Mycosis fungoides bullosa simulating pyoderma gangrenosum

A patient with mycosis fungoides (MF) bullosa had a rapidly growing, painful necrotic mass on the left ankle which extended by peripheral bulla formation, clinically resembling pyoderma gangrenosum. Histopathology confirmed MF bullosa with both intraepidermal and subepidermal bulla formation.     

Mycosis fungoides presenting as Ofuji''s papuloerythroderma

We report three patients presented with clinical features of Ofuji's papuloerythroderma (pruritic erythematous papules and extensive erythema sparing all skin folds), however, showing histopathological findings of mycosis fungoides (Pautrier's microabscess, haloed lymphocytes, disproportionate epidermotropism, and wiry collagen bundles). One case was associated with plaque stage of mycosis fungoides and follicular mucinosis. T-cell receptor (TCR) gene rearrangement analysis in the lesional skin tissue demonstrated rearrangement of the gamma chain in all cases. HTLV-1 serology was negative for two patients who conducted HTLV-1 test.We think that Ofuji's papuloerythroderma might be a variant of early mycosis fungoides rather than secondary skin manifestations to certain cutaneous inflammatory diseases.     

具有多种表现的蕈样肉芽肿一例

患者,男,80岁。手足反复出现多种皮损伴瘙痒、疼痛4年,加重1年。皮肤活检和组织病理示：表皮内及真皮浅层血管周围灶状淋巴样细胞浸润。免疫组化示：CD3（+）、CD4（+）、CD5（-）、CD7（+）、CD8（+）、CD20（+）,Ki67（10％）。诊断：蕈样肉芽肿。予以抗组胺药物、糖皮质激素药膏、窄谱中波紫外线照射治疗,随访。     

Mycosis fungoides mimicking erysipelas

Mycosis fungoides may rarely simulate facial erysipelas. In a patient with that clinical presentation, a biopsy specimen revealed a diffuse dermal infiltrate with numerous neutrophils that also mimicked erysipelas histopathologically. The diagnosis of mycosis fungoides was made on the basis of atypical lymphocytes with focal epidermo‐ and folliculotropism. It was confirmed by typical findings of mycosis fungoides in a second biopsy from a clinically inconspicuous patch. An identical T‐cell receptor γ‐gene rearrangement was detected in lymphocytes of both biopsy specimens.     

大疱性蕈样肉芽肿

报告1例大疱性蕈样肉芽肿.患者男,40岁.因躯干、四肢红斑伴瘙痒6年,出现斑块半年、水疱1个月就诊.皮肤科检查:面部、躯干及四肢泛发红斑和斑块,部分斑块浸润明显.右下腹斑块上可见0.5 cm×2 ca的水疱.皮损组织病理检查:真皮浅层明显水肿,部分区域可见表皮下疱形成,真皮浅层可见异形明显的单一核细胞呈带状浸润.部分单一核细胞侵入表皮,形成Pautrier微脓肿.免疫组化染色结果示LCA( )、CD4( )、CD45RO( ).结合临床、免疫组化和组织病理检查结果,诊断为大疱性蕈样肉芽肿.     

以四肢色素沉着为主要表现的蕈样肉芽肿1例

报告1例以四肢色素沉着为主要表现的蕈样肉芽肿,患者男,56岁,手足及四肢伸侧黑褐色斑4年,境界不清楚,进行性加重,伴轻度瘙痒,组织病理检查见多个深梁的单一核细胞侵入表皮,并形成Pautriet微腺肿,真皮内单一核细胞带状浸润,浸润细胞轻度异形,免疫组化提示为CD4( )T细胞来源的肿瘤,给予糖皮质激素及氮芥外用,皮损略缓解,临床随访6年,病情稳定.     

