A Case of Granulomatous Slack Skin with Hypereosinophilia

Abstract

We reported a 53-year-old woman with a 28-year history of many atrophic, skin-covered, pendulous, bulky tumors located on her face, chest, bilateral axillae, arms, groin, and upper limbs. She has leukocytosis (59,200/μl), peripheral eosinophilia (90%), and a high titer of IgE (215,523 U/ml). The biopsy specimens showed a massive infiltration of low grade malignant lymphocytes and histiocytes with multinucleated giant cells in the dermis and into the subcutaneous tissue. Most of the infiltrating lymphoid cells were CD3⁺, CD4⁺. A monoclonal rearrangement of the TCR-β gene was detected in the skin lesion by Southern blot analysis. The diagnosis of granulomatous slack skin (GSS) with hypereosinophilia was established. Prednisolone (5 mg/day) was effective for lesional control. Forty-eight cases of GSS have been reported to date, and hypereosinophilia has been demonstrated in 4 patients, including our case, who showed the most hypereosinophilia. The association of GSS with preexistng lymphoproliferative disorders such as MF and GMF was about 71% (5 of 7 cases), and that with subsequent lymphoproliferative disorders such as HD and NHD was about 80% (11 of 14 cases).