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  • She was diagnosed in August and received

    2018-11-01

    She was diagnosed in August 2011 and received one course of chemotherapy with the ProMACE regimen. She next received oral psoralen plus ultraviolet A therapy at the out-patient department with improvement (C). However, chest computed tomography revealed newly found multiple nodules of lung involvement in July 2013. Chemotherapy (ProMACE–CytoBOM) was resumed in August 2013. GMF is a rare histological variant of mycosis fungoides (MF), characterized by a granulomatous infiltrate pattern. The first description of this variant was reported by Ackerman and Flaxman in 1970. However, the clinical features of GMF have no distinct difference from classic MF. Patients often present with typical stepped patch, plaque, or tumor stages. Poikiloderma as a clinical presentation has also been reported. The diagnosis of GMF can be challenging, because its granulomatous NVP-TNKS656 pattern can be mistaken for other types of granulomatous dermatitis. Sarcoid granuloma patterns are most often found, and granuloma annulare patterns and tuberculoid-like granuloma have also been reported. Other causes of infectious and noninfectious granulomatous disease must be ruled out. CD4-positive GMF is most common, although rare cases of CD8-positive GMF have been reported. Atypical lymphocytes, epidermotropism, Pautrier microabscesses, and dermal fibrosis are common histological findings of classic MF. These findings are also typical features for GMF, but epidermotropism has been found in approximately half of reported GMF cases. Atypical lymphocyte infiltration and clinical correlation are important for differential diagnosis. T-cell receptor gene clonal rearrangement detection may be helpful for diagnosis. However, MF in its granulomatous form can still be difficult to diagnose. A previous study found that the average duration before diagnosis of GMF was longer than for classic MF (8.4 years vs. 4.3 years, respectively). The granulomatous infiltrate pattern has not been found to be a conclusive positive prognostic marker. Initially, a granulomatous reaction was thought to be an immune response reaction and a sign of a better prognosis. However, a case–control study reported more frequent disease progression in granulomatous MF than in classic MF (46% and 30%, respectively). Compared with classic MF, the progression-free rate of GMF was significantly lower at 5-year (84% of classic MF vs. 56% of GMF) and 10-year follow-up (59% of classic MF vs. 33% of GMF). However, no significant difference was found in overall survival. Poorer response to skin-directed therapy was observed in GMF patients versus classic MF patients, and more aggressive treatment may be required for the former. Our patient received one cycle of ProMACE chemotherapy, followed by PUVA therapy. Disease progression with multiple lung nodule involvement was noted 23 months later.
    A 45-year-old man had a past history of psoriasis and pulmonary tuberculosis with postantituberculosis treatment status. Psoriasis was diagnosed from typical clinical presentation and histopathology findings. He received irregular treatment with oral methotrexate and topical steroids for psoriasis. The patient was admitted for inpatient treatment because of severe sepsis and erythrodermic psoriasis (). However, he developed multiple severe painful ulcerations on the abdomen, back, and buttocks for 3 days. Some preceding itchy erythematous papules were noted on the lower abdomen 2 days before this episode. No obvious herpes labialis, herpes genitalis, or herpes zoster infection were found. On physical examination, he had widespread and progressive clusters of painful punched-out ulcerations on the abdomen, back, and buttocks (A and C). Tzanck smear from the bases of these lesions tested positive for multinucleated giant cells and keratinocytes with ballooning change (B). We also performed a skin biopsy of the erythroderma to rule out other possibilities; the histopathological diagnosis was psoriasis. In addition, multiple hyperkeratotic papuloplaques and excoriations were noted on the interdigital folds of the hands and bilateral axillae. Skin scrapings from these lesions by potassium hydroxide examination revealed the presence of scabies mites, and the lesions were diagnosed as scabies infestation. The patient was started on a regimen of parenteral acyclovir sodium (Zovirax 250 mg, 3 times daily for 14 days), parenteral teicoplanin (Targocid 400 mg daily for 14 days) for methicillin-resistant bacterial infection, and oral ivermectin (Stromectol 12 mg once and repeated at Day 14) for scabies infestation. Topical silver sulfadiazine cream twice a day was used for wound care. There were significant re-epithelialization and healing after the treatment.