Introduction Angiosarcoma developing in chronically lymphedematous tissue, or Stewart‐Treves syndrome (STS), is a rare and lethal complication of lymphedema. This systematic review summarizes characteristics and outcomes of STS and other cutaneous malignancies arising in chronic lymphedema. Methods MEDLINE and EMBASE databases were searched on February 19th, 2021, to identify 200 articles included in the analysis. Results Of 369 included patients, 89.7% (n = 331/369) had STS and 10.3% (n = 38/369) had other associated malignancies. Mean age of onset was 61.2 years, and 85.9% (n = 317/369) of cases were female. Common risk factors were previous cancer history (69.8%, n = 258/369) and radiation history (53.7%, n = 198/369). Lymphedema was most commonly attributed to surgical causes (68.3%, n = 252/369). STS begins on average 14.9 years after lymphedema with mortality of 53.9% (n = 178/331) and remission rate of 16.1% (n = 53/331). Other malignancies begin on average 23.7 years after lymphedema, with mortality of 10.5% (n = 4/38) and remission rate of 31.6% (n = 12/38). STS and other malignancies had 5‐year survivals of 22.4% and 65.2%, respectively (P = 0.00145). For all patients, patients initially treated with excision had the best survival (median: 48 months, 5‐year survival: 43.3%) and radiotherapy had the worst survival (median: 10 months, 5‐year survival: 6.5%) (P = 0.0141). Conclusion Malignancy can appear in lymphedematous tissue many years after lymphedema onset. STS has poorer prognosis compared to other malignancies. Further research should be conducted to better understand the causes, risk factors, and management of this phenomenon.
Background: Palisaded neutrophilic granulomatous dermatitis is a rare inflammatory dermatosis with possible underlying systemic conditions including rheumatoid arthritis, autoimmune connective tissue disease, and malignancies. Case Summary: We report a case of an 84-year-old man presenting with a 3-week eruption of asymptomatic annular plaques on his neck, which progressed to involve his back and legs. Skin biopsies confirmed a diagnosis of palisaded neutrophilic granulomatous dermatitis, and he was treated with prednisone. Full workup related to potential underlying causes of palisaded neutrophilic granulomatous dermatitis was completed. Conclusion: Palisaded neutrophilic granulomatous dermatitis may precede the onset of underlying systemic conditions or occur concomitantly. Following the diagnosis, clinicians should perform a comprehensive focused history, physical examination, and laboratory investigation related to the associated underlying diseases.
A case of elastosis perforans serpiginosa associated with long-term treatment with penicillamine Dear Editor, A 42-year-old man with a history of Wilson's disease controlled on penicillamine for 26 years presented to the dermatology clinic with a 6-month history of asymptomatic grouped crateriform violaceous papules in the left axilla and on the posterior neck (Fig. 1). Histopathological findings showed a hyperplastic epidermis with a dilated follicular infundibulum and elimination of eosinophilic fragmented elastic fibers through a channel (Fig. 2). Trichrome stain highlighted increased numbers of coarse
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