Charles B. Chen, MD; Srikanth Garlapati, MD; Jeffrey D. Lancaster, MD; Zachary Zinn, MD; Patrick Bacaj, MD; Kamakshya P. Patra, MD
From the West Virginia University School of Medicine, Morgantown. Drs. Chen, Garlapati, Lancaster, and Patra are from the Department of Pediatrics; Dr. Zinn is from the Department of Dermatology; and Dr. Bacaj is from the Department of Pathology.
The authors report no conflict of interest.
Correspondence: Kamakshya P. Patra, MD, Department of Pediatrics, West Virginia University Children’s Hospital, 1 Medical Center Dr, PO Box 9214, Morgantown, WV 26506 (kapatra@hsc.wvu.edu).
Although bullous lesions are relatively common in adult-onset HSP (16%–60% of patients), they are very rare in pediatric patients (2% of patients).2-4 We performed a PubMed search of articles indexed for MEDLINE for bullous Henoch-Schönlein purpura in childhood using the search term Henoch-Schönlein purpura and bullous. The Table provides a summary of our search results from the English-language literature.5-22
Bullae often develop on several parts of the body but are more commonly observed on the legs.17 Pathergy and edema have been implicated in the pathogenesis, as these findings have been observed in sites such as malleoli and legs, respectively.12 Matrix metalloproteinases secreted in polymorphonuclear neutrophils have been found to be elevated in blister fluid and can cause bullae formation via degrading collagen in the basement membrane.9 Corticosteroids, by virtue of their inhibition of proinflammatory transcription factors (eg, nuclear factor κβ, intranuclear activator protein 1) and decreasing metalloproteinase levels, may be efficacious in bullous HSP. Although there is no consensus, corticosteroid therapy seems to be efficacious in treating the bullae, according to several reports.17-22
The use of glucocorticoids in bullous HSP in childhood remains controversial. Studies report shortening of the duration of abdominal pain, reducing risk of intussusception, decreasing recurrence risk, and reducing the risk of renal involvement with use of steroids in HSP.23-25 The use of systemic steroids has been described in children with bullous HSP to reduce the severity of HSP-related bullae and its associated painful ulcers and necrosis.16,21,25,26 The duration of steroid use ranged from a short burst to a prolonged course of weaning over weeks. Azathioprine also has been used in conjunction with methylprednisolone, prednisone, and dexamethasone.17,22 Because of its anti-IgA antioxidant antineutrophil effects, dapsone has been shown to be effective in the treatment of cutaneous HSP.27 In our patient, we used dapsone to help in weaning the patient off the prednisone. Based on our review of the literature, few cases of bullous HSP in children have reported remission without drug therapy. IgA was not found in all the reported cases in which a skin biopsy was done. As shown by the comparison of the 2 biopsies in our patient, biopsying an early lesion within 48 hours of appearance is essential to make a diagnosis because the biopsy of the older lesion could not rule out bullous pyoderma gangrenosum. Immunoreactants (IgA, C3) are destroyed within 48 hours and might lead to false-negative results on immunofluorescence in old and necrotic lesions.28,29 Most reported cases of bullous HSP showed resolution, but few resulted in scarring and/or pigmentation.10,17,18 Henoch-Schönlein purpura usually is self-limited but relapses can be seen in one-third of cases.1 One of the reported cases of bullous HSP showed recurrence of lesions.15 One of the cases showed persistent hematuria.8 Our patient also was started on lisinopril for persistent proteinuria.
