Aleukemic leukemia cutis presenting as benign-appearing exanthema.

Aleukemic leukemia cutis is a rare condition characterized by the infiltration of the skin by leukemic cells before their appearance in the peripheral blood or bone marrow. We report here a 62-year-old seemingly healthy patient who presented with disseminated erythematous maculae. A skin biopsy showed leukemia cutis of monocytic type. No involvement of bone marrow or peripheral blood was found. The patient developed acute monocytic leukemia 7 months later. We present this case to illustrate how leukemia cutis can masquerade as a clinically benign-appearing cutaneous eruption without leukemic changes in blood or bone marrow. To confirm the diagnosis of aleukemic leukemia cutis, immunohistochemistry of the skin lesions as well as a complete staging procedure is necessary.     

Leucemia cutánea aleucémica: presentación de un caso

—The term aleukemic leukemia cutis describes the invasion of the skin by leukemic cells in the absence of peripheral blood and/or bone marrow involvement. Although the pathogenic mechanisms of this disease are still unknown, it is widely recognized its poor prognosis. Most of the reported patients completely developed the hematologic disease (the majority acute granulocytic or monocytic leukemias) in 10-14 months after diagnosis of aleukemic leukemia cutis, and they had a median survival of 22 months.We report on a case of a 77-year-old man with aleukemic leukemia cutis who presented with numerous erythematous to violaceous papulonodular lesions, mainly located on the trunk and head, lack of peripheal blood involvement and very early alterations in the bone marrow aspirate. Inmunohistochemical stains of skin and bone marrow biopsies revealed the infiltration of both tissues by blast cells of monocyte-histiocytic lineage. Our patient presented spontaneous resolution of skin lesions and his hematological disease showed no progression during 9 months after diagnosis, although he did not undergo any treatment.We emphasize the low incidence of association between myelodysplastic syndromes and aleukemic leukemia cutis as well as the surprising spontaneous remission of cutaneous lesions in our patient.     

Aleukemic monocytic leukemia cutis

Aleukemic leukemia cutis is a rare condition in which leukemic cells invade the skin before they appear in peripheral blood or bone marrow specimens. The condition frequently is misdiagnosed as atypical lymphoma. Generally, the diagnosis is made retrospectively, after the leukemic cells appear in peripheral blood or bone marrow samples. Immunohistochemical studies are the primary methods for diagnosis. Prognosis is usually poor. We describe the case of a 75-year-old woman with acute aleukemic monocytic leukemia cutis who developed systemic disease 1 1/2 years after skin involvement.     

Acute myelomonocytic leukemia presenting as a benign-appearing cutaneous eruption

Aleukemic leukemia cutis is a rare condition in which patients have skin lesions containing leukemic cells before evidence of leukemia can be detected in the peripheral blood. There are only 23 cases of this phenomenon documented in the English literature. We describe a 62-year-old woman who developed a diffuse, clinically benign-appearing cutaneous eruption, which histologically showed an atypical infiltrate of cells, 4 months before leukemic cells were found in her peripheral blood and the diagnosis of acute myelomonocytic leukemia was made by bone marrow aspiration. This case illustrates the difficulty in diagnosing leukemia cutis from examination of routine histologic sections and the importance of specialized marker studies in determining the cause of an atypical cellular infiltrate of the skin. It also illustrates how leukemia cutis can masquerade as a clinically benign-appearing cutaneous eruption in a seemingly healthy patient with normal blood parameters.     

Aleukemic leukemia cutis

Aleukemic leukemia cutis has always been a dermatological curiosity. It is a rare condition characterized by the infiltration of skin by leukemic cells before their appearance in the peripheral blood or bone marrow. A 20 year old man had presented with nodular swelling on the scalp of 6 months duration along with cervical lymphadenopathy. Biopsy and immunohistochemistry revealed myeloid sarcoma. The initial presentation was aleukemic and repeated peripheral blood counts and marrow examinations were normal. However, the outcome was fatal within 3 months of diagnosis of cutaneous lesions.     

