Year : 2022 | Volume
: 7 | Issue : 1 | Page : 34--36
Henoch–Schönlein purpura: Simultaneous occurrence in two siblings—Case reports
Mumtahena Mahmuda1, Nusaiba Deen Muhammad1, Nita Chowdhury1, Deva Pratim Barua2, Susmita Biswas3, Mohammed Maruf-Ul- Quader3,
1 Department of Pediatrics, Chittagong Medical College, Chattogram, Bangladesh
2 Department of Skin and VD, Chittagong Medical College, Chattogram, Bangladesh
3 Department of Pediatric Nephrology, Chittagong Medical College, Chattogram, Bangladesh
Dr. Mohammed Maruf-Ul- Quader
Department of Pediatric Nephrology, Chittagong Medical College, Chattogram
Henoch–Schönlein purpura (HSP) is one of the most common vasculitis in children. Typically, HSP is considered to be self-limiting, although renal involvement (HSP nephritis) is the principal cause of morbidity from this disease. Familial cases of this disease are not common. Only a few cases have been reported. A 7-year-old girl presented with a history of (H/O) abdominal pain, arthritis, and purpuric rash on lower extremities. Laboratory investigations revealed hematuria, proteinuria, and thrombocytosis with a normal IgA level. Renal biopsy revealed mesangial and focal endocapillary proliferative glomerular morphology with partial cellular crescent. Two weeks later, a sister of the first case presented with palpable purpura without any arthritis or nephritis. In both the cases, there was no H/O any upper respiratory tract infection or atopy. Herein, we speculated that the environmental factors may be responsible for the simultaneous occurrence of HSP in a genetically susceptible environment.
|How to cite this article:|
Mahmuda M, Muhammad ND, Chowdhury N, Barua DP, Biswas S, Quader MM. Henoch–Schönlein purpura: Simultaneous occurrence in two siblings—Case reports.Paediatr Nephrol J Bangladesh 2022;7:34-36
|How to cite this URL:|
Mahmuda M, Muhammad ND, Chowdhury N, Barua DP, Biswas S, Quader MM. Henoch–Schönlein purpura: Simultaneous occurrence in two siblings—Case reports. Paediatr Nephrol J Bangladesh [serial online] 2022 [cited 2023 Jan 29 ];7:34-36
Available from: http://www.pnjb-online.org/text.asp?2022/7/1/34/346340
Henoch–Schönlein purpura (HSP) is an acute vasculitis of children with an unknown etiology. The diagnostic criteria must include palpable purpura. In addition, either diffuse abdominal pain, acute arthritis, arthralgia, hematuria, proteinuria, or a tissue biopsy showing predominant IgA deposition should be present. HSP occurs worldwide and affects all age groups. The incidence is the highest in children aged 4–7 years. In Asia, the incidence is as high as approximately 70 cases/100,000 children per year. However, the familiar occurrence of HSP is rare. Various factors have been implicated as triggers, including Streptococcus, Campylobacter, parvovirus B19, mycoplasma, and chlamydia. Some reports suggest that certain types of human leukocyte antigen (HLA)-A2, HLA–A11, and HLA-B35 are related to the occurrence of renal involvement.
The case is presented here with a view to aware the pediatrician about the possibility of familial occurrence of HSP.
A 7-year-old girl was admitted to the Chittagong Medical College Hospital, Chattogram, Bangladesh, with 20 days’ history of (H/O) a painful left ankle joint, abdominal pain, and diffuse recurrent rash on her lower extremities followed by puffy face and scanty micturition. The patient was otherwise healthy. She was not receiving any medication and no H/O any atopy. On examination, she appeared well but hypertensive. Blood pressure was 150/90 mm of Hg with leg edema and oliguria. Bedside urine albumin was 3+; diffuse symmetrical palpable purpuric rash of varying sizes was present in extremities and buttocks. Mucous membranes were not involved. On admission, there was no sign of arthritis or limitation of movement of any joints or abdominal pain.
