Skin involvement in chronic myelomonocytic leukaemia as a predictor of transformation into acute myeloid leukaemia.

We report on the case of a patient with myelodysplastic syndrome (MDS) who presented with leukaemia cutis preceding development of acute myeloid leukaemia. Leukaemic infiltration of the skin should be considered an early manifestation of leukaemic transformation and an indicator of poor prognosis in MDS.     

Leukaemia cutis and leukaemic vasculitis

BACKGROUND: Leukaemia cutis (LC) and leukaemic vasculitis (LV) are uncommon but important features of haematological malignancy. OBJECTIVES: We aimed to evaluate clinicopathological features of a series of such cases. METHODS: Thirty-four patients with LC were studied. Nineteen had acute myeloblastic leukaemia, six had acute lymphoblastic leukaemia, three had myelodysplastic syndrome-refractory anaemia with excess blast in transformation, three had non-Hodgkin's lymphoma in leukaemic phase, two had chronic lymphocytic leukaemia and one had multiple myeloma. RESULTS: Cutaneous lesions were widely dispersed in 15 patients, but were restricted to the extremities in eight patients. In other patients lesions were localized on the face, hands and feet. Cutaneous lesions were generally erythematous, maculopapular, squamous, nodular, haemorrhagic or necrotic. Two localizations were very interesting: in one case, lesions were confined to the site of previous varicella zoster virus infection; in the other case, leukaemic infiltration occurred within a lesion of cutaneous leishmaniasis. All of the patients had high white blood cell count and/or other findings of high tumour burden. Vasculitis with leukaemic cell infiltration (LV) was detected in 24 patients, and was high-grade LV in 16 patients. Patients with high-grade LV tended to have a higher white blood cell count and other findings associated with an aggressive clinical course compared with patients not having LV. Thirteen patients with LV died within 1 month but five patients lived more than 1 year. CONCLUSIONS: LC, and especially LV, is generally an indicator of poor prognosis. Its pathogenesis must be evaluated further with additional studies, including adhesion molecules, angiogenic factors and other biological parameters.     

Aleukaemic leukaemia cutis as an initial manifestation of myeloid/NK cell precursor acute leukaemia

Aleukaemic leukaemia cutis is a rare condition characterized by infiltration of leukaemic cells into the skin before they appear in the peripheral blood. We report a case of an aleukaemic leukaemia cutis, which had a history of exposure to atomic bomb radiation. A 57-year-old Japanese woman initially presented with a 20-week history of multiple red papules and plaques mainly over the trunk. Histological examination revealed the infiltration of atypical monocytic cells in the dermis, but no leukaemic cells were detected in the peripheral blood. Twenty-three weeks after the appearance of the eruption, leukaemic cells were detected in the peripheral blood for the first time. The results of immunohistochemistry of the skin biopsy specimen and flow cytometry of the peripheral blood indicated the rare phenotype of myeloid/NK cell precursor acute leukaemia. This is the first case report of myeloid/NK cell precursor acute leukaemia presenting as aleukaemic leukaemia cutis in the English literature, and awareness of this clinical presentation may be important to reach the correct diagnosis.     

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.     

Mycosis fungoides and chronic lymphocytic leukaemia--composite T-cell and B-cell lymphomas presenting in the skin

Composite lymphomas involving cutaneous B-cell and T-cell lymphomas are very uncommon. We report here the unique circumstance of a patient with mycosis fungoides (primary cutaneous T-cell lymphoma) who later developed chronic lymphocytic leukaemia (B-cell lymphoproliferation, B-CLL), which presented in the skin (leukaemia cutis) as a composite lymphoma affecting an earlobe. The presence of both lymphoproliferative disorders was confirmed with immunophenotyping and the finding of both immunoglobulin gene rearrangements and T-cell receptor gene rearrangements in the ear and the same T-cell receptor gene rearrangement in a plaque lesion of mycosis fungoides on the arm.     

