SUDANESE JOURNAL OF PAEDIATRICS

2021; Vol 21, Issue No. 2

CASE REPORT

Atypical purpura location in a Sudanese infant with Henoch-Schönlein purpura

Mumen Abdalazim Dafallah (1), Alaa Abdualazem Dafalla (2), Esraa Ahmed Ragab (1), Fawzeia Hamad (2)

(1) Faculty of Medicine, University of Gezira, Wad Medani, Sudan

(2) Wad Medani Pediatric Teaching Hospital, Wad Medani, Sudan

Correspondence to:

Esraa Ahmed Ragab

Faculty of Medicine, University of Gezira, Wad Medani, Sudan

Email: Esraragab96 [at] gmail.com

Received: 02 March 2021 | Accepted: 26 April 2021

How to cite this article:

Dafallah MA, Dafalla AA, Ragab EA, Hamad F. Atypical purpura location in a Sudanese infant with Henoch-Schönlein purpura. Sudan J Paediatr. 2021;21(2):186–189.

https://doi.org/10.24911/SJP.106-1614703471

ABSTRACT

Henoch-Schönlein purpura (HSP) is a self-limited systemic vasculitis seen most commonly in paediatric group with multi organ involvements. We report a case of an infantile female who presented with cough, diarrhoea and vomiting. Two days later, she suddenly developed an erythematous, non-pruritic rash involving feet, thighs, buttocks and face; the lesion spared the trunk. Joints pain and swellings were also noticed. Coronavirus disease-19 Polymerase chain reaction (PCR) test was negative. She was diagnosed with HSP based on the American College of Rheumatology and European League Against Rheumatism, and Paediatric Rheumatology European Society criteria. The rash faded spontaneously within 2 weeks. We conclude that the careful skin examination is crucial for diagnosis of HSP.

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KEYWORDS

Henoch-Schönlein purpura; Child; Atypical location; Sudan.

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INTRODUCTION

Henoch-Schönlein purpura (HSP), also known as immunoglobulin A vasculitis, is the commonest form of childhood vasculitis [1]. The annual paediatric incidence is 8-20 per 100,000 [1]. Deposition of IgA in the small vessels wall leads to manifestations involving the skin, kidneys, joints and abdomen. Although the exact pathophysiology is not fully understood, the condition is usually following an acute upper respiratory tract infection. The diagnosis is made on the presence of palpable purpura or petechiae (without thrombocytopenia) in addition to one or more of the followings: abdominal pain, arthritis and/or arthralgia, renal involvement and positive histological findings on skin and/or renal biopsy (Table 1) [2].

The rash associated with HSP is typically distributed symmetrically over the extensor surfaces of the lower extremities and buttocks [3]. Here, we report a unique case of HSP in 11-months-old infant who presented with rash involving the face besides the classical sites.

Table 1. Criteria to diagnose HSP.

Palpable purpura or petechiae (without thrombocytopenia) [mandatory criteria] in the presence of at least one of the following four criteria:

Abdominal pain

Arthritis and/or arthralgia

Any biopsy showing predominant IgA deposition

Renal involvement (haematuria and/or proteinuria)

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CASE REPORT

An 11-month-old Sudanese female presented to Wad Medani Pediatric Teaching Hospital, Wad Medani, Sudan with cough, diarrhoea and vomiting for 3 days. She was given oral antibiotics by her primary care doctor to treat her symptoms. After 2 days, she suddenly developed an erythematous, non-pruritic rash involving feet, thighs, buttocks and face (Figure 1). The lesion spared the trunk (Figure 2).

On the following days, she developed painful knee and ankle joints swelling ‘arthritis’. There were no other associated symptoms and no relevant past medical history was apparent.

On physical examination, vital signs were as follow: respiratory rate = 40/minute; heart rate = 138/minute, blood pressure 90/65 mmHg and temperature 37.5°C. Examination of the chest was clear except for crepitations. The reminder of her physical examination was normal.

Figure 1. Purpura involving the thigh (A), the buttocks bilaterally (B); and the face, particularly the cheeks (C).

