Aleukemic congenital leukemia cutis

A newborn girl had typical "blueberry muffin" skin lesions, which showed histopathologic features of myelomonocytic leukemia cutis. We could not demonstrate leukemic infiltration of bone marrow in four aspirates. Her course was complicated with primary pulmonary hypertension, which led to death at 7 months of age. We emphasize the persistence of skin lesions in the absence of bone marrow infiltration by leukemia throughout the course of the disease.     

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.     

Spontaneous regression of a congenital melanocytic nevus

Congenital melanocytic nevus (CMN) may rarely regress which may also be associated with a halo or vitiligo. We describe a 10-year-old girl who presented with CMN on the left leg since birth, which recently started to regress spontaneously with associated depigmentation in the lesion and at a distant site. Dermoscopy performed at different sites of the regressing lesion demonstrated loss of epidermal pigments first followed by loss of dermal pigments. Histopathology and Masson-Fontana stain demonstrated lymphocytic infiltration and loss of pigment production in the regressing area. Immunohistochemistry staining (S100 and HMB-45), however, showed that nevus cells were present in the regressing areas.     

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

A 46-year-old man presented with nodular skin lesions, a biopsy specimen of which demonstrated a poorly differentiated malignancy. Touch preparations with histochemical staining and electron microscopy confirmed leukemia cutis. Results from a bone marrow aspirate disclosed focal areas of increased myeloblasts, and cytogenetic analysis revealed an abnormal karyotype as follows 46,XY, t(1;2) (q44p13). Antileukemic therapy resulted in prompt disappearance of the skin nodules, and a return of the patient's bone marrow to normal was noted, but after six months of intensive chemotherapy leukemia cutis recurred without morphologically identifiable leukemia in the bone marrow. The patient underwent successful bone marrow transplantation from an HLA-identical sibling but died 70 days after the transplant from disseminated aspergillosis.     

Congenital leukemia cutis

Abstract:  We describe a premature neonate who was born with pancytopenia and a single subcutaneous nodule on her right lower extremity. A biopsy specimen from the nodule demonstrated a dense infiltrate of pleomorphic mononuclear cells that extended throughout the dermis and into the subcutaneous tissue. Immunohistochemical stains and bone marrow examination confirmed a diagnosis of acute myelogenous leukemia. Cytogenetic studies on peripheral blood by G-banding analysis revealed an abnormal karyotype of 46, XX, ins[inv(10)(p11.2q22.2);11](q22.2;q13.2q23.2). A split in the mixed lineage leukemia gene was identified by fluorescence in situ hybridization. Induction chemotherapy was started but was complicated by multiorgan failure. The patient died on the eleventh day of life. As leukemia cutis more typically presents as multiple infiltrative papules, nodules, or plaques, we stress the importance of including leukemia in the differential diagnosis of a solitary nodule in a neonate.     

Chilblain-like leukemia cutis

We present a 6-year-old girl with an 8-month history of fluctuating chilblain-like lesions on the toes. A full blood count showed slight thrombocytopenia and monocytosis. A skin biopsy specimen showed a dense perivascular and superficial dermal cellular infiltrate which was CD3(-), CD43(+), and lysosyme + on immunocytochemistry, suggesting a monocytic origin. Juvenile myelomonocytic leukemia was diagnosed after a bone marrow biopsy and spontaneous marrow colony growth in culture.     

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.     

Monocytic aleukemic leukemia cutis

Congenital poikiloderma is an uncommon hereditary disorder. It has been reported in association with various syndrome. No case has previously been reported from this environment. We report a case of congenital poikiloderma in a two and a half year old female Nigerian associated with unusual generalised hypopigmentation and acral blisters at birth. The child subsequently developed macular hyperpigmentation on an erythematous background and atrophy of the skin. Although she had some features which were suggestive of Rothmund-Thomson syndrome (RTS), the presence of hypopigmentation at birth, along with acral blistering, was noted to be peculiar to this child. We, therefore, feel that this case presents a distinct variant of congenital poikiloderma that has not been described previously.     

Aleukemic leukemia cutis.

Specific leukemic cutaneous infiltrates preceded observable leukemic cells in the blood in a case of acute lymphoblastic leukemia. Examination of a cutaneous biopsy specimen led to early diagnosis of the disease.     

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。     

Spontaneous regression of congenital melanocytic nevi without evidence of the halo phenomenon

Spontaneous regression of a congenital melanocytic nevus is rare and has always been thought to be associated with a halo phenomenon, suggesting that an immunological mechanism is involved in the regression process. We describe herein 2 cases of complete congenital melanocytic nevus regression without the halo phenomenon in 2 Japanese girls. To our knowledge, such a clinical progression has not been previously reported. We will also discuss several different possible mechanisms of regression.     

皮肤白血病1例

患者男性，５７岁，病期１个月，患者先出现乏力、发热、面色苍白，半月后于颈部、双上肢发现皮疹，无自觉症状。右前臂结节组织病理报告为皮肤白血病，经骨髓检查及免疫单抗检测确诊为急性粒细胞性白血病Ｍ２型。化疗后１７天，皮疹完全消退。１个月后，皮疹又再次出现。     

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）。