Charli Joseph et al compared five

Charli-Joseph et al compared five cases of metastatic fibrous histiocytoma with five cases of cellular fibrous histiocytoma and seven cases of atypical fibrous histiocytoma using array-based comparative genomic hybridization analysis. A higher frequency of chromosomal aberrations in metastatic fibrous histiocytoma was found. In addition, more frequent gains of chromosome 7 and chromosome 8q, and loss of chromosome Xq were found in metastatic fibrous histiocytomas. They suggested conducting array-based comparative genomic hybridization analysis for the fibrous histiocytoma once there was clinical suspicion for metastatic potential. There is currently no consensus on the treatment of metastatic fibrous histiocytoma. Some reported successful treatment with resection of the metastatic lesions. Others reported using chemotherapy for multiple metastases, including ifosfamide-adriamycin-based regimen, radiation therapy, and one case has been treated with sorafenib with limited effects. Knowing the limited effect of conventional therapy in his case, our patient insisted on receiving imatinib treatment after reading reports of dermatofibrosarcoma protuberans. The patient experienced severe myalgia and no obvious clinical improvement despite the slight softening of the axillary lesion. Radiologically, the lesions remained unchanged in plain chest X-ray. To our knowledge, this is the first case report of metastatic fibrous histiocytoma treated with imatinib.
An 89-year-old man presented with pustules superimposed on painful erythematous plaques covering his scalp after a recent minor abrasion. Despite aggressive debridement, changing dressing, and administration of systemic KU60019 for 4 weeks, the lesions progressed extensively. A physical examination revealed numerous small pustules studded on the yellowish-crusted plaques and superficial erosions. Detachment of the crusts exhibited erosive patches with atrophic skin change (). Repeated cultures from the pustules were unable to demonstrate bacterial or mycological microorganisms. Laboratory investigations showed leukocytosis (15,500/mcL) and elevated levels of C-reactive protein (12 mg/dL). Both serum rapid plasma reagin test and human immunodeficiency virus screening test results were nonreactive. Rheumatoid factor and antineutrophil cytoplasmic antibody were also negative. The titer of antinuclear antibody was slightly elevated (1:40) with a mixed staining pattern. A skin biopsy of the pustular lesions disclosed a dense neutrophilic infiltration and subcorneal microabscesses. In the papillary dermis, diffuse leukocytoclasia and severe papillary edema were observed (A). Vasculitis was not apparent. The inflammatory infiltrate spread into the deep dermis, and the follicles and sebaceous glands displayed remarkable necrosis with infiltration of neutrophils, mononuclear cells, and plasma cells (B). Periodic acid–Schiff stains and direct immunofluorescence study results were negative. Based on the clinical and histological presentations, a diagnosis of erosive pustular dermatosis (EPD) of the scalp was made. We then initiated treatments including oral prednisolone (10 mg/d), topical fusidic acid, and desoximetasone 0.25% ointment. The lesions recovered and only a small area of erosion remained in the 2-month follow-up. In 1979, Pye et al reported six patients with previously undescribed inflammatory dermatosis confined to the scalp, and characterized by sterile pustules, erosions, crusts, and macerated keratin formation, which they termed “erosive pustular dermatosis.” EPD is characteristically reported in elderly female patients, beginning as localized amicrobial pustular lesions, which over a period of months to years can evolve into large eroded areas covered by superficial crusts. Scarring alopecia may occur after a slow and protracted course. The pathogenesis of EPD remains uncertain, but in most cases, a history of local trauma has been documented. Other predisposing factors have included local treatment (cryotherapy, radiotherapy, photodynamic therapy, laser therapy, topical retinoic acid, fluorouracil, or imiquimod), previous varicella zoster viral infection, sun-burn injuries, chronic nonhealing burn wounds, the administration of epidermal growth factor receptor antagonists (e.g., gefitinib), and other associated autoimmune diseases.