Aleukemic leukemia cutis in a child preceding T-cell acute lymphoblastic leukemia

An 8-year-old boy presented with a widespread cutaneous eruption featuring macules, papules, nodules, and ulcers. The histologic infiltrate showed T lymphoblasts, but there was no sign of systemic involvement, so aleukemic leukemia cutis was diagnosed. Two months later, he developed leukemia in peripheral blood and bone marrow that was characterized as T-cell acute lymphoblastic leukemia.     

Spontaneous regression of aleukemia congenital leukemia cutis

A full-term 2-week-old boy was referred to the pediatric dermatology clinic with numerous blue to violaceous nodules present since birth. TORCH titers (against toxoplasmosis, cytomegalovirus, herpes simplex virus, rubella, and syphilis) were negative. Complete blood count and peripheral smear were normal. A skin biopsy specimen showed an atypical cellular infiltrate suspicious for leukemia or lymphoma. A bone marrow biopsy specimen demonstrated acute myelogenous leukemia (M4 subtype). Following consultation with pediatric oncology and the recognition of the potential for spontaneous regression, chemotherapy for the infant's condition was not recommended. He remained otherwise healthy and was followed-up with biweekly to monthly complete blood counts and physical examinations, which were repeatedly normal. By 3 months of age, the nodules had completely resolved and there was no evidence of recurrence at 8 months of follow-up. We report this instance of aleukemic congenital leukemia with spontaneous regression of leukemia cutis without therapeutic intervention.     

Clinicopathologic correlations in leukemia cutis

This clinicopathologic study involved 42 cases of leukemia cutis: 3 of acute lymphocytic leukemia (ALL), 16 of chronic lymphocytic leukemia (CLL), 12 of acute granulocytic leukemia (AGL), 3 of chronic granulocytic leukemia (CGL), 5 of acute monocytic leukemia (AML), and 3 of acute myelomonocytic leukemia (AMML). The clinical appearance of leukemia cutis included papules, macules, plaques, nodules, ecchymoses, palpable purpura, and ulcerative lesions, and these were seen in all types of leukemias. Gingival hypertrophy was seen only in AML or AMML, and erythroderma and bullous lesions of leukemic infiltration were observed only in CLL. Cutaneous leukemic lesions may be concomitant with or preceding the diagnosis of systemic leukemia. Therefore, skin biopsy may be helpful in detecting the leukemia and may facilitate the work-up. Leukemia cutis probably is a dissemination of systemic leukemia to the skin, and the demonstration of leukemia in skin is associated with a very poor prognosis.     

Cutaneous presentation of aleukemic monoblastic leukemia cutis - a case report and review of literature with focus on immunohistochemistry

Aleukemic monoblastic leukemia cutis is a rare cutaneous manifestation of a systemic hematological disorder associated with dermal infiltration of monoblasts preceding bone marrow or peripheral blood involvement. We report a case of a 75-year-old woman who presented with an erythematous maculopapular rash, which was clinically diagnosed as viral exanthema. Microscopy of the skin biopsy showed features of monoblastic leukemia. Her general physical condition rapidly deteriorated and she died 4 weeks later. We present this case to alert dermatologists of innocuous erythematous skin lesions clinically resembling a viral exanthema, which, in rare instances, may be a presenting feature of an aleukemic monoblastic leukemia cutis. This entity poses problems for dermatopathologists even on immunohistochemistry as monoblasts are negative for hemopoietic precursor cell antigens like CD34, Terminal deoxynncleotidy1 transferase (TdT) and CD117.     

Bullous pyoderma gangrenosum as a manifestation of leukemia cutis

The authors present the case of a 67-year-old patient in whom bullous pyoderma gangrenosum was the first symptom of acute myeloid leukemia. Histologically leukemic cells were found in the skin infiltrate, on the basis of which this lesion satisfied the criteria of leukemia cutis. It was underlined that in the background of such atypical bullous cases there are often hemoblastoses or their malignant transformation. Finally the connection between bullous pyoderma gangrenosum and atypical vesiculous Sweet syndrome is discussed.     