伴有狮面的蕈样肉芽肿累及毛囊一例

患者，女，67岁。躯干、四肢斑丘疹4年，面部肥厚红斑2年，伴剧烈瘙痒。皮肤组织病理示棘层及毛囊上皮pautrier微脓肿和真皮单一核细胞带状浸润，异形性明显。免疫组化示CD3、CD4强阳性，Ki-67 20%。TCR基因重排示TCRγ、TCRβ、TCRδ均阳性。诊断：蕈样肉芽肿肿瘤期累及毛囊。给予口服美卓乐24 mg/d，2周后加量至32 mg/d，隔日肌肉注射一次300万IU重组人干扰素α-2a，2周后患者面部肿胀及瘙痒明显消退。     

以色素性紫癜性皮病为表现的蕈样肉芽肿一例

患者,男,70岁。左下肢皮疹10余年,泛发全身伴痒3个月。皮肤科检查：四肢为主的广泛对称的紫癜性暗红色斑片、斑块,躯干散在的暗红色斑疹表面可见明显鳞屑。皮肤组织病理示符合蕈样肉芽肿诊断。患者给予重组人干扰素α2b,肌注,300MIU,1周3次,甲氨蝶呤10 mg,1周1次,同时配合窄谱中波紫外线（NB-UVB）1周3次治疗。经1年余治疗后患者明显好转。     

Mycosis fungoides: radiation therapy

Radiation therapy is the most effective single agent for the treatment of mycosis fungoides. There are well-defined dose-response relationships for achieving a complete response as well as the durability of this response. Techniques of electron beam therapy have been developed that permit treatment of the entire skin. Total-skin electron beam therapy is an important form of management, especially for patients who have thick generalized plaque or tumorous disease. Radiation therapy may also be used selectively for treatment of extracutaneous disease.     

Pathogenesis of Mycosis fungoides

Mycosis fungoides is the most common type of primary cutaneous lymphomas. The phenotype of the tumor cell corresponds to an effector/memory‐type of helper T cell which, given its repertoire of homing receptors, is specialized for recirculation through the skin. In recent years genetic analyses have uncovered various chromosomal aberrations in the tumour cells of mycosis fungoides. Their relevance to the pathogenesis and clinical appearance are discussed in the following.     

Mycosis fungoides responding to systemic itraconazole

Mycosis fungoides is a form of cutaneous lymphoma with a typically indolent course. We describe an unexpected, repeated response to a systemic antifungal agent, itraconazole. The mechanism of action is speculative but itraconazole has marked immunosuppressive activity on T-lymphocyte proliferation in vitro.     

表现为进行性色素性紫癜性皮病的蕈样肉芽肿1例

患者,女,76岁。双下肢出现红色瘀点、进行性增多伴轻微瘙痒半年。组织病理示:表皮及真皮浅层单一核细胞浸润,表皮内可见Pautrier微脓肿。免疫组化:CD3(+)、CD4(+)、CD8(散在+)、CD45R0(+)、Ki-67(20%左右+)。诊断:蕈样肉芽肿。     

Use of Becaplermin for nondiabetic ulcers: pyoderma gangrenosum and calciphylaxis

Large difficult to heal ulcers of various etiologies carry a high morbidity and mortality rate. Becaplermin is a recombinant platelet‐derived growth factor approved for treatment of diabetic ulcers. In this two‐case series, we report the use of becaplermin in the treatment of ulcers due to (i) calciphylaxis, an often fatal condition resulting from systemic calcification and thrombosis of vessels and (ii) pyoderma gangrenosum (PG), a neutrophilic dermatosis. We also report that topical collagenase worsened PG ulcers, consistent with pathergy. Becaplermin can be used to help treat ulcers resulting from calciphylaxis and PG. These encouraging results lend support for the utilization of becaplermin in the treatment of nondiabetic chronic ulcers of various etiologies.     