皮肤白血病特异性皮损与病情演变预后分析

分析了皮肤白血病特异性皮损与病情的演变预后，选择24例皮肤白血病患者（10例皮肤白血病为特异性皮损，14例皮肤白血病为非特异性皮损）进行临床，病理，实验室和细胞免疫学分类等综合分析，将有特异性皮损的患者病情演变过程及预后转归与未发生特异性皮损的皮肤白血病患者进行比较，并做统计学处理，结果白血病患者发生特异性皮损，病情处于进展加重状态，预后较未发生特异性皮损的白血病患者差，两者之间有显著性差异，表明皮肤白血病患者出现特异性皮损提示病情加重，肿瘤在广泛转移，有迅速发展死亡的危险，应及时有效治疗控制病情。     

Therapy-related leukaemia cutis: a review

Leukaemia cutis following chemotherapy for a malignancy is a multifactorial process that is dependent on the chemotherapeutic agent used, the dosing regimen, and the cumulative dose as well as potential contributing therapies such as radiation and possibly even hematopoietic support from granulocyte colony stimulating factor. In the right combination and in a patient with a conducive milieu of epigenetic factors, leukaemia can develop as a treatment complication. Leukaemia cutis is the specific infiltration of the skin by leukaemic cells and occurs most commonly when the underlying leukaemia is an acute myeloid leukaemia. Although it is well reviewed in the literature as a result of primary leukaemia, leukaemia cutis has only very rarely been reported in association with therapy-induced leukaemia. This article reviews the factors that contribute to therapy-related leukaemia and the development of leukaemia cutis.     

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.     

Cryotherapy in cases with leishmaniasis cutis

Aim/Objective To prove the effectiveness of cryotherapy in the treatment of leishmaniasis cutis in a large group of patients. Subjects Two hundred and sixty-six lesions of 227 patients with leishmaniasis cutis. Methods Cryotherapy with a double freeze-thaw cycle by a CS-76 apparatus and liquid nitrogen. Results All lesions responded well with cosmetically acceptable scars. The recurrance rate was only 4%. Conclusion Cryotherapy is an effective method of treatment of leishmaniasis cutis.     

Leukaemia cutis in a patient treated for breast cancer

A 47-year-old woman with a history of breast cancer presented with eruptive cutaneous nodules on the trunk and extremities. Treatment for her breast cancer had included surgery, radiation and chemotherapy with doxorubicin and cyclophosphamide. Biopsy of the skin lesions revealed leukaemia cutis, which led to the discovery of acute myelogenous leukaemia. This was felt to be primarily induced by doxorubicin. Treatment included induction chemotherapy in preparation for a bone marrow transplant, which resulted in the disappearance of the cutaneous lesions. However, the patient later succumbed to her leukaemia.     

Three cases of osteoma cutis occurring in infancy. A brief overview of osteoma cutis and its association with pseudo-pseudohypoparathyroidism

We report three cases of primary osteoma cutis in children, two of whom (siblings) were associated with Albright's hereditary osteodystrophy (AHO), manifesting as short stature with autosomal dominant inheritance from the father, but no dysmorphic features and no parathyroid hormone (PTH) resistance. Osteoma cutis can manifest as an isolated skin disease, a secondary condition to other skin diseases (such as acne), or in association with several syndromes, including AHO, which in turn may be associated with PTH resistance. The management and prognosis of patients diagnosed with osteoma cutis is determined by whether the skin manifestation has occurred in isolation, in association with a syndrome, or as a secondary skin disease. These three paediatric cases highlight the importance of understanding the aetiology and associations of osteoma cutis in order to appropriately investigate and manage patients who present with this rare skin disease.     

A Case of Congenital Leukemia Cutis

Congenital leukemia is a rare condition diagnosed at birth to 6 weeks of life with an estimated incidence of 4.7 per million live births. In a review, about 25~30% of cases were well documented as leukemia cutis. We report a case of a 3-month-old infant presented with asymptomatic multiple erythematous to bluish patches and nodules, which had developed about two months ago. Biopsy of the cutaneous lesions revealed immature cells which are overall monotonous with large kidney shaped nucleus, prominent nucleoli and moderate cytoplasm. These atypical cells stained positive for leukocyte common antigen, lysozyme and CD68. With this result, a congenital leukemia cutis was diagnosed. Six months later, she underwent cord blood stem cell transplantation. Seven months after the cord blood stem cell transplantation, the bone marrow examination revealed a normal result. Leukemia can be presented in the skin, the eruption may be nonspecific and it may precede systemic involvement. The recognition is important because early diagnosis can lead to appropriate treatment, followed by better prognosis.     

