Skin-limited idiopathic hypereosinophilic syndrome presentin
Idiopathic hypereosinophilic syndrome (IHES) is a rare disorder defined by (1) eosinophilia (eosinophil count > 1.5 × 109/L) on 2 examinations 1 month apart, (2) organ damage and/or dysfunction attributable to tissue hypereosinophilia, and (3) lack of clear alternate cause.

A 51-year-old Taiwanese male smoker with dyslipidemia presented with 2 weeks of painful ulcers and cyanosis of the distal lower extremities associated with 7 months of pruritic papulosquamous eruptions of the trunk and extremities refractory to doxycycline, terbinafine, topical and intralesional triamcinolone, ivermectin, and low-dose prednisone. One month before presentation, he experienced hand and foot numbness and progression of rash on the lower extremities with hyperkeratosis, scale, and erythema. Home medications were atorvastatin and as-needed oxycodone and acetaminophen, with no recent changes.

Physical examination was notable for retiform purpura and edema of the bilateral lower extremities, with ulcerations on a background of livedo and cyanosis. The trunk and extremities had firm, erythematous, hyperpigmented, dome-shaped, excoriated papules and nodules. Right dorsalis pedis pulse was undetectable on Doppler studies. The patient was admitted for ambulation-limiting pain, Doppler studies suggesting complete vascular occlusion, and prominent eosinophilia.

Source: JAAD case reports

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