Amyloidosis cutis dyschromica: a rare pigmentary disorder

Amyloidosis cutis dyschromica represents a rare type of primary cutaneous amyloidosis with few reported cases worldwide. It is characterized by asymptomatic, generalized hyperpigmentation with intermingled hypopigmented macules without atrophy or telangiectasia. We report herein a 19-year-old female who developed this pigmentary abnormality at 4 years of age. Her aunt and great grandfather also had similar skin pigmentation. An unusual finding in our patient was the presence of papules in addition to characteristic macules. Amyloid deposits were shown histopathologically in both dyschromic macules and papules.     

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.     

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.     

Calcinosis cutis: a rare feature of adult dermatomyositis

Dermatomyositis is an idiopathic inflammatory myopathy with characteristic cutaneous manifestations. We describe a case of a 55-year-old woman with dermatomyositis who presented with dystrophic calcinosis resistant to medical treatment.     

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.     

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.     

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.     

Myeloid leukemia cutis: a histologic and immunohistochemical review

Background:  The histologic diagnosis of myeloid leukemia cutis (LC) can be difficult, requiring confirmatory immunohistochemical stains.
Objective:  We reviewed 21 biopsy-proven cases of LC with emphasis on the use of immunohistochemistry in the diagnosis.
Materials and Methods:  Clinical and histologic features were reviewed on 21 cases of biopsy proven LC. Immunohistochemical stains for CD4, CD34, CD56, CD68, CD117, CD123, TdT, lysozyme and myeloperoxidase were performed on 12 with available tissue blocks.
Results:  Ages ranged from 24 to 88 years (mean = 57), with 12 men: 9 women. Primary hematologic diagnoses included acute myeloid leukemia (n = 14), myelodysplastic syndrome (n = 3), essential thrombocythemia (n = 1) and myeloid leukemia, NOS (n = 3). Monocytic myeloid LC was most common (35%). There was 100% positivity with CD68 and lysozyme. Myeloperoxidase, CD117 and CD34 immunostains were less sensitive in myeloid LC (58%, 33% and 17%, respectively). CD4 was positive in 67%. CD56 was positive in 33%.
Conclusion:  Myeloid leukemia with monocytic differentiation more commonly involves the skin than other types of myeloid leukemia. CD68 and lysozyme immunostains, although not lineage specific for monocytes/macrophages, are the most sensitive immunostains in the detection of myeloid LC. Myeloperoxidase immunostains are useful, but immunostains for CD117 and CD34 are insufficiently sensitive. CD4 expression is common, but CD56 expression is not.     

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.     

Acquired localized cutis laxa of the face: a rare presentation

We report a rare case of acquired localized cutis laxa in a teenage boy, without any preceding skin lesions. The area affected was the midface, extending to the chin, and involving the ears, leading to a prematurely aged appearance. Only five such cases have been previously published in the literature.     

Concurrent chronic lymphocytic leukemia cutis and acute myelogenous leukemia cutis in a patient with untreated CLL

Patients who have chronic lymphocytic leukemia (CLL) are known to have a high frequency of second malignant neoplasms. However, acute myelogenous leukemia (AML) occurring concurrent with or after a diagnosis of CLL is extremely rare. In this article we report a case of AML developing in a 55-year-old male with a 6-year history of untreated CLL. The diagnosis was facilitated by touch preparation of a skin punch biopsy specimen. The patient presented with a two-week history of fever, weakness, anasarca, and a skin rash. Physical examination revealed pink to skin-colored firm papules, which coalesced into indurated plaques on his trunk, upper extremities, and face. The lesions, in combination with generalized edema, produced a leonine facies. Touch prep of the biopsy showed medium to large blasts, large monocytoid cells, and numerous small mature lymphocytes, providing the preliminary diagnosis of a second, previously undiagnosed myelomonocytic malignancy in this patient. The initial diagnosis was subsequently confirmed by histologic, cytochemical, immunohistochemical and flow cytometry studies. This is the first reported case of CLL with concurrent AML in which rapid touch prep of a skin punch biopsy facilitated diagnosis.     

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.     

急性早幼粒细胞白血病所致皮肤白血病一例

患者男,28岁,因全身皮疹3天,发现粒细胞减少1天来本科门诊就诊.就诊前3天发现躯干、四肢、面部皮肤出现红丘疹伴轻度瘙痒,逐日增多,自觉乏力外,无其他不适,门诊查血常规WBC 1.5 × 10~9/L、PLT 79 × 10~9/L,以粒细胞缺乏症收住血液科.     

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.     

Cutaneous granulomatous vasculitis associated with chronic lymphocytic leukemia: case report and review of literature of a rare entity

Cutaneous granulomatous vasculitis associated with lymphoproliferative disorders is a rare entity, with only 14 cases previously reported in the English literature. Patients generally present with nodules or ulcers involving the extremities, which can appear months or years before or after onset of systemic disease. Granulomatous vasculitis has a poor prognosis when associated with underlying lymphoproliferative disorders, with the majority of reported cases fatal. Knowledge of this unusual entity is important to allow for proper clinical evaluation, follow-up, and therapy. We report a 77-year-old female with chronic lymphocytic leukemia and granulomatous vasculitis, which highlights the features of this association, and expands the clinical data.