|Year : 2013 | Volume
| Issue : 3 | Page : 88-91
Hemorrhagic bullous lesions in a 4-year-old girl with Henoch-Schöenlein purpura
Mojgan Safari, Arash Dehghan
Department of Pediatrics, Hamadan University of Medical Sciences, Hamadan, Iran
|Date of Web Publication||26-Nov-2013|
N 15, Day Clinic, Boali Street, Hamadan
Source of Support: None, Conflict of Interest: None
Henoch-Schonlein purpura (HSP) is an Immunoglobulin A mediated vasculitis and mainly affects skin, joints, gastrointestinal tract and kidneys. Skin lesions usually present as erythematous maculopapular, petechiae and purpura and often involve lower extremities and buttocks. Dermatological complications are rarely seen in HSP. Herein, we described a 4-year-old girl with typical skin lesions of HSP and hemorrhagic bullous lesions in her thighs.
Keywords: Bullous, child, Henoch-Schonlein purpura
|How to cite this article:|
Safari M, Dehghan A. Hemorrhagic bullous lesions in a 4-year-old girl with Henoch-Schöenlein purpura. Indian J Paediatr Dermatol 2013;14:88-91
|How to cite this URL:|
Safari M, Dehghan A. Hemorrhagic bullous lesions in a 4-year-old girl with Henoch-Schöenlein purpura. Indian J Paediatr Dermatol [serial online] 2013 [cited 2020 Apr 10];14:88-91. Available from: http://www.ijpd.in/text.asp?2013/14/3/88/122176
| Introduction|| |
Henoch-Schonlein purpura (HSP) is an Immunoglobulin A (IgA) mediated vasculitis and mainly affects skin, joints, gastrointestinal tract and kidneys. The main manifestations of this small vessel vasculitis are palpable purpura, arthritis, abdominal pain and renal disease.  In some cases raised IgA and erythrocyte sedimentation rate (ESR) are helpful to diagnosis. Skin lesions usually present as erythematous maculopapules, petechiae and purpura and often involve lower extremities and buttocks. Herein, we described a 4-year-old girl with typical skin lesions of HSP in her legs and hemorrhagic bullous lesions in the inner side of both thighs.
| Case Report|| |
A 4-year-old girl was referred to Besat Hospital affiliated to Hamadan University of Medical Sciences because of arthralgia, abdominal pain and hemorrhagic bollous lesions in inner sides of both thighs especially in the right side. 10 days before admission, her mother noticed mild fever and multiple eruptions that were concentrated on the buttocks and upper and lower extremities. Four days later, the patient complained of arthralgia in the left knee and ankles, abdominal pain and a single episode of vomiting. Bollous lesions appeared in inner sides of both thighs at this time. The color of urine was normal. Past medical history was unremarkable. Physical examination on admission revealed a body temperature of 37.3°C, pulse rate of 90 bpm., respiratory rate of 24/min and blood pressure of 100/60 mmHg. There were multiple purpuric lesions in lower extremities, varying in size from 2 mm to 7 mm in diameter [Figure 1]. The bullous hemorrhagic lesions were seen in inner sides of both thighs [Figure 2] and [Figure 3] especially in right [Figure 3]. The right knee was swollen and painful. The examination of the abdomen revealed no abnormality. The remainder of physical examination was normal.
Laboratory examinations showed hemoglobin 11.7 g/dL, white blood cell count 11,400/μL with normal differentiation, platelet count 351,000/μL. ESR was 12 mm/h.
Coagulation tests, blood urea nitrogen, creatinine and electrolytes were normal.
Urinalysis revealed no abnormality. Stool analysis was normal and without occult blood. Although the first diagnosis was HSP, but the presence of bullous lesions in atypical site, put a question mark front of the diagnosis.
A biopsy specimen of skin showed small blood vessels of both papillary and reticular dermis showing neutrophils infiltration throughout venule walls and perivascular connective tissue and darkly staining nuclear dust (leukocytoclasis) [Figure 4] and [Figure 5], which helped to confirm the diagnosis of HSP.
The signs and symptoms resolved with supportive therapy and steroids didn't administrate during the treatment course. The bullous lesions began to resolve within a few days. There have been no complications of HSP during 6 months of follow-up.
|Figure 4: Leukocytoclastic vasculitis: Perivascular infiltrate of neutrophils, neutrophilic nuclear dusts in postcapillary venules H and E, 40 × 10|
Click here to view
|Figure 5: Leukocytoclastic vasculitis: Perivascular infiltrate of neutrophils, neutrophilic nuclear dusts in postcapillary venules H and E, 25 × 10|
Click here to view
| Discussion|| |
HSP is the most common systemic vasculitis of childhood with reported incidence of 10-20 cases/100,000 children/year.  HSP is characterized by cutaneous purpura, arthritis, abdominal pain and nephritis.  Dermatological complications such as bullous lesions are rarely seen. These can spread over the whole body and can result in ulcers, necrosis and scars.  The pressure is likely a factor into the pathogenesis of bullae. 
Bullous hemorrhagic lesions are more common in adults with HSP. , The incidence is 16% in adults and 60% in the studies by Cream et al.  and Tancrede-Bohin et al.  respectively. Bullous lesions have rarely seen in children with HSP, Abdel-A1 et al.  reported bullous lesions in 2% of children with HSP. The presence of bullous lesions produces confusion in diagnosis of HSP.
