Conflict of interest: none declared.
CPD • Clinicopathological case
Annular erythematous plaques in a patient with asthma
Article first published online: 21 MAY 2012
© The Author(s). CED © 2012 British Association of Dermatologists
Clinical and Experimental Dermatology
Volume 38, Issue 1, pages 102–104, January 2013
How to Cite
deLeon, D., Paniz Mondolfi, A. E., Stadecker, M. J. and Lizzul, P. (2013), Annular erythematous plaques in a patient with asthma. Clinical and Experimental Dermatology, 38: 102–104. doi: 10.1111/j.1365-2230.2012.04378.x
- Issue published online: 18 DEC 2012
- Article first published online: 21 MAY 2012
- Accepted for publication 5 January 2012
A 17-year-old woman presented with annular urticarial plaques concomitant with exacerbation of her pre-existing asthma. In addition to the asthma, she had a history of nasal polyposis and recurrent sinusitis. Six months before her presentation, the patient had undergone endoscopic sinus surgery, septoplasty and adenoidectomy, which had significantly improved her nasal patency and airflow. At the same time, she had developed scattered, mildly pruritic, erythematous annular urticarial plaques on the legs and trunk. These waxed and waned over time, each lasting from a few days to a week, and their appearance correlated with her asthma flares. The patient’s asthma treatment comprised montelukast, inhaled albuterol–ipratropium, fluticasone nasal spray and oral cetirizine. Her primary care physician had prescribed topical corticosteroids and antifungal cream for the eruption. Neither of these was helpful, but some improvement was seen with oral prednisone.
On physical examination, numerous annular, erythematous indurated plaques, many with accentuated raised borders, were seen distributed over both thighs, both upper arms and the trunk (Fig. 1). Some erythematous macules coalescing into plaques with no scaling were also noted, and there was mild pruritus at the sites of eruption. No lymphadenopathies were present, and the reminder of the physical examination was unremarkable.
Laboratory investigations identified peripheral blood eosinophilia (31%, 4.7 K/μL; normal range 0.0–0.3 K/μL) and increased serum IgE (1579.6 IU/mL; 1–170 IU/mL). Urinalysis, erythrocyte sedimentation rate, and levels of C-reactive protein, C3, C4, antinuclear antibody, anti-double-stranded DNA, rheumatoid factor, and cytoplasmic and perinuclear antineutrophil cytoplasmic antibodies (c-ANCA, p-ANCA) were within normal limits. Tests for Lyme IgM and IgG antibodies were negative.
On histological examination of a biopsy specimen taken from the right thigh, pandermal palisading granulomas were seen, with perivascular and interstitial lymphohistiocytic and eosinophilic infiltrates (Fig. 2a), and focal, subtle leucocytoclastic vasculitis (Fig. 2b, inset). The centre of the granulomas displayed fibrinoid collagen degeneration admixed with degranulating eosinophils forming flame figures, and surrounded by lymphocytes, histiocytes and giant cells (Fig. 2b). Eosinophilic abscesses were also evident.
What is your diagnosis?
Churg–Strauss syndrome (CSS).
CSS, also known as allergic granulomatosis, is a vasculitis of small- and medium-sized vessels, occurring largely in patients with asthma and eosinophilia. It is a rare disease, with an incidence of 0.5–6.8 per million of the population per year, and a prevalence of 10.7–13 per million. CSS mainly appears in middle age, with a mean age of 48 years at diagnosis, and no particular gender predilection.1
The American College of Rheumatology (ACR) requires four or more of the following six criteria to diagnose CSS: asthma, blood eosinophilia > 10%, paranasal sinus abnormalities, pulmonary infiltrates, mononeuropathy or polyneuropathy, and extravascular eosinophils on biopsy. Presence of ≥ 4 criteria yields a sensitivity of 85% and specificity of 99.7% for the diagnosis of CSS.2 ANCA are present in nearly 40% of patients with CSS, with p-ANCA more common than c-ANCA. Antibody levels may vary according to disease severity and activity.3 Despite the lack of ANCA positivity, the clinical presentation of this patient fulfilled both the ACR and Chapel Hill criteria for the diagnosis of CSS.
The three clinical phases described in CSS comprise: (i) onset of atopic diatheses, such as asthma, allergic rhinitis, and sinusitis; (ii) onset and relapses of eosinophilia affecting one or more organs (virtually always the lungs); and (iii) systemic vasculitis. This final phase is the most life-threatening, as it can result in inflammation of vessels supplying vital organs, including the gastrointestinal tract, kidneys, heart and peripheral nervous system.4
Cutaneous involvement occurs in 40–81% of patients with CSS, and is the presenting sign in 14% of these patients. Among the proportion of patients with CSS who have cutaneous lesions, the commonest features are palpable purpura (> 50%) followed by urticarial lesions (12–31%), which usually involve the limbs, followed by the trunk and other parts of the body. Bullous, livedo reticularis-like, and annular lesions (as seen in this case) are extremely rare.5 Interestingly, our patient recalled that her cutaneous lesions worsened while on treatment with leucotriene antagonists (LTRAs) for her asthma exacerbations. Although controversial, a link has been suggested between LRTAs and the triggering of CSS symptoms.1
The histopathological features of cutaneous CSS often include eosinophil-rich vasculitis, dermal eosinophilia with flame figure formation, and ‘red (eosinophil-rich) granulomas’ with a core of collagen degeneration as seen in the present case.4 These features, however, are not pathonogmonic of the disease, and may overlap histologically with other clinical conditions such as Wegener granulomatosis, Wells syndrome (eosinophilic cellulitis), hypersensitivity reactions, and other autoimmune and inflammatory conditions.5
The cornerstone of treatment for CSS with active organ involvement includes high-dose systemic corticosteroids and the addition of immunosuppressive therapy, such as cyclophosphamide, azathioprine, mycophenolate mofetil, methotrexate, ciclosporin or rituximab. One recent case reported complete resolution of cutaneous lesions specifically after treatment with dapsone and low-dose corticosteroids.6 We treated our patient with low-dose oral corticosteroids (prednisone), and complete clearing of skin lesions and adequate control of her respiratory symptoms was achieved after 5 months.
Although cutaneous involvement is not required for diagnosis, biopsy of involved skin is often helpful in confirming cases of CSS. Dermatologists must consider CSS in the differential diagnosis of skin lesions in patients with asthma and eosinophilia, as cutaneous features such as palpable purpura and urticarial lesions may be the presenting sign.
- •CSS is a systemic vasculitic syndrome commonly associated with a history of asthma.
- •Cutaneous involvement is common and may be the presenting sign in some patients. Lesions are regularly purpuric or urticarial.
- •Patients usually present with peripheral blood eosinophilia and may test positive for c-ANCA.
- •The histological pattern comprises palisading granulomas with central eosinophilic abscesses and collagen degeneration, and leucocytoclastic vasculitis.
- •Neither the clinical nor histological features are pathognomonic for this disease.