Laboratory evaluation showed a hemoglobin of 11.2 g/dL, white blood cell count 8870/mm3 with a normal differential count, platelet count 5,17,000/mm3, elevated erythrocyte sedimentation rate of 65 mm in first hour. Urine routine microscopic examination showed hematuria and proteinuria. Serum cholesterol was 276 mg/dL, spot urinary protein creatinine ratio was 26.21; and serum creatinine, serum electrolyte, serum albumin, bleeding time, clotting time, activated partial thromboplastin time, serum IgA levels, and complements were within the normal limit. Antinuclear antibody was negative and anti-double stranded DNA was normal. Renal biopsy showed mesangial and focal endocapillary proliferative glomerular morphology. One glomerulus showed overlying partial cellular crescent. She was treated with methylprednisolone, mycophenolate mofetil, and Ca channel blockers.[INLINE:1]
The second case was a 2-year-old sister of the first case who was admitted for rash of 10 days, which started after 2 weeks of her sister’s illness. This patient had only skin manifestation but no arthritis, abdominal pain, and renal involvement. Skin biopsy showed signs of leukocytoclastic vasculitis. Serum immunoglobulin A was 1.03 g/L (normal range 0.7–4.0 g/L). Serum creatinine, urine microscopy, level of complements, and anti-nuclear antibody were normal. She was treated with prednisolone for the recurrence of rash.
There are only a few reports of the familial occurrence of HSP.,, Although familial occurrence has been described, the simultaneous onset of HSP has not been well documented. There are two previous reports of the simultaneous onset of HSP in family members. Levy-Khademi et al. described the simultaneous onset of HSP in two sisters, 1 day after wearing of a new synthetic slippers. De Veber described the first family with three members having had HSP. Two of them were siblings who developed HSP at about the same time. These cases were preceded by streptococcal pharyngitis. Motoyama and Iitaka showed 67% HSP patients with preceding H/O upper respiratory tract infection and 45% patients with nephritis. Antistreptolysin O titer is elevated in most of the cases. Influenza A antigen was detected in the throat of some patients.
Chen et al. reported three families with HSP. One family had an H/O upper respiratory tract infection and other two families had shown renal involvement. In one of the patients, renal biopsy revealed crescentic glomerulonephritis. Two siblings were reported by Grech Victor who developed HSP nephritis associated with infectious mononucleosis. They had shown the features of HSP with lymphadenopathy, hepatosplenomegaly, and other features suggestive of Epstein–Barr virus infection. Endothelial basement membrane IgM antibodies were positive in both cases. Our patients had neither an H/O infection prior to the onset of HSP nor clinical evidence of infection on admission, and ASO titer was normal in both of them.
Motoyama and Iitaka, and Zhang et al. suggested that the interval of onset within familial cases is the most important character to be considered, because the clustering of one disease in families might be due to either genetic background or environmental factors.,
If the interval is really short (several days to weeks), the environmental factors such as infectious agents, allergic causes, or drugs may play important roles in the pathogenesis of familial HSP. However, if the onset interval is longer (months to years), a genetic immune factor may play a role in the mechanism of the familial occurrences of HSP. In this study, the interval of the two sisters getting infected was 2 weeks. Zhang et al. reported two families of HSP. One out of the two families presented with nephritis and renal biopsy revealed crescentic deposit in less than 50% glomeruli. In this study, one of the sisters had HSP nephritis and biopsy showed glomerular and focal endocapillary proliferation with crescentic deposit in one glomerulus.
Recent studies have indicated that individuals with HLA-A2, A11, B35 are more susceptible to HSP. Yu-Hung Chenet al. have reported 6 cases of familial HSP in 3 Taroko families whose human leukocyte typing did not correlate well with the susceptible human leukocyte antigen types. This could suggest that there are more human leukocyte antigen types susceptible to HSP. This could suggest that there are more HLA types susceptible to HSP.
Nearly simultaneous onset of symptoms in our cases suggests that a common environmental factor either infectious agent or allergy is possible. Although the trigger causing HSP was not identified in these cases, we speculated the environmental factor may be responsible for the simultaneous occurrence of HSP in genetically susceptible patients.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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