急性白血病的非特异性皮肤表现2例及文献复习

2例患者均为急性白血病,其中1例无明显诱因双下肢皮肤出现红斑、结节,伴有疼痛,后自行消退遗留色素沉着,经检查诊断为急性白血病;另1例双足部皮肤出现红斑伴压痛,经检查诊断为急性单核细胞白血病。在临床看到不典型皮损伴有外周血异常(红细胞、白细胞、血红蛋白或血小板异常)时应考虑白血病皮肤表现的可能,需通过组织病理、骨髓涂片、骨髓病理和流式细胞学检查确诊,以免怠误病情。     

Leukemia cutis in acute myelomonocytic leukemia: infiltration to minor traumas and scars

We describe a patient with acute myelomonocytic leukemia who demonstrated leukemic cell infiltration to scratched wounds and scars from trauma. A 65-year-old Japanese woman developed low grade fever, headache and exanthema. Hematology testing disclosed leukocytosis of 95,600/mm3 with 65% monocytes and 9% blast cells. Infiltrated erythema and nodules were disseminated over most of her body. Moreover, linear scratched wounds and traumatic scars were indurated. Skin biopsy showed dense atypical mononuclear cell infiltration with monocytic characteristics. We discuss the possible reasons for the infiltration of leukemia cells into the wounds and scars from trauma.     

Leukemia Cutis in Three Children: Clinical and Immunohistochemical Studies

Abstract: We report 3 children with leukemia cutis observed at the initial diagnosis of systemic leukemia. Leukemia subtypes in the three children were congenital monocytic, acute undifferentiated, and acute monocytic, respectively. The patients were girls age 10 days, 14 years, and 11 months, respectively, at diagnosis. We describe the clinical features of the cases and the results of immunohistochemical studies on paraffin-embedded skin biopsy specimens. The skin lesions were tumors and areas of reddish purple erythema in the first child, pigmented erythema in the second, and bright red erythema in the third. In the first two patients skin lesion biopsy specimens had dense leukemic infiltrates in the dermis with reactive T lymphocytes scattered among them. In the third patient, the infiltrating cells were almost all reactive T lymphocytes, with a few leukemic cells. A relationship between the leukemic-reactive cell ratio and the prognosis was suggested; dense leukemic cell infiltrates may be associated with a poor prognosis.     

Pernio as the presenting sign of blast crisis in acute lymphoblastic leukemia

A previously healthy 5‐year‐old girl presented with acute onset of blue toes and red spots on the nose and fingers. The striking nature of these lesions, along with the finding of submandibular lymphadenopathy, prompted further evaluation. Laboratory findings were remarkable for anemia, high transaminase levels, and high blast count. Histopathologic findings were consistent with early pernio. Further examination revealed acute B‐cell lymphoblastic leukemia. Treatment of the leukemia led to resolution of the pernio.     

长期以头部皮肤损害为表现的急性髓细胞性白血病1例

报告1例长期以头部皮肤损害为表现的急性髓性白血病(AML)并分析皮肤损害特征、组织病理及髓过氧化物酶(MPO)免疫组化染色特点。该患者为男性,63岁,以局限性头部皮损反复发作7月为其主要症状。皮损呈多形性,以红斑、脓疱、糜烂及溃疡为主,短期内四肢出现浸润性斑块。骨髓病理及流式细胞术提示急性髓性白血病M2型。头部皮肤组织病理见血管周围散在的肿瘤细胞,表达MPO阳性。白血病临床表现复杂,头部浸润是其皮肤受累部位之一。在白血病的诊断以前出现头部皮疹,活检提示有异型细胞浸润,提示白血病的进展及预后不良。     

Subcutaneous histiocytoid Sweet's syndrome in a patient with myelodysplastic syndrome and acute myeloblastic leukemia

Subcutaneous histiocytoid Sweet's syndrome is a rare variant of histiocytoid Sweet's syndrome (SS). We present a 68‐year‐old woman with subcutaneous histiocytoid SS in association with refractory myelodysplastic syndrome transformed to acute myeloblastic leukemia (AML), status post induction chemotherapy and with persistent blasts (50%) in the bone marrow and blood, accompanied with neutropenia. The patient presented to the emergency room with fever and altered mental status. Clinical examination revealed approximately 20 scattered 0.5–2 cm, pink to pink‐purple non‐tender firm nodules on the legs and left arm. The differential diagnosis included Sweet's syndrome (deep), leukemia cutis, infection, polyarteritis nodosa and erythema nodosum. Histopathologic examination of a biopsy from the left arm revealed a nodular infiltrate of neutrophils and histiocytoid mononuclear cells solely in the lobular compartment of the subcutaneous fat with focal areas of necrosis. Most cells in the infiltrate labeled with myeloperoxidase (MPO) including the histiocytoid cells. The cells were negative for CD34 and CD117. All special stains for microorganisms were negative. A diagnosis of subcutaneous histiocytoid SS was made. A subcutaneous histiocytoid SS should be suspected when a neutrophilic/histiocytoid panniculitis, occurring in the setting of myeloid disorders, is encountered and after exclusion of an infectious process and leukemia cutis.     

