Evidence linking atopy and staphylococcal superantigens to the pathogenesis of lymphomatoid papulosis, a recurrent CD30+ cutaneous lymphoproliferative disorder

BackgroundLymphomatoid papulosis (LyP) is a self-healing CD30+ cutaneous lymphoproliferative disorder (CLPD) with paradoxical histology of a malignant lymphoma. Case reports and small patient series suggest an association of LyP with atopy. However, the prevalence of atopy depends on patients recall which is not always reliable. More objective criteria of atopy include skin reactivity to allergens and IgE reactivity to allergens. This study was undertaken to determine if atopy is prevalent in LyP patients using IgE specific antibodies to aeroallergens, and if Staphylococcal aureus enterotoxins might be a pathogenic factors for LyP as proposed for other skin disorders. MethodsThirty-one samples of CD30CLPD were tested for total serum IgE (IgE-t) and 10 IgE- specific airborne allergens with the Phadiatop multiallergen test, which if positive, is regarded as evidence of atopy. Sera was tested for IgE reactive against three Staphylococcal enterotoxins with superantigenic properties (SSAg-IgE). Control sera were obtained from adult subjects evaluated for rhino-sinusitis and a negative Phadiatop test. Patients history of atopy was obtained by retrospective chart review. FindingsNearly 50% of patients with the most common LyP types had a positive Phadiatop test and IgE-t was increased compared to non-atopic controls. Seven of 28 (25%) LyP patients had at least one SSAg-IgE at the concentration used to define serologic atopy ([≥] 0.35 kUa/L) compared to 3/52 (6%) controls (P= 0.028). TSST1-IgE was detected in 7 (23%) specimens of CD30CLPD, often together with SEB-IgE; SEA-IgE was not detected. TSST1-IgE exceeded the 0.35 kUa/L threshold in 3 (6%) controls. ConclusionsLyP patients have an increased prevalence of atopy as determined by the Phadiatop test and increased prevalence of SSAg-IgE compared to controls. Prevalence of serologic atopy exceeded that reported by patients medical history. The results support the hypothesis that an atopic diathesis and possibly SSAg contribute to the pathogenesis of LyP. SummaryContrary to patients recall of atopic disorders, IgE specific antibodies to aeroallergens, Staphylococcal aureus superantigens and total serum IgE are increased in patients with lymphomatoid papulosis.