Leukemia cutis in a patient with acute monocytic leukemia presenting as unique facial erythema

A 67‐year‐old woman was referred to our department with a 1‐month history of facial exanthemas. She had been diagnosed as having acute monocytic leukemia (French–American–British classification, M5b) based on the histological findings of bone marrow. Physical examination revealed diffuse edematous erythema on her cheeks, eyelids and glabella with scattered reddish papules. Histological examination demonstrated dense infiltration of atypical mononuclear cells in the dermis. Specific cutaneous lesions could occur in acute monocytic leukemia more frequently than in other types of leukemia, but rarely show symmetrical edematous erythema limited to the face.     

急性淋巴细胞性皮肤白血病

报告1例急性淋巴细胞性皮肤白血病.患者女,17岁.右胫前肿块2个月余,右大腿出现紫红色结节、躯干出现紫红色浸润性斑块1个月余,偶有疼痛和瘙痒.血常规检查正常.皮损组织病理榆查示真皮浅层有一狭窄无浸润带,在真皮和皮下组织可见大小不一的单一核细胞浸润,部分胞核较大,深染,偶见核丝分裂.免疫组化染色结果示CD3、CD4、CD5、CD8、CD20、CD79、MPO标志均为阴性,CD68阳性,且TdT标记30%～40%单一核细胞为阳性,提示为淋巴细胞前体细胞,结合骨髓细胞学检查诊断为急性淋巴细胞白血病(ALL-L2).给予患者DOCP(吡柔比星、地塞米松、环磷酰胺及长春新碱)方案化疗2个疗程后,胫前肿块缩小变平,身体其他部位皮损均消退.     

A case of symmetrical leukemia cutis on the eyelids complicated by B-cell chronic lymphocytic lymphoma

We describe herein a case of symmetrical bilateral leukemia cutis on the eyelids, especially on the lower lids, in a patient with B-cell chronic lymphocytic leukemia (CLL). The patient was a 63-year-old Japanese man who had been diagnosed with CLL. Eyelid swelling is well known to result from local skin or systemic diseases including tumors, hyperthyroidism, nephritic syndrome, and hypoalbuminemia. To our knowledge, symmetrical, bilateral, flesh-colored leukemia cutis on the eyelids has not been previously reported.     

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.     

皮肤白血病1例

报告1例以皮肤为首发表现的急性粒单细胞白血病。患者女,63岁。因面部、颈部、胸部斑块6个月,发热3个月就诊,根据临床和实验室检查确诊为急性粒单细胞白血病。本例特点是首先出现白血病的特异性皮肤表现（皮肤白血病）。     

Leukemia cutis - epidemiology, clinical presentation, and differential diagnoses

Leukemia cutis is an extramedullary manifestation of leukemia. The frequency and age distribution depend on the leukemia subtype. The clinical and morphological findings have a wide range of cutaneous manifestations and may present with nodular lesions and plaques. Rare manifestations include erythematous macules, blisters and ulcers which can each occur alone or in combination. Apart from solitary or grouped lesions, leukemia cutis may also present with an erythematous rash in a polymorphic clinical pattern. Consequently, leukemia cutis has to be distinguished from numerous differential diagnoses, i. e. cutaneous metastases of visceral malignancies, lymphoma, drug eruptions, viral infections, syphilis, ulcers of various origins, and blistering diseases. In the oral mucosa, gingival hyperplasia is the main differential diagnosis. The knowledge of the clinical morphology is of tremendously importance in cases in which leukemia was not yet known.     

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.     

Dermoscopic findings in neonatal leukemia cutis

Leukemia cutis is the direct infiltration of cutaneous tissues by leukemic cells and can present as a blueberry muffin baby. We present a case of neonatal leukemia cutis highlighting its dermoscopic features, the presence of fine telangiectatic arborizing vessels distributed within a pink background within all lesions.     

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.     

急性髓细胞性白血病(M4型)

报告1例急性髓细胞性皮肤白血病(M4型).患者女,48岁.全身出现丘疹、红色结节14d,伴剧烈瘙痒.体格检查:全身泛发大小不等的红色丘疹、结节,质韧,无压痛.皮损组织病理检查:真皮内弥漫淋巴样细胞浸润,有明显异形及较多核分裂象.免疫组化组织病理检查:CD68阳性(灶性),MPO阳性(少量).骨髓穿刺:白血病细胞大量增生,免疫标记:CD68、CD11b、MPO及HLA-DR均阳性.诊断:急性髓细胞性白血病(M4型).患者经过2次DA(伊达比星、阿糖胞苷)方案化疗后,再次行骨髓穿刺示缓解,但皮损仍有复发.     

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.     