Cutaneous accumulation of plasmacytoid dendritic cells associated with acute myeloid leukemia: a rare condition distinct from blastic plasmacytoid dendritic cell neoplasm

A cutaneous infiltrate composed of plasmacytoid dendritic cells may occasionally occur in a patient suffering from a myeloid neoplasm. To date, the clinical and pathological features associated with this event remains poorly characterized. Herein, we report a patient with acute myeloid leukemia who developed pruritic papules or erythematous plaques scattered on the skin. Microscopic examination showed a dermal infiltrate rich in plasmacytoid dendritic cells expressing CD4, CD43, CD68, granzyme B, CD123, CD303 [blood dendritic cell antigen 2 (BDCA-2)], CD2-associated protein (CD2AP) and T-cell leukemia/lymphoma oncogene 1 (TCL1). Our observation illustrates further that cutaneous lesions associated with some myeloid neoplasms, especially those featuring a monocytic component, may be composed of plasmacytoid dendritic cells. Because of differences in clinical, pathological and genetic features, this rare condition should be distinguished from blastic plasmacytoid dendritic cell neoplasm.     

Cutaneous involvement as a presenting feature of monocytic leukemia: morphological and immunohistochemical studies

The clinical and pathological findings in a patient with monocytic aleukemic leukemia presenting initially as multiple monoblastic tumors of the skin is described. The patient was a 35-year-old Japanese woman, who had first noticed multiple, asymptomatic, reddish-brown papules on her trunk. Asymptomatic enlargements of several lymph nodes were present in the bilateral cervical and axillary areas. There was no hepatosplenomegaly, sternal tenderness, bruising, or bleeding. The skin and lymph node biopsies were originally interpreted as malignant lymphoma. The diagnosis of acute monocytic leukemia was established when bone marrow involvement was detected. Immunohistochemical observation of the skin eruptions revealed the following: Positive staining with lysozyme was noted in almost half of the infiltrating atypical cells. Most of the infiltrating cells reacted positively with antisera to Leu-M5 and some of them reacted to Leu-M1. The helper T cell antibody, Leu-3a+3b, showed weak positive staining of most infiltrating cells. However, there were no reactions with antisera to Leu-6, Leu-7, Leu-14, CALLA, OKT 6, OKT 8, OKT 16, OKB 19, OKM 14, beta F1, or delta TCS1. OKM 5-positive keratinocytes were observed in some parts of the upper epidermis, although no OKM 5 expression could be detected on any tumor cells. Cytochemistry, immunohistochemistry, and electron microscopy can aid in the diagnosis of monocytic leukemia. This case illustrates the importance of using an expanded panel of monoclonal antisera in certain hematopoietic tumors.     

皮肤白血病1例

患者，男性，22岁。1999年5月出现发热，经血液和骨髓穿刺证实为急性骨髓性白血病M5型（急性单核细胞性白血病）。2000年2月行自身外周血干细胞移植并顺利植活。移植后4个月患者发现前胸部出现紫红色结节、斑块，组织病理为“继发皮肤白血病”，骨髓穿刺和皮损免疫组化诊断为“急性骨髓白血病M5型复发”。经10天化疗，患者皮损减轻。     

毛囊黏蛋白病二例并文献复习

报道2例毛囊黏蛋白病并进行文献复习.患者1,男,44岁,颈项部局限性浸润性红斑伴疼痛2个月,组织病理示:真皮毛囊水肿,毛囊内外淋巴组织细胞浸润,个别嗜酸粒细胞.阿新蓝染色:毛囊黏蛋白阳性.患者2,男,32岁,左面部浸润性暗红斑45天,组织病理示:表皮大致正常,毛囊上皮水肿,淡蓝色黏液样物质沉积,毛囊内及毛囊周围较多嗜酸...     

Cutaneous mucormycosis in a patient with acute lymphocytic leukemia

We describe a patient with invasive necrotizing cutaneous mucormycosis caused by Rhizopus oryzae. The patient, who had been suffering from acute lymphocytic leukemia (ALL) for eight months, had erythema and necrosis surrounded by swelling on the dorsum of his left hand. Debridement was performed, and microscopic examination of the obtained specimens revealed mucormycosis. Because amphotericin B was ineffective, amputation at the left shoulder joint was performed. Bone marrow transplantation (BMT) was successfully carried out 22 days after surgery. However, the patient died 162 days after the BMT due to progression of the ALL. Patients such as the present one should be evaluated promptly by tissue biopsy and appropriate cultures, so that vigorous treatment can be started without delay. Where necessary, amputation should be performed.     