Mycosis fungoides: a dermatological masquerader

Mycosis fungoides (MF), a low-grade lymphoproliferative disorder, is the most common type of cutaneous T-cell lymphoma. Typically, neoplastic T cells localize to the skin and produce patches, plaques, tumours or erythroderma. Diagnosis of MF can be difficult due to highly variable presentations and the sometimes nonspecific nature of histological findings. Molecular biology has improved the diagnostic accuracy. Nevertheless, clinical experience is of substantial importance as MF can resemble a wide variety of skin diseases. We performed a literature review and found that MF can mimic >50 different clinical entities. We present a structured framework of clinical variations of classical, unusual and distinct forms of MF. Distinct subforms such as ichthyotic MF, adnexotropic (including syringotropic and folliculotropic) MF, MF with follicular mucinosis, granulomatous MF with granulomatous slack skin and papuloerythroderma of Ofuji are delineated in more detail.     

Adalimumab in Japanese patients with active ulcers of pyoderma gangrenosum: Twenty-six-week phase 3 open-label study

This phase 3 multicenter study, including 26-week treatment and extension periods, evaluated the efficacy and safety of adalimumab in Japanese patients with active ulcers due to pyoderma gangrenosum. Patients received adalimumab 160 mg at week 0, 80 mg at week 2, and then 40 mg every week starting at week 4. Of the 22 enrolled patients, 12 (54.5%, P < 0.001) achieved the primary efficacy end-point of pyoderma gangrenosum area reduction 100 (PGAR 100, defined as complete skin re-epithelialization) for the target ulcer at week 26 assessed by digital planimetry. PGAR 100 response was observed as early as week 6 (13.6%) and continued to increase over time. The mean percent change from baseline in target ulcer area was −31.8% at week 6 and −63.8% at week 26. A Physician’s Global Assessment score of 0 (PGA 0, all ulcers completely clear) was achieved by two patients (9.1%) at week 6 and eight (36.4%) at week 26, while PGA 0/1 (completely/almost clear) was achieved by five (22.7%) and 12 patients (54.5%) at week 6 and 26, respectively. Mean changes from baseline in pain numeric rating scale (−1.8 at week 6 and −2.5 at week 26) and the Dermatology Life Quality Index (−3.1 at week 6 and −3.6 at week 26) improved over time. Adverse events were reported by 18 patients, most commonly infections (n = 11), and serious adverse events by four. These results suggest that adalimumab is effective and generally well tolerated in Japanese patients with active ulcers of pyoderma gangrenosum.     

Case of pyoderma gangrenosum showing oral and genital ulcers, misdiagnosed as Behcet's disease at first medical examination

It is occasionally difficult to distinguish between Behcet's disease (BD) and pyoderma gangrenous (PG). Our case showed ulcers of the oral, vaginal and perineal areas, and in the ileum, thus resulting in our initial diagnosis of BD. However, the patient showed a continued leukopenia, and she was subsequently diagnosed by bone marrow biopsy as having a myelodysplastic syndrome, which will sometimes accompany PG. In addition, following a hysterectomy, the ulcers of the stump in the vagina and the perineum showed the characteristic findings of a PG-like destructive ulceration. Based on these findings, we finally diagnosed our case to have PG.     

Treatment of pyoderma gangrenosum with oral Tripterygium wilfordii multiglycoside

Two patients with refractory pyoderma gangrenosum (PG) were treated with oral Tripterygium wilfordii multiglycoside (TWG). TWG is a Chinese medicine extracted from a medicinal herb, Tripterygium wilfordii Hook F, and has potent anti-inflammatory and immunosuppressive effects. The effect of TWG on PG was demonstrated by clinical findings. Improvement of the lesions occurred within two weeks, and the ulcers healed about a month. Mild side effects such as gastrointestinal disturbances were observed in both patients. These side effects were patient-acceptable, and there was no need to stop the treatment. Transient elevation of serum ALT was observed in one patient; the serum ALT returned completely to normal after the discontinuation of TWG. These results suggest that TWG may be an effective alternative for refractory PG and that careful monitoring of liver function during TWG treatment is necessary.