Angiolymphoid Hyperplasia with Eosinophilia in a Patient With Coccidioidomycosis
Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare nodular mass that has not commonly been reported in the literature in association with coccidioidomycosis (CM). Coccidioidomycosis has other known skin manifestations including erythema nodosum and interstitial granulomatous dermatitis. Pulmonary CM is the most common form of the disease and the most common cause of CM-associated rash. This is an important clinical consideration for patients with ALHE who reside in CM-endemic areas, which notably include the southwestern region of the United States, Mexico, and South America. We report the case of an ALHE lesion that resolved following treatment for CM.
Practice Points
- Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare entity of unknown etiology.
- There is an association between ALHE and coccidioidomycosis (CM). Patients who present with ALHE and reside in a CM-endemic region should be examined for CM.
The function of eosinophils in ALHE is poorly understood; it is unclear whether they act as a primary driver of pathogenesis or are simply indicators of secondary infiltration or infection. Our review of the current literature suggests that eosinophils are unnecessary for progression of ALHE but might be involved at its onset. As reported, even monoclonal antibody therapy (eg, mepolizumab and benralizumab) that effectively depletes eosinophil levels by negating IL-5 signaling do not slow progression of ALHE.11 Symptomatic changes are modest at best (ie, simply softening the ALHE nodules).
Our patient had no peripheral eosinophilia, suggesting that the onset of ALHE might not be caused by eosinophilia but a different inflammatory process—in this patient, by CM. Because peripheral eosinophilia was not seen in our patient, the presence of eosinophils in the ALHE lesion likely is unnecessary for its onset or progression but is a secondary process that exacerbates the lesion. The pathogenesis is unknown but could be directed toward lymphocytes and plasma cells, with eosinophils as part of the dynamic process.11
Conclusion
Because reports of an association between CM and ALHE are limited, our case is distinguished by a unique clinical presentation of ALHE. When a patient is given a diagnosis of ALHE, it therefore is important to consider exposure to CM as a cause, especially in patients who reside in or travel to a region where CM is endemic.
