Purpuric lip lesions in a young boy with IgA vasculitis
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CLINICAL IMAGE
Purpuric lip lesions in a young boy with IgA vasculitis Aman Gupta 1
&
Megha Garg 2
Received: 2 September 2020 / Revised: 24 October 2020 / Accepted: 27 October 2020 # International League of Associations for Rheumatology (ILAR) 2020
Presentation
Discussion
A previously healthy 6-year-old boy presented to the pediatrics department with skin rash, pain in the right ankle joint, and abdominal pain. Examination showed an afebrile child with diffuse palpable purpura over lower limbs (Fig. 1a), buttocks, trunk, upper limbs, palms, and soles with swelling and restricted movements of right ankle joint. Purpuric non-blanchable lesions were also noted over the lips (Fig. 1b). No pallor or lymphadenopathy was noted. Systemic and oral examination was unremarkable. Investigations showed normal hemoglobin (13.5 g/ dL), platelet count (402 X 109/L), urine examination and renal functions (Urea: 7.5 mg/dl, creatinine 0.43 mg/dl). Antinuclear antibody (ANA) by indirect immunofluorescence was negative. Skin biopsy was not performed. A diagnosis of IgA vasculitis was proffered. He received intravenous dexamethasone for painful abdomen followed by oral prednisolone for 7 days which was gradually tapered over 2 weeks. Purpuric lesions over lips resolved parallel with the generalized palpable purpura during follow-up visit after 2 weeks.
IgA vasculitis (Henoch-Schonlein Purpura) is a small vessel vasculitis and one of the most common vasculitides of childhood [1]. Cutaneous manifestations in IgA vasculitis include palpable purpuric rashes predominantly involving the lower extremities and buttocks, but have also been described over other areas [2]. Lesions may vary from petechiae, ecchymoses to hemorrhagic bullae resulting in necrosis of the skin [3]. Mucosal lesions may include swelling, hyperaemia, petechiae, purpuric lesions, erosions, ulcers and hemorrhages involving the esophagus, stomach and intestines [4, 5]. However, mucosal involvement with purpura over lips has rarely been reported in children with IgA vasculitis. These lesions should be differentiated from idiopathic thrombocytopenic purpura (unlikely in view of normal platelet count), systemic lupus erythematosus and other vasculitis syndromes (unlikely in view of negative ANA with typical clinical manifestations of IgA vasculitis) [1]. Skin lesions of IgA vasculitis may resemble cutaneous manifestations associated with certain periodic fever syndromes, including familial Mediterranean fever and periodic fever with aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome [6, 7]. Further reports may be needed to elucidate the correlation between purpuric lesions at unusual sites with disease severity and renal involvement.
* Aman Gupta [email protected] 1
Department of Pediatric Rheumatology and Immunology, MEDENS Hospital, Panchkula, Haryana 134113, India
2
Department of Dermatology & Cosmetology, MEDENS Hospital, Panchkula, Haryana 134113, India
Clin Rheumatol Fig. 1 a Non-tender, non-pruritic purpuric lesions over bilateral lower extr
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