Cutaneous presentation preceding acute myeloid leukemia with CD4+/CD56+ expression misdiagnosed as a blastic plasmocytoid dendritic cell neoplasm: A case report

Acute myeloid leukemia (AML) may initially present as cutaneous lesions corresponding to blasts involving the skin as the first clinical manifestation prior to blood and bone marrow (BM) infiltration. Such presentation is known as myeloid leukemia cutis (LC). Blastic plasmocytoid dendritic cell neoplasm (BPDCN) is an aggressive tumor derived from the precursors of plasmocytoid dendritic cells with cutaneous and BM involvement and leukemic dissemination. Myeloid LC and BPDCN may be difficult to distinguish as they share similar clinical and histopathological features, in particular AML with monocytic differentiation. Nevertheless, the correct diagnosis has to be made to determine adequate and effective therapy. Here, we report the case of a 61‐year‐old woman who presented with an AML with MLL rearrangement and CD4+/CD56+ expression presenting as LC and that was misdiagnosed as BPDCN. We emphasize that careful and exhaustive analyses should be performed to make the correct diagnosis.     

Extramedullary acute leukemia developing in a pre‐existing osteoma cutis

Primary osteoma cutis (cutaneous ossification) is an uncommon disease in which there is bone formation within the skin in the absence of a demonstrable pre‐existing condition. Osteoma cutis is a chronic and benign condition. We report a case of a 45‐year‐old man who developed extramedullary acute leukemia with a myeloid immunophenotype (myeloid sarcoma) with its initial presentation within an isolated pre‐existing osteoma cutis in the post‐auricular scalp without evidence of systemic acute leukemia or chronic myeloid stem cell disorders. The tumor was surgically excised without complications. Four months later, acute leukemia recurred in the contralateral posterior mandible and showed an immunophenotype consistent with acute lymphoblastic leukemia/lymphoma. The patient now has been treated by standard protocols for acute leukemia. The diagnosis of an extramedullary acute leukemia is challenging because of its inconsistent clinical and histopathologic presentations. Extramedullary acute leukemia developing in a pre‐existing osteoma cutis is very unusual and has not been previously reported in the literature.     

Trisomy 8 in myeloid leukemia cutis confirmed by fluorescence in situ hybridization analysis

We present a case of a 64‐year‐old man with refractory acute myeloid leukemia and trisomy 8 who developed leukemia cutis. Interphase fluorescence in situ hybridization (FISH) was performed on a paraffin‐embedded skin section. FISH confirmed a population of cells with trisomy 8 in the blastic infiltrates involving the skin. This case illustrates a novel application of interphase FISH to confirm the diagnosis of leukemia cutis.     

Eosinophilic dermatosis of hematologic malignancy

Cutaneous involvement by an eosinophil-rich process (eosinophilic dermatosis) may be encountered in the setting of various hematologic malignancies, including mantle cell lymphoma, acute monocytic leukemia, acute lymphoblastic leukemia, large cell lymphoma, myelofibrosis and chronic lymphocytic leukemia (CLL). Of the various hematologic malignancies, eosinophilic dermatosis has been most frequently described in association with CLL. Published previously as insect bite-like reaction and eosinophilic dermatosis of myeloproliferative disease, this rare dermatitis presents as a pruritic, papular and occasionally vesicular eruption associated with an eosinophil-rich infiltrate histopathologically. Although clinical and histopathologic features are similar to insect bites, affected patients frequently deny a history of insect bites. We report a case of eosinophilic dermatosis of hematologic malignancy in a patient with known history of CLL.     

Leukemia cutis. A histopathologic study of 42 cases

Biopsy specimens of skin from 42 patients with leukemia cutis were studied. Typing of leukemia was based on histopathologic and histochemical findings in peripheral blood and bone marrow. There were three patients with acute lymphocytic leukemia, 16 with chronic lymphocytic leukemia, 12 with acute granulocytic leukemia, three with chronic granulocytic leukemia, five with acute monocytic leukemia, and three with acute myelomonocytic leukemia. In general, leukemia cutis shows a diffuse infiltration of leukemic cells in the dermis and subcutaneous tissue, often infiltrating between collagen bundles. Extensive involvement and disruption of blood vessels and skin adnexa are characteristic findings in granulocytic, monocytic, and myelomonocytic leukemia cutis, but biopsy specimens of skin in patients with different types of leukemia show a wide range of histopathologic changes that are variable among the various types of leukemia and sometimes even among different patients with the same type of leukemia. A final typing of leukemia should not rely only on regular histopathologic findings of a skin biopsy, but should depend more on morphologic and histochemical studies of smears of peripheral blood or of bone marrow or both.     

