (1)
Tables (1)
Table 1. Literature review of paediatric patients diagnosed with haemorrhagic bullous IgA vasculitis.
Abstract

Haemorrhagic bullous form of IgA vasculitis (IgAV), or Schönlein-Henoch purpura, is an unusual presentation of the disease in paediatric patients (<2%). Blistering eruptions can sometimes be very striking, leading to hospital admissions and administration of high-dose steroids and even immunosuppressants. Review of the literature, however, does not suggest that this clinical form carries a worse prognosis than the other forms of IgAV. In fact, the prognosis of the disease depends on the organic involvement.

We present the case of a 5-year-old girl that is very representative. She developed palpable purpura and four days later the skin lesions evolved into blistering lesions. She did not receive any anti-inflammatory nor immunosuppressive treatment and the lesions spontaneously subsided within 14 days. She did not develop any extracutaneous nor systemic involvement.

Keywords:
Case reports
Child
Haemorrhagic bullae
Henoch-Schönlein purpura
Vasculitis
Leukocytoclastic
Resumen

La forma hemorrágico-ampollosa de la vasculitis IgA (VIgA) o púrpura de Schönlein-Henoch, es una de las presentaciones menos frecuentes de la enfermedad en pacientes pediátricos (<2%). Cursa con una afectación cutánea muy llamativa que, con frecuencia, motiva ingresos hospitalarios y tratamiento con corticoides a dosis elevadas, incluso con inmunosupresores. Sin embargo, la revisión de la literatura realizada no sugiere que su pronóstico sea distinto al de otras formas de VIgA, lo que sí parece es que depende de la afectación orgánica existente.

Se presenta el caso de una niña de 5 años que resulta muy representativo. Fue diagnosticada de VIgA hemorrágico-ampollosa, desarrollando lesiones ampollosas 4 días después de la aparición de las lesiones purpúricas. En ningún momento precisó tratamiento antiinflamatorio ni inmunosupresor, resolviéndose las lesiones 14 días después, sin complicaciones significativas.

Palabras clave:
Informes de casos
Niño
Bulas hemorrágicas
Púrpura de Henoch-Schönlein
Vasculitis
Leucocitoclástica
Full Text
Introduction

IgA vasculitis (IgAV) or Schönlein-Henoch purpura is the most common vasculitis in infancy.1–7 Palpable purpura predominantly on the lower limbs2–7,9 is the characteristic cutaneous manifestation for diagnosis,8 although it can also present with vesicles and haemorrhagic blisters.4–7 These lesions are much less frequent in children (<2%)1,4,6,9 than in adults, described in fewer than 90 paediatric patients. Therefore, generally no more than one new case is reported per publication (Table 1).

Table 1.

Literature review of paediatric patients diagnosed with haemorrhagic bullous IgA vasculitis.

Authors  Nº of cases reviewed  Nº of cases reviewed not included by other others  Nº of newly reported cases  Sex  Mean age (years)  Symptomatic treatment  Corticosteroid or immunosuppressant therapy  Progression without treatment  Progression with treatment 
Maguiness et al., 20104  14  14 -----------------------------  ------------------------6  Female: 36%--------------Female: 50%  6.9----------------10  42%*-------------------33%  58%*--------------------------------67%  ---------------------------  --------------------------------- 
Trapani et al., 20106  17  16-----------------------------  ------------------------3  Female: 50%--------------Female: 67%  7.6----------------9  37%-------------------33%  63%--------------------------------67%  ---------------------------Resolution: 100%  --------------------------------- Residual skin lesions: 50% – Resolution: 50% 
Allali et al., 20165  Male  –  100%  –  Resolution: 100% 
Hooper et al., 20169  Male  –  100% (topical clobetasone)  –  Resolution: 100% 
Ramelli et al., 20171  39  –  Female: 83%  7.9  –  –  –  – 
Su et al., 20182  38  27-----------------------------  ------------------------1  Female: 52%--------------Male  7.4----------------15  33%--------------------  67%--------------------------------100%  - Resolution: 67%- Residual skin lesions: 22%- Recurrence: 11%---------------------------  - Resolution: 67% – Residual skin lesions: 28%- Haematuria/proteinuria: 17%-------------------------------Residual skin lesions: 100% 
Mauro et al., 20197  Female  11  –  100%  –  Resolution: 100% 
Nothhaft et al., 20193  39  Male  –  100%  –  Residual skin lesions: 100% 
Current case, 2020    Female  100%  –  Residual skin lesions: 100%  – 
*

The authors had no data on the treatment of 2 of the 14 patients reported.

It is often ascribed a poor prognosis, and results in high rates of hospitalisation and more aggressive treatment such as IV methylprednisolone and immunosuppressants. Since these lesions can occur in the context of IgAV, without being correlated with poor prognosis,1–4,6 it is important to recognise the condition to differentiate it from other entities with similar presentations but with a worse prognosis.2,6

Clinical observation

A healthy 5-year-old girl who, 24 h after onset of fever, cough, and rhinorrhoea, presented with purpuric lesions on the buttocks and lower limbs. She was assessed at her referral hospital where, after taking vital signs and blood (haemogram, coagulation, biochemistry, acute phase reactants) and urine test (urine system), she was diagnosed with IgAV, without complications. On the third day from onset, she began to experience abdominal pain and swelling of the knees and ankles, presenting 24 h later with haemorrhagic blisters. She was assessed at 72 h by paediatric rheumatology, and purpuric lesions and haemorrhagic blisters on the lower limbs were observed (Fig. 1). Her blood pressure and urine system remained unaltered.