Laboratory tests were as followed: white blood cells count: 16.7 × 109/l (neutrophils: 60%, lymphocyte: 35%, and monocytes 5%), haemoglobin: 11.3 g/dl, haematocrit: 36%, mean corpuscular volume: 69 fl, platelet count: 419 × 10³/cmm. Coronavirus disease-19 PCR test was negative. Blood urea nitrogen: 14 mg/dl, serum creatinine: 0.2 mg/dl, serum sodium: 131 mmol/l and serum potassium: 4.7 mmol/l. D-dimer was 4.5 mg/l (normal value is up to 0.5 mg/l), Antistreptolysin O (ASO) titer and C-reactive protein were normal. Urine analysis showed albumin +++ and 24-hour urinary protein was 0.6 g/day (normal value is up to 0.1 g/day). Total serum protein: 3.2 g/dl, serum albumin: 1.7 g/dl and serum globulin: 1.5 g/dl. Coagulation profile and antinuclear antibody profile were within normal limits. Abdominal ultrasonography showed no abnormalities.

The patient was diagnosed with HSP based on the American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR), and Paediatric Rheumatology European Society (PReS) criteria (Table 1). Diagnosis of HSP was based on the presence of petechiae (without thrombocytopenia), associated with arthritis and proteinuria. Skin and/or renal biopsies were not done due to logistic causes.

She was treated with crystalline penicillin 1 million IU/6 hourly for 7 days and paracetamol syrup for joints pain. The joints pain and rash resolved gradually within 2 weeks and was discharged in a good condition (Figure 3).

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DISCUSSION

To the best of our knowledge, this is the first case report of HSP involving the face in a Sudanese infant. The diagnosis was made according to the ACR and EULAR, and PReS criteria (Table 1). HSP had been found to affect four organs, skin, kidneys, joints and abdomen. Cutaneous manifestations were present in all patients with HSP [4]; arthritis and/or arthralgia was found in 70%; abdominal pain, nausea and vomiting in 65% and renal involvement in 25% [5].

Figure 2. The skin leisons (petechiae) spared the trunk anteriorly (A) and posteriorly (B).

Figure 3. Disappearance of petechiae after 2 weeks.

Buttocks and the extensor surfaces of the lower extremities are the classical sites for the rash associated with HSP. It rarely affects the upper extremities and face; and usually spare the trunk [5,6]. The lesion is usually non pruritic and change in colour from red to purple and then becomes rust-coloured before disappearing [7].

Nussinovitch et al. [8] evaluated the pattern of skin involvement in 155 children diagnosed with HSP over 20 years; and found that only 4.5% of patients had face and upper limbs involvement. The lesions tend to predominate in dependent areas that are subjected to pressure such as buttocks and lower extremities, so facial involvement is rare in HSP [8].

There is no definitive test to diagnose HSP. In 2010, the American College of Rheumatology and EULAR and PReS established criteria to diagnose HSP. The criteria include palpable purpura or petechiae (without thrombocytopenia) as a mandatory criterion in addition to one of the followings: abdominal pain, arthritis and/or arthralgia in any joint, renal involvement evidence by haematuria and/or proteinuria and any skin biopsy showing predominant ImmunoGlobulin (IgA) deposition. This criterion has good diagnostic accuracy, high sensitivity (100%) and specificity (87%) [9,10].

Symptomatic and supportive cares are the main stay of treatment for HSP, including intravenous fluids for rehydration, analgesia to kill the pain and wound care if there is ulcerative skin lesion [11].

HSP is self-limited disease with an excellent outcome particularly in patients without renal involvements [7]. Most of cases fully recover in 4 weeks [7]. The long-term morbidity depends on the degree of HSP nephritis as 1% of patients will develop end stage renal disease and may require renal transplantation [12].

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CONCLUSION

We conclude that cutaneous manifestation of HSP may occur in any part of the body. Although facial involvement is rare, it should not be forgotten as it may be the only presenting symptom.

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CONFLICT OF INTEREST

The authors declare that they have no conflicts of interest.

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FUNDING

Nil.

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ETHICAL APPROVAL

Written informed consent was obtained from the parents of the patient for participation and publication of this case report and accompanying images. Ethics approval of the study was granted by Research Ethics Committee, University of Gezira Faculty of Medicine, Sudan.

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