Erythema nodosum and granulomatous lesions preceding acute myelomonocytic leukemia

A 65-year-old female with a one-month history of painful eruptions on her lower extremities was admitted to our hospital. Histological examination revealed erythema nodosum (EN), and the patient was treated with oral prednisolone (PSL; 20 mg daily). The eruptions subsided in two weeks. One month later, painful reddish eruptions recurred on her upper limbs and abdomen in addition to her lower extremities. A skin biopsy from an abdominal erythematous plaque revealed a non-caseating granuloma without microorganisms or foreign-body materials. These eruptions also disappeared with treatment with oral PSL (20 mg daily). No underlying disease, including sarcoidosis, diabetes mellitus, or rheumatoid arthritis, was found. However, five months later, the patient developed conspicuous leukocytosis. She was diagnosed with acute myelomonocytic leukemia (M4) and treated with chemotherapy. After complete remission had been achieved, the EN reappeared, in association with an increase in blastic cells in the bone marrow. Serum levels of tumor necrosis factor-alpha and interleukin-1 beta, which are thought to be essential for granuloma formation and induction of EN, were markedly elevated. Physicians must remember that recurrent EN and granulomatous lesions can be a prodromal sign of leukemia.     

Ponatinib‐induced neutrophilic panniculitis

Ponatinib is a bcr‐abl tyrosine‐kinase inhibitor (TKI) used to treat resistant and refractory chronic myeloid leukemia and Philadelphia chromosome‐positive acute lymphoblastic leukemia that express bcr‐abl. Neutrophilic panniculitis has been described in rare cases of patients on other TKIs in the same class as ponatinib. We present the first case of neutrophilic panniculitis following treatment with ponatinib and summarize the other cases of panniculitis caused by TKIs.     

不累及腋臭手术部位的间擦疹型手足综合征一例国际首报

【摘要】 患者女,25岁,因手足、腋下和腹股沟红斑水疱伴疼痛反复1个月就诊。7年前患者因腋臭双腋下曾行小切口汗腺切除术。1个月前因颈部滑膜肉瘤（ⅡB期）术后行多柔比星脂质体化疗,3次化疗期间,出现手足、腋下和腹股沟红斑水疱伴疼痛,皮损逐次加重。皮肤科检查：双手掌、足跖及腋下、腹股沟等间擦部位可见大片水肿性红斑,边界较清楚,上有粟粒至黄豆大小水疱,可见糜烂;皮疹处皮温高,触痛明显,尼氏征阳性;双腋下行小切口腋臭手术的部位无皮损,无疼痛。腋下皮损组织病理检查：基底层下水疱形成及部分汗腺坏死。诊断：多柔比星脂质体相关间擦疹型手足综合征。根据该病例合并腋臭手术史,手术部位皮肤正常,推测该病发病机制可能为药物经汗腺排泄到皮肤后诱发的皮肤毒性反应。     

Entero‐Behçet's disease coexisting with long‐term epilepsy and schizophrenia‐like symptoms

Entero‐Behçet's disease coexisting with long‐term epilepsy and schizophrenia‐like symptoms is presented. A 43‐year‐old woman presented with repeatedly occurring aphthous stomatitis for several years. She had been treated for absence seizures, epilepsy and schizophrenia since she was 9 years old. She presented with multiple aphthous stomatitis on her gingiva, erythema nodosum‐like symptoms on the right lateral aspect of her leg and genital ulcers on her perianal area. She also showed polyarthritis. Laboratory examinations revealed elevated C‐reactive protein, elevated neutrophil counts, decreased serum Fe and elevated serum Cu. Histological examination showed perivascular neutrophil and mononuclear cell infiltrates and eosinophilic change of the vessel wall in the lobules of subcutaneous fat tissue. Six weeks after the oral prednisolone therapy, she showed resolution of aphthous stomatitis, folliculitis‐like eruption and genital ulcer. She experienced severe abdominal pain after the start of treatment of Behçet's disease. Plain computed tomography revealed edematous change in the appendix, and ascending and transverse colon. These results led to the diagnosis of entero‐Behçet's disease acute exaggeration. Treatment with infliximab (300 mg/once) was started. Eight weeks after the start of infliximab, her abdominal pain disappeared and C‐reactive protein decreased, followed by the successful change to adalimumab infusion therapy.