INTRODUCTION

Annular erythema is a group of chronic, annular and erythematous skin rashes that can occur at any time of life, from infancy to old age. It most commonly affects the thighs and legs, but can also occur on the face, trunk, and arms. The lesions rarely cause mild itching or stinging^[1]. Sometimes it is associated with underlying diseases and conditions such as drug use, bacterial and viral infections, Graves’ disease, cholestatic liver disease and food. But often no specific cause of annular erythema is found^[2]. It can be confirmed by histopathology, which shows a perivascular lymphocytic infiltration, and rarely, annular leukocytoclastic vasculitis (ALV)^[3]. Here, we report a case of ALV as the first clinical feature of immunoglobulin A vasculitis (IgAV).

CASE DESCRIPTION

A 36-year-old woman with no personal medical history presented with a macular erythematous rash with raised and infiltrated edges, confluent in places forming a large annular dermatitis, non-itchy, non-painful and non-oozing, located on the forearms (Fig. 1A). She also had a circular lesion in concentric rings (target lesions) located on the fingers (Fig. 1B). The patient described the onset of the eruption as a small, raised pink-red spot that slowly enlarged and formed a ring shape, with the central area flattening and clearing. The rings enlarged progressively to reach a diameter of approximately 6 cm on the forearms and 3 cm on the fingers.

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Figure 1. Skin lesions: A) annular lesions, B) target lesions and C) petechial purpura.

Moreover, she developed petechial and necrotic purpura of the lower limbs (Fig. 1C). She complained of arthralgia of the knees and ankles with long-lasting fever of one week. There were no additional mucosal lesions. She had no abdominal pain, blood in the stool, recent drug use or toxic exposure. Physical examination revealed a high temperature of 38 °C, heart rate of 80 beats per minute, blood pressure of 130/80 mmHg, a normal respiratory rate of 16/min and normal cardiorespiratory auscultation. Routine laboratory tests showed leukocytes at 10.56 g/l (normal range 4–10 g/l) with neutrophils at 6.76 g/l (normal range 1.5–7 g/l), haemoglobin at 12.2 g/l (normal range 11.5–15 g/l) and platelets at 312 g/l (normal range 150–450 g/l). The C-reactive protein level was 62 mg/l (normal range: <3 mg/l). Liver tests, serum creatinine and electrolytes were within the normal ranges. The 24-hour proteinuria test was negative. Serum electrophoresis and immunoglobulin (IgG, IgA and IgM) levels were normal. Hepatitis B virus, hepatitis C virus and HIV serologies were negative. Antinuclear antibodies, antineutrophil cytoplasmic antibodies, cryoglobulinemia, rheumatoid factors, and C3/C4 levels were normal. A computed tomography scan and cardiac echogram were normal. A skin biopsy showed perivascular infiltration of neutrophils, nuclear dust, extravasated red blood cells with endothelial swelling, and fibrinoid necrosis of the vessel wall. Direct immunofluorescence testing showed perivascular deposition of IgA (Fig. 2). The diagnostic of ALV related to IgAV was made. Treatment consisted of topical corticosteroids and bed rest, with a favourable outcome. The skin lesions cleared rapidly, and joint tenderness, fever and biological abnormalities normalised within one week. Topical corticosteroids were continued for one month. At 12 months follow-up, the patient was asymptomatic and considered to be in complete remission.

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Figure 2. Leukocytoclastic vasculitis on the forearms: A) Haematoxylin and eosin magnification ×20; B) Haematoxylin and eosin magnification ×64.

DISCUSSION

To the best of our knowledge, this is the first observation of ALV related to IgAV. As mentioned by Meissner et al.^[4], ALV was first described by Degos and Guiliane. It is histologically defined by neutrophilic invasion and fibrinoid necrosis around small dermal blood vessels.

Cribier et al.^[5] describe ALV as a distinct subtype that fulfils the following criteria: (1) multiple attacks over years with sudden onset and spontaneous resolution after 7–10 days; (2) annular purpuric patches with centrifugal extension; (3) extension to the limbs and trunk producing polycyclic lesions that clear, leaving mild haemosiderin deposition; (4) no extracutaneous symptoms and good general health; (5) histological changes of leukocytoclastic vasculitis with mild vascular changes and intense polymorphonuclear cell infiltration, and (6) complete clearance of all lesions with dapsone.

We performed a literature review of reported ALV. Table 1 summarises the epidemiological, clinical presentation, aetiological spectrum, and management of ALV. In our case, the patient improved rapidly with topical steroids without relapse. In previous cases, dapsone seems to have been effective as well^[4,6]. Typically, patients with IgAV present with palpable skin purpura predominantly on the lower limbs. It can extend to the upper extremities and on the trunk. Some patients showed necrotic lesions, ulcerations or bullae.

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Table 1. Etiological specter of ALV: Literature review of annular leukocytoclastic vasculitis since 2000.

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