Multiple miliare Osteome des Gesichts

Osteoma cutis is a single or multiple ectope calcification with development of bony structures in the skin. We distinguish between primary and secondary ossification. Multiple miliary osteoma in the face has mostly been described secondary to preexisting acne vulgaris. We present a 62-year-old woman who developed multiple miliary osteoma in the face together with repeated doses of estrogen and discuss pathogenesis and therapeutic possibilities.     

Presence of varicella zoster virus in zosteriform leukaemia cutis

Leukaemia cutis is a relatively rare manifestation in chronic lymphocytic leukaemia, characterized by a diverse morphology of skin lesions. We report two patients who developed zosteriform skin symptoms; however, the histological analysis revealed leukaemia infiltration as the cause of their symptoms. Contrary to previous reports, varicella zoster virus DNA was detectable in the lesions. These findings suggest that varicella zoster virus plays an active role in the development of zosteriform leukaemia cutis.     

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.     

Autosomal recessive type 2 pseudoxanthoma elasticum presenting with generalized skin laxity

Herein, we describe a sporadic case of recessive type 2 pseudoxanthoma elasticum. A 26-year-old woman without family history presented with cutis laxa-like marked wrinkling involving the whole-body and a serpiginous streak on the upper left arm. She denied any other systemic problems related to difficulty with visual acuity or vascular disease. A skin biopsy specimen from the loose skin showed the accumulation of calcified degenerated elastic fibers and foci of ossification in the dermis. Histopathological study from a serpiginous streak revealed mineralized debris that was eliminated through the epidermis, the finding consistent with elastosis perforans serpiginosa. Recessive type 2 pseudoxanthoma elasticum is very rare and the presenting case is interesting in that this patient presented with lesions of secondary ossification and elastosis perforans serpiginosa in association with pseudoxanthoma elasticum.     

应用聚合酶链反应诊断疣状皮肤结核1例报告

报告１例４５岁女性疣状皮肤结核患者，病期１３年。于右臀部至右大腿后上方、右耳廓及右胸部呈现肉芽肿性皮损。组织病理疑为结核，抗酸染色（一）。遂将石蜡切块作聚合酶链反应（ＰＣＲ）检查，扩增出结核杆菌特异ＤＮＡ片段。经抗痨治疗皮损治愈。     

Cutaneous leukaemic infiltrations in a patient with previously undiagnosed myelodysplastic syndrome

We report the rare case of a patient with leukaemia cutis first presenting only on the hand and fingers and then subsequently spreading over the trunk and face. The lesions heralded the transformation of a previously undiagnosed myelodysplastic syndrome type RAEB (refractory anaemia with blast excess) into frank myeloid leukaemia. The haematological disease was first detected by the dermatohistopathologist. This case underlines the need to look meticulously for skin changes and perform early skin biopsies in haematological patients, as the skin can reveal the first clinical signs of an otherwise not evident bone marrow disorder. Leukaemia cutis as the initial clinical presentation of a transforming myelodysplastic syndrome type RAEB into acute myeloid leukaemia has been reported only very rarely.     

Solitary Milialike Idiopathic Calcinosis Cutis: A Case Unassociated with Down Syndrome

We report a unique case of solitary milialike idiopathic calcinosis cutis (MICC) in a healthy Korean woman, which is not associated with Down syndrome. This case of MICC would be a form of idiopathic calcinosis cutis, which can be solitary or multiple, sporadic or associated with Down syndrome.     

疣状皮肤结核患者皮损中结核杆菌DNA的检测

本文采用PCR技术，对10例病史2年以上的疣状皮肤结核患者皮损进行了结核杆菌DNA检测，结果显示10例患者1例阳性，而作为阳性对照的4例淋巴结结核组织3例阳性，2例正常组织均为阴性。从而说明疣状皮肤结核后期组织结核菌极少或无有。