Concurrent cutaneous localization of Langerhans cell sarcoma and chronic lymphocytic leukemia/small lymphocytic lymphoma in a patient with a history of chronic lymphocytic leukemia/small lymphocytic lymphoma

The phenomenon of histiocytic/dendritic cell sarcomas arising through transformation of a pre-existed lymphoproliferative disease is called transdifferentiation. Langerhans cell sarcoma transdifferentiating from chronic lymphocytic leukemia/small lymphocytic lymphoma is extremely rare and all the reported cases were localized in lymph nodes. We present a case of concurrent cutaneous localization of Langerhans cell sarcoma and chronic lymphocytic leukemia/small lymphocytic lymphoma, in which the chronic lymphocytic leukemia/small lymphocytic lymphoma preceded the development of the Langerhans cell sarcoma. A cutaneous lesion from a 63-year-old patient with a history of chronic lymphocytic leukemia/small lymphocytic lymphoma was biopsied. The histologic examination revealed a mixture of two cell populations infiltrating diffusely the dermis. The first was composed of small lymphoid cells with somewhat monotonous appearance and mild nuclear atypia positive for PAX5, CD79a, CD20, CD23, CD5, and LEF1. The second was composed of large cells with abundant cytoplasm and pleomorphic nuclei. These cells were positive for CD1a, CD207, and S100 protein and exhibited a high mitotic rate and a high MIB-1 immunostaining index. Therefore, two different entities, chronic lymphocytic leukemia/small lymphocytic lymphoma and Langerhans cell sarcoma, were detected in the same skin fragment. The patient died 3 years after initial diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma.     

Leukemia cutis in B-cell chronic lymphocytic leukemia presenting as an episodic papulovesicular eruption

A 53-year-old man presented with a recurrent pruritic eruption accompanied by oral sores. His past medical history was significant for subclinical B-cell chronic lymphocytic leukemia (CLL), which had never been treated. On exam, there were erythematous papules and plaques studded with vesicles on the neck, trunk, and upper extremities. Two skin biopsies showed common features of a perivascular and periadnexal lymphocytic infiltrate in the superficial to mid-dermis. Immunohistochemical staining of the lymphocytes showed co-expression of CD20, CD23, CD5, and CD43, consistent with a diagnosis of cutaneous involvement by the patient's CLL. This case highlights the importance of considering leukemia cutis in patients with underlying CLL presenting with unusual clinical features.     

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.     

Leukemia cutis originating in the extravasation site of i.v. gabexate mesilate infusion

Leukemia cutis is a localized or disseminated skin infiltration by leukemic cells. A 64-year-old man was diagnosed with acute myeloid leukemia (AML) complicated by disseminated intravascular coagulation. During the course of treatment with gabexate mesilate, the substance accidentally leaked from the infusion site in his elbow. One month later, a dark red erythema and induration accompanied by severe pain appeared in the area proximal to the gabexate mesilate injection site. The biopsy specimen demonstrated not only inflammation but infiltration of leukemic cells as well. Immunohistochemical staining for intercellular adhesion molecule-1 and platelet/endothelial cell adhesion molecule-1 showed strong expression of endothelial cells and leukemic cells. We speculate that the gabexate mesilate might have played a role in the induction of leukemia cutis via adhesion molecules in our case.     

Angioinvasive Fusarium and concomitant Enterococcus infection arising in association with leukemia cutis

Leukemia cutis represents the cutaneous infiltration of neoplastic leukocytes or their precursors that results in clinically identifiable skin lesions. For patients with myelodysplastic syndrome, developing such a lesion may indicate impending leukemic transition. These patients are also often immunocompromised, putting them at risk for infection by opportunistic fungal pathogens such as Fusarium. We describe an 85-year-old man with myelodysplastic syndrome who presented with a reddish-purple nodule with a surrounding erythematous plaque on his shin. Histopathologic examination revealed a dense diffuse infiltrate of large atypical cells in the reticular dermis, with ulceration and necrosis. Immunohistochemical studies showed positive staining with CD15, CD68 and myeloperoxidase of constituent large cells. Concurrently, there were branching and septate hyphae with occasional macroconidia-like structures throughout the infiltrate. Cultures from this lesion grew Fusarium and Enterococcus, supporting the diagnosis: leukemia cutis with superinfection involving both Fusarium and Enterococcus. To our knowledge, this is a novel report of two separate infections occurring in a lesion of leukemia cutis. This case shows that in patients with a hematologic malignancy and skin lesions, a high index of suspicion for infection is necessary when reviewing both the clinical and histopathological data to avoid overlooking an important occult infectious agent.     

Successful treatment of leukaemia cutis with cladribine in a patient with B-cell chronic lymphocytic leukaemia

Cutaneous presentation of B-cell chronic lymphocytic leukaemia (B-CLL) is uncommon, and the influence of skin changes on B-CLL prognosis is unclear. We report a patient with B-CLL Rai II, with multiple nodular skin infiltrations on the trunk, upper arms and thighs as well as constitutional symptoms, who was successfully treated with cladribine. The peripheral blood (PB) lymphocytes were CD19, CD20, CD23 and CD5 positive, which confirmed the diagnosis of B-CLL. Skin biopsy of one of the lesions showed an intense infiltrate composed of small lymphocytes with no epidermotropism. These cells also showed the expression of CD19, CD20, CD23 and CD5 antigens similar to those presented on PB lymphocytes. Polymerase chain reaction performed on bone marrow lymphocytes and a lesional skin biopsy using consensus primers for immunoglobulin heavy-chain genes also showed the same monoclonal population of B lymphocytes both in the bone marrow and in the skin. The patient received four courses of cladribine 0.12 mg kg-1 daily as a 2-h infusion for five consecutive days. The courses were repeated at monthly intervals. The lymphocytosis gradually decreased and the PB count normalized after three courses. At the same time, a significant decrease in the cutaneous symptoms was observed. The patient became free of skin tumours after the fourth course of cladribine; only slight discoloration at the previous sites of cutaneous infiltration remained. There was no relapse of leukaemia cutis during a further 7 months of observation.