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。     

Leukemia cutis in acute lymphocytic leukemia masquerading as viral exanthem.

Leukemia cutis is a specific skin lesion caused by infiltration of leukemic cells into the skin. It is uncommon in acute lymphocytic leukemia (ALL). It typically manifests as red or violaceous papules, nodules, or plaques, mainly on the face. Leukemia cutis presenting with a generalized viral exanthem-like maculopapular eruption appears to be rare in the English literature. We report such a case. A 19 year-old man presented with a generalized purpuric maculopapular eruption of eight day's duration. Hematologic studies showed changes of acute lymphocytic leukemia, T-cell type. A skin biopsy specimen revealed a cuff-like, dense, perivascular infiltration of atypical lymphocytes in the upper and mid-dermis, consistent with leukemia cutis. The rash resolved in two weeks after chemotherapy. Our case illustrates that leukemia cutis should be considered in the differential diagnosis of a generalized morbilliform viral exanthem-like eruptions. Skin biopsy is important in establishing the 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.     

Diffuse calcinosis cutis in a patient with congenital leukemia and leukemia cutis

We report an unusual case of congenital leukemia with leukemia cutis (LC) and diffuse calcinosis cutis. A newborn girl presented with widespread dusky red and yellowish cutaneous nodules and papules. Bone marrow morphology was consistent with the diagnosis of acute monocytic leukemia of the FAB M5 type. Skin biopsy specimens confirmed the presence of a leukemic infiltrate and revealed calcium salt deposition in the papillary and reticular dermis. Calcinosis was diffuse in the whole skin but spared other organs. Vascular calcification was not present. Serum calcium levels oscillated between 2.5 and 2.86 mmol/l, and phosphorus, parathyroid hormone and 25-hydroxyvitamin D(3) levels were normal. There were diffuse osteoporosis and spontaneous fractures of small tubular bones. The patient responded to chemotherapy but, following consolidation treatment, developed sepsis and died at 120 days of age. Congenital leukemia is rare and LC is uncommon. Hypercalcemia may be a complication of leukemia, which leads to multiorgan metastatic calcification. Despite the absence of frank hypercalcemia, the presence of bone lesions suggests that the patient's calcinosis cutis was of the metastatic type. However, the cutaneous leukemic infiltrate may also represent a triggering factor for calcium deposition in the skin.     

Leukemic vasculitis: a rare pattern of leukemia cutis

Although non‐specific skin lesions are quite common in patients with leukemia, the specific infiltration of the skin by blast cells, known as leukemia cutis, is rare. Its incidence ranges from 1 to 50% and depends on the specific type of leukemia. Leukemic vasculitis represents a rare form of leukemia cutis consisting of the involvement and destruction of vessel walls by leukemic cells, which in themselves cause the vascular injury. To date, only few cases of leukemic vasculitis have been described. Here, we report two cases of this rare skin condition, one of which mimicked cutaneous polyarteritis nodosa. Cañueto J, Meseguer‐Yebra C, Román‐Curto C, Santos‐Briz A, Fernández‐López E, Fraile C, Unamuno P. Leukemic vasculitis: a rare pattern of leukemia cutis.     

Clinical and pathological features of myeloid leukemia cutis

BackgroundMyeloid leukemia cutis is the terminology used for cutaneous manifestations of myeloid leukemia.ObjectiveThe purpose of this study was to study the clinical, histopathological and immunohistochemical features of myeloid leukemia cutis.MethodsThis was a retrospective study of clinical and pathological features of 10 patients with myeloid leukemia cutis.ResultsOne patient developed skin lesions before the onset of leukemia, seven patients developed skin infiltration within 4-72 months after the onset of leukemia, and two patients developed skin lesions and systemic leukemia simultaneously. Of these patients, five presented with generalized papules or nodules, and five with localized masses. The biopsy of skin lesions showed a large number of tumor cells within the dermis and subcutaneous fat layer. Immunohistochemical analysis showed strong reactivity to myeloperoxidase (MPO), CD15, CD43 and CD45 (LCA) in most cases. NPM1 (nucleophosmin I) and FLT3-ITD (Fms-like tyrosine kinase 3-internal tandem duplication) mutations were identified in one case. Five patients with acute myelogenous leukemia and one patient with chronic myelomonocytic leukemia died within two months to one year after the onset of skin lesions.Study limitationsThis was a retrospective and small sample study.ConclusionsIn patients with myelogenous leukemia, skin infiltration usually occurs after, but occasionally before, the appearance of hemogram and myelogram abnormalities, and the presence of skin infiltration is often associated with a poor prognosis and short survival time. myeloid leukemia cutis often presents as generalized or localized nodules or masses with characteristic pathological and histochemical findings.     