Fig. 1.

Purpuric lesions with haemorrhagic blisters on lower limbs.

(0.08MB).

She was diagnosed with haemorrhagic bullous IgA vasculitis, and regular blood pressure and urine test strips were taken, which showed no abnormalities during follow-up. She was kept under watchful waiting and did not require corticosteroids or immunosuppressants. The lesions disappeared after 14 days.

The patient was seen for the last time 6 months after the onset of the symptoms and remained asymptomatic, with only a few residual hyperpigmented lesions on the distal area of the lower limbs.

Discussion

Haemorrhagic bullous IgA vasculitis is rare in children, but relatively frequent in adults. It is essential to be aware of its existence to differentiate it from other processes with similar skin lesions that are serious, such as erythema multiforme, toxicoderma (toxic epidermal necrolysis), infections (bullous impetigo, staphylococcal scalded syndrome), autoimmune diseases (pemphigus), and genetic diseases (epidermolysis bullosa).3,9 If there are diagnostic doubts, a skin biopsy can be performed,1 although some authors argue that, with typical clinical symptoms, as in our case, it is not necessary.3,6,9 Characteristically, the blisters appear less than 14 days after the onset of purpura (median 4 days)1 and resolve within 2–4 weeks.1,6 There is no consensus on their treatment,2,5,7 which is not addressed in the European Consensus either,10 unless there is kidney involvement.10 Most authors agree that watchful waiting is a good option,2–4,9 especially in patients in good general condition, as anti-inflammatory treatment helps to control symptoms, but does not seem to reduce the risk of kidney damage, relapse or secuelae.1 In some patients with extensive skin involvement or severe abdominal pain, corticosteroids,2,3 azathiprine,2,3,6 colchicine,3,5 dapsone2,3 and inmunoglobulins7 have been used with variable efficacy.2–4,7 However, these patients appear to have a similar rate of residual skin lesions.2,3 In this regard, up to 25% of patients with blistering forms have been reported to have skin hyperpigmentation, as was the case in our patient, and/or residual scarring,2,3 which is unusual in other forms of IgAV.

Conclusions

The haemorrhagic bullous form of IgAV is not usually associated with a worse prognosis and does not require different treatment from the usual forms of IgAV.

Conflict of interests

The authors have no conflict of interests to declare.

References
[1]
V. Ramelli, S.A.G. Lava, G.D. Simonetti, M.G. Bianchetti, G.P. Ramelli, G.P. Milani.
Blistering eruptions in childhood Henoch-Schönlein syndrome: a systematic review of the literature.
Eur J Pediatr., 176 (2017), pp. 487-492
[2]
H.-W. Su, C.-Y. Chen, Y.-H. Chiou.
Hemorrhagic bullous lesions in Henoch-Schönlein purpura: a case report and review of the literature.
BMC Pediatr., 18 (2018), pp. 157-162
[3]
M. Nothhaft, J. Klepper, H. Kneitz, T. Meyer, H. Hamm, H. Morbach.
Hemorrhagic bullous Henoch-Schölein purpura: case report and review of the literature.
Front Pediatr., 6 (2019), pp. 413-419
[4]
S. Maguiness, A. Balma-Mena, E. Pope, M. Weinstein.
Bullous Henoch-Schönlein purpura in children: a report of 6 cases and review of the literature.
Clin Pediatr., 49 (2010), pp. 1033-1037
[5]
S. Allali, S. Fraitag, B. Terrier, C. Bodemer, M. Chalumeau.
Efficacy of colchicine in a child with relapsing bullous Henoch-Schönlein purpura.
Eur J Pediatr., 175 (2016), pp. 147-149
[6]
S. Trapani, P. Mariotti, M. Resti, L. Nappini, M. de Martino, F. Falcini.
Severe hemorrhagic bullous lesions in Henoch Schonlein purpura: three pediatric cases and review of the literature.
Rheumatol Int., 30 (2010), pp. 1355-1359
[7]
A. Mauro, S. Mauro, R. Rega, L. Martemucci, R. Sottile.
Successful treatment of hemorrhagic bullous Henoch-Schonlein purpura with intravenous immunoglobulins.
Pediatr Dermatol., 36 (2019), pp. 34-36
[8]
S. Ozen, S.D. Marks, P. Brogan, N. Groot, N. de Graeff, T. Avcin, et al.
European consensus-based recommendations for diagnosis and treatment of Immunoglobulin A vasculitis – the SHARE initiative.
Rheumatology (Oxford)., 58 (2019), pp. 1607-1616
[9]
J.E. Hooper, C. Lee, D. Hindley.
Case report: bullous Henoch-Schönlein purpura.
Arch Dis Child., 101 (2016), pp. 124
[10]
S. Ozen, A. Pistorio, S.M. Iusan, A. Bakkaloglu, T. Herlin, R. Brik, et al.
EULAR/PRINTO/PReS criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification of childhood vascultitides.
Ann Rheum Dis., 69 (2010), pp. 798-806

Please cite this article as: Alonso de la Hoz J, Martínez Antequera CE, Fernández Manso B, Llorente Otones L, de Inocencio Arocena J. Vasculitis IgA (púrpura de Schönlein-Henoch) hemorrágico-ampollosa, ¿tiene peor pronóstico? Reumatol Clin. 2021;17:549–551.

Copyright © 2020. Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología
Idiomas
Reumatología Clínica (English Edition)

Subscribe to our newsletter

Article options
Tools
es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?