Eosinophilia and multiple erythematous indurated plaques. Idiopathic hypereosinophilic syndrome (IHS)

The eosinophilias, including the idiopathic hypereosinophilic syndrome

“…The cause of eosinophilic lung disease can be distinguished by thin‐section CT scans in 61% of patients (Johkoh et al, 2000). The dermatological manifestations are protean, and include angioedema (van den Hoogenband, 1982), urticaria (Nir & Westfried, 1981), papulonodular lesions, multiple erythematous indurated plaques (De Yampert & Beck, 1997) and recurrent incapacitating mucosal ulceration (Leiferman et al , 1982). Urticaria per se and vesicobullous lesions have also been reported (Brugger et al , 1980; Fitzpatrick et al , 1987) as has one patient with solar urticaria (Aragone et al , 1999).…”

Interferon Treatment for Hypereosinophilic Syndromes and Systemic Mastocytosis

“…IFN-α therapy has proven effective in the treatment of these lesions [19, 23, 63], although in 1 reported case [23] there was temporary worsening of lesions. In other reports, IFN-α has controlled other skin lesions (pruritic papules, nodules, plaques) associated with HES [16, 64] and has been effective in the treatment of eosinophilic pustular folliculitis (Ofuji’s disease) [65]. …”

Association of the blood eosinophil count with end-organ symptoms

“…So far, no association between levels of blood eosinophils and organ manifestations has been demonstrated. However, it seems as if eosinophils show a predilection for certain organ systems such as heart [[11], [12], [13], [14], [15], [16], [17]], lungs [[18], [19], [20], [21], [22], [23]], gastrointestinal system [[24], [25], [26], [27]], nervous system [[28], [29], [30], [31], [32], [33], [34], [35]] and skin [[36], [37], [38], [39], [40]]. A scoring system guiding therapy based on certain paraclinical determinations was introduced some thirty years ago for patients with idiopathic hypereosinophilic syndrome [41,42], but this has not been implemented in clinical work, and today the degree of blood eosinophilia as mild (≥0.5 × 10 9 /l-1.5 × 10 9 /l), moderate (≥1.5 × 10 9 /l-5.0 × 10 9 /l) and severe (≥5.0 × 10 9 /l) is arbitrary and not based on risk stratification for organ manifestations [1,3].…”