In the majority of reported cases, the bullous lesions have developed in the sites of typical skin involvement of HSP. Our presenting case had typical rash of HSP, but bullous lesions had been appeared in inner sides of both thighs, especially right side. So far as we know, it was the first case with such distributions of lesions.
Leung et al. reported an 8 years boy with HSP who developed hemorrhagic bullae.  He suggested that pressure might be a factor in the pathogenesis; because in his study, distributions of bullous lesions were similar to typical rash of HSP and typically, the rash was located in a pressure-dependent distribution. In case, we report the lesions were in the inner side of thigh, which that aren't pressure-dependent site. It seems that the rubbing of the skin may be a factor in developing of bullous lesions. Park and Lee reported a 47 months girl with multiple hemorrhagic bullous and purpuric lesions on both feet and lower legs. 
den Boer et al. reported a 6 years boy with multiple bullous lesions on arms and legs and a 10 years boy with hemorrhagic bullous lesions on the legs.  Leung and Robson reported 8 years Chinese boy with multiple palpable purpuric lesions on the buttocks and lower limbs.  Chan et al. reported 13 years Chinese boy with an acute bullous eruption on the lower limbs.  Abdulla et al. reported a 16-year-old white girl with centrally necrotic, hemorrhagic bullae on the lower extremities, buttocks, lower abdomen and inner arms.  Ishii et al. reported a 4 years boy with many vesicles and hemorrhagic bullae, varying in size from 5 to 20 mm in diameter, on his face, penis, buttocks, dorsum of the hands and both leg.  Raymond and Spinks reported a 9 years girl with hemorrhagic bullae began to form on her feet and ankles. 
Our patient had a usual course of disease without renal involvement. It seems that the presence of bullous lesions in HSP does not affect the course of the disease in children.  Bullous lesions in adults with HSP are associated with more severe course of illness. 
| Conclusion|| |
Bullous hemorrhagic lesions in HSP are seen in children, rarely. They do not effect on the course of the disease. The presence of bullae may lead to diagnostic confusion.
| Acknowledgments|| |
The authors are grateful to Mr. Mahdi Sufian and Ms. Zabihian for help in the preparation of the manuscript.
| References|| |
|1.||Robson WL, Leung AK. Henoch-Schönlein purpura. Adv Pediatr 1994;41:163-94. |
|2.||Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 2002;360:1197-202. |
|3.||Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997;337:1512-23. |
|4.||den Boer SL, Pasmans SG, Wulffraat NM, Ramakers-Van Woerden NL, Bousema MT. Bullous lesions in Henoch Schönlein Purpura as indication to start systemic prednisone. Acta Paediatr 2010;99:781-3. |
|5.||Trapani S, Mariotti P, Resti M, Nappini L, de Martino M, Falcini F. Severe hemorrhagic bullous lesions in Henoch Schonlein purpura: Three pediatric cases and review of the literature. Rheumatol Int 2010;30:1355-9. |
|6.||Cream JJ, Gumpel JM, Peachey RD. Schönlein-Henoch purpura in the adult. A study of 77 adults with anaphylactoid or Schönlein-Henoch purpura. Q J Med 1970;39:461-84. |
|7.||Tancrede-Bohin E, Ochonisky S, Vignon-Pennamen MD, Flageul B, Morel P, Rybojad M. Schönlein-Henoch purpura in adult patients. Predictive factors for IgA glomerulonephritis in a retrospective study of 57 cases. Arch Dermatol 1997;133:438-42. |
|8.||Abdel-Al YK, Hejazi Z, Majeed HA. Henoch Schönlein purpura in Arab children. Analysis of 52 cases. Trop Geogr Med 1990;42:52-7. |
|9.||Garland JS, Chusid MJ. Henoch-Schoenlein purpura: Association with unusual vesicular lesions. Wis Med J 1985;84:21-3. |
|10.||Park SE, Lee JH. Haemorrhagic bullous lesions in a 3-year-old girl with Henoch-Schölein purpura. Acta Paediatr 2011;100:e283-4. |
|11.||Leung AK, Robson WL. Hemorrhagic bullous lesions in a child with Henoch-Schönlein purpura. Pediatr Dermatol 2006;23:139-41. |
|12.||Chan KH, Tang WY, Lo KK. Bullous lesions in Henoch-Schönlein purpura. Pediatr Dermatol 2007;24:325-6. |
|13.||Abdulla F, Sheth AP, Lucky AW. Hemorrhagic, bullous Henoch Schonlein purpura in a 16-year-old girl with previously undiagnosed dominant dystrophic epidermolysis bullosa. Pediatr Dermatol 2010;27:203-4. |
|14.||Ishii Y, Takizawa T, Arakawa H, Saga R, Mochizuki H, Tokuyama K, et al. Hemorrhagic bullous lesions in Henoch-Schönlein purpura. Pediatr Int 2005;47:694-7. |
|15.||Raymond M, Spinks J. Bullous Henoch Schonlein purpura. Arch Dis Child 2012;97:617. |
|16.||Saulsbury FT. Hemorrhagic bullous lesions in Henoch-Schönlein purpura. Pediatr Dermatol 1998;15:357-9. |
|17.||Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, García-Fuentes M, González-Gay MA. Henoch-Schönlein purpura in adulthood and childhood: Two different expressions of the same syndrome. Arthritis Rheum 1997;40:859-64. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]