Nancy B Murray, MD; Francis A. Barb...

Nancy B Murray, MD; Francis A. Barber, MD; and Kevin M Murray, MD FCCP

A 49-year-old man with a 12-month history of rhinitis and asthma not awayed with complaints of cough, progressive shortness of breath, and weight los of individual month's duration. Ten days prior to admission he had also disentangleed a purpuric eruption and migratory arthralgias.

Physical examination revealed a pale, dyspneic male. Auscultation of the lung revealed decreased breath undecayeds bilaterally and diffuse expiratory wheezing. The extremities were remarkable for purpuric papules and erythematous nodules of the palms, fingertips, and toes (Fig 1 and 2) Examination of the nails revealed small subungual hemorrhages. Petechiae were noted forward the palate and buccal mucosa. There was edema and tendernes through the whole extent of the dorsum of the right wrist with painful unless full range of motion. Neurologic examination was remarkable for decreased sensation from one side of to the other the dorsum of the left hand and the lateral aspect of the left foot

Admission chest x-ray films showed bilateral patchy interstitial infiltrates (Fig 3) kin gas analysis showed a pH of 74 a [Posub2] of 615 mm Hg and a P[cosub2] of 38 mm Hg The WBC estimate was 32,000/cu mm with 52 percent eosinophils. Histopathologic examination of skin biopsy specimens revealed papillary dermal edema with a diffuse neutrophilic infiltrate admixed with eosinophils (Fig 4) Many small sailing crafts were destroyed, with fibrin and nuclear fragments seen within the tube walls. Histiocytes surrounding foci of degenerating collagen hinted palisading granuloma formation. Special stains and tissue refinements were negative.

What is the diagnosis?

a) Chronic eosinophilic pneumonia b) Wegener's granulomatosis c) Polyarteritis nodosa d) Allergic bronchopulmonary aspergillosis e) Churg-Strauss syndrome f) Disseminated coccidioidomycosis Answer: e) Churg-Strauss syndrome

Churg-Strauss syndrome (CSS) or allergic granulomatosis, is a disease characterized from adult-onset asthma with peripheral eosinophilia and necrotizing eosinophilic vasculitis in at least brace extrapulmonary sites.[1] Allergic rhinitis is many times the first symptom of this disease, as it was in revealed patient. Rhinitis is characteristically accurate and may progress to nasal obstruction and pansinusitis. Asthma also befalls early and may worsen or improve prior to the attack of vasculitic.[2] After an interval of month to years, the vasculitic phase of CS begins and may involve cardiovascular, GI, nervous, and musculoskeletal systems[2] Renal disease is usually mild.[3]

The vasculitic phase of CS chiefly commonly involves the lung.[3,4] Pulmonary infiltrates are usually patchy and diffuse, repeatedly with a peripheral distribution. Hilar adenopathy may be seen the two military and nodular infiltrates appear but only a single case of cavitation has been reported.[5]

The skin is the in the greatest degree common site of extrapulmonary involvement during the vasculitic phase of CS with cutaneous lesions occurring in 70 percent of cases.[1-3] The skin lesions are of several types[6] Palpable purpura is in the greatest degree common, indistinguishable from that associated with other necrotizing vasculitides. delicate subcutaneous nodules are characteristic. Purpuric and nodular lesions may become necrotic and ulcerate, and may be interposed with urticarial plaques. Erythematous maculopapular eruptions can mimic erythema multiforme. All lesions serve to occur in crops and are usually seen forward the extremities and scalp; the body is less commonly involved.

The histopathologic findings in the skin, as discussed by means of Lanham and associates,[2] include a necrotizing vasculitis, primarily of small sailing crafts with an associated eosinophilic infiltrate. Extravascular palisading granulomas may or may not be present

Chronic eosinophilic pneumonia may mimic CS Patients generally are not atopic, although they may perform the operations indicated in asthma in association with weight los febrile disease and characteristic peripheral pulmonary infiltrates.[7] Eosinophilia is variable, and although a mild vasculitis may be seen in the lung cutaneous vasculitis is not characteristic.

Wegener's granulomatosis may near with necrotizing vasculitis, but is not associated with asthma.[8] Histologic examination of purpuric nodules reveals a necrotizing vasculitis with perivascular granuloma formation if it be not that without an eosinophilic infiltrate.

Polyarteritis nodosa (PAN) is characterized according to a vasculitis which involves small- to medium-sized arteries rather than the small arterioles of the dermis involved in CSS[9] Skin manifestations of PAN include penetrating subcutaneous nodules with or without ulceration and rarely ecchymosis.[10]

Allergic bronchopulmonary aspergillosis (ABPA) is also characterized at asthma and peripheral eosinophilia.[11] Transient pulmonary infiltrates are similar to those seen in CS Systemic vasculitis, however, is not seen in ABPA.

Disseminated coccidioidomycosis may near with pulmonary infiltrates and peripheral eosinophilia.[12] The skin manifestations of this condition include pustules, verrucous nodules, and plaques, as well as cutaneous and subcutaneous abcesses with cellulitis and sinus tract formation. Vasculitis is not a feature of disseminated coccidiodomycosis.