UPP - Department of Dermatology, Dermatopathology Unit
Adar Berghoff, MD, John McSorley MD, Jon Ho, MD, and Arash Radfar, MD PhD
FEBRUARY 2009 WEB CASE OF THE MONTH
A 63 year old white male with a history of allergic sinus disease, hypereosinophilia and a two year history of a pruritic eruption was referred to our dermatology clinic for evaluation. The patient related a remote history of asthma and exposure to Agent Orange in the Vietnam War. The patient previously underwent an extensive workup which included a skin biopsy revealing vasooclusive vasculopathy with a prominent eosinophilic infiltrate, which was non-specific. His workup included a bone marrow biopsy which was not diagnostic, but showed an increased presence of eosinophils. Also, he was noted to have an elevated absolute eosinophil count for approximately 18 months. He reported significant clearing of his rash when taking oral prednisone, but recurrence once the dosage was tapered. Unfortunately, the patient also had type II diabetes mellitus which was uncontrollable while on the oral steroids. He was previously treated with hydroxyurea, imatinib, and azathrioprine all of which caused hypersensitivity reactions requiring hospitalization. Two biopsies were performed at his most recent clinic visit. The patient denied any systemic symptoms at his most recent clinic visit.
Multiple, well-demarcated erythematous, plaques were present on the patient's back, bilateral flanks and bilateral posterior thighs. The lesions were non-blanchable, purpuric and palpable but non-tender. There was no visible scale and no discharge. The purpura were not in a particular pattern, including no annular or concentric lesions, and no livedoid lesions as well (see figures 1, 2).
Notably, there was an eosinophilia of 35%, with up to 5000 absolute eosinophils. Other studies including stool O&P, PCR for FIP1-L1-PDGRA translocation, SPEP, ANCA, CMP, LFTs all within normal limits or negative except for an elevated glucose; normal flow cytometry and PCR for TCR gene rearrangements.
Computed tomography of the chest, abdomen and pelvis revealed a mildly enlarged spleen but was otherwise normal. The patient also had a normal CXR.
Our patient had a very interesting history and had been evaluated by multiple specialties, including his primary care physician, a hematologist and dermatology. Unfortunately, the only diagnosis he carried at the time of his presentation with his purpuric eruption was hypereosinophilic syndrome (HES). The differential diagnosis was broad and included vasculitis, either cutaneous small vessel disease (i.e. leukocytoclastic vasculitis) or systemic vasculitis, particularly Churg-Strauss Syndrome, versus a coagulopathic disorder. A punch biopsy was performed at this time (see figures 3, 4, 5).