以皮疹为首发症状的皮肤白血病一例

报道1例以皮疹为首发症状的急性淋巴细胞白血病.患者女,31岁,胸、腹部红斑伴轻度疼痛3个月就诊.初诊时血常规正常,皮疹活检提示皮肤白血病.1个月后原有红斑上出现瘀点,外周血及骨髓细胞学检查确诊为急性淋巴细胞白血病,腹部皮损免疫组化标记与外周血流式细胞仪分析,证实瘤细胞来源于前驱B淋巴细胞.脑脊液检查示中枢神经系统受累.化疗两次后缓解,1个月后复发,并死于呼吸循环衰竭.     

Leucemia cutánea en un paciente con leucemia mieloide aguda M2

—The leukemia cutis is the cutaneous infiltration by leukemics cells. Patients with acute myelogenous leukemia (AML) present specific cutaneous involvement in approximately 10% of the cases.We report the case of a leukemia cutis in a 73-year-old male with AML-M2. The patient presented with a one-week history of general malaise, asthenia, cough, dysnea and fever. Physical examination revealed and indurated red-brown plaque on his back of two moths duration, which was pruritic. Concomitance blood and bone marrow findings were diagnostic of AML-M2. A cutaneous biopsy was consistent with leukemia cutis. A CT and a bronchial biopsy showed pulmonary involvement. Since starting chemotherapy the patient had complete remission and the cutaneous lesion cleared, but 15 days later the skin lesion reappeared. A new bone marrow examination revealed recurrent leukemia. He died one month later.     

Leukemia Cutis Presenting with Fingertip Hypertrophy

Background: Patients with leukemia often manifest cutaneous findings, which include nonspecific lesions and specific leukemic infiltrates termed leukemia cutis. Objective: A case of leukemia cutis involving distal finger pads is reported and literature describing hand involvement of specific leukemic infiltrates is reviewed. Methods and Results: An 80-year-old woman with a 10-year history of chronic lymphocytic leukemia developed painful symmetric tumors of her distal finger pads. Histopathological examination of the biopsy specimen revealed infiltration by neoplastic lymphocytes. Only a few cases of leukemia cutis involving the hands have been reported in the literature, none with this particular presentation. The clinical and histopathologic features of leukemia cutis are reviewed. Conclusion: This case emphasizes the importance of obtaining a biopsy specimen for histopathological examination of any suspicious skin lesion in a patient with leukemia.     

Immature myeloid precursors in chronic neutrophilic dermatosis associated with myelodysplastic syndrome

Sweet syndrome (SS) associated with myeloproliferative disorders has been considered an inflammatory process mediated by neutrophils in which immunologic mechanisms are operative. The authors report the case of a 68-year-old man suffering from a myelodysplastic syndrome, who presented with a relapsing skin eruption resembling SS. Histopathologically, the skin infiltrates showed prominent neutrophilic features masking the underlying malignant process. Extensive immunophenotypic studies of skin revealed the presence of a few immature myeloid cells intermingled with an overwhelming infiltrate of neutrophils. The atypical cells in the skin had a phenotype identical to that of leukemic cells in the peripheral blood and bone marrow. Whether or not immature myeloid cell precursors constitute a specific infiltrate of leukemia cutis or are a result of recruitment of circulating leukemic cells to this area of inflammation is discussed.