Remission of Langerhans cell histiocytosis with thalidomide treatment.
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Aged↗
Biomedical subjects
L Misery
Publications and source records attributed to L Misery.
Multiple juvenile xanthogranulomas without foam cells and giant cells.
A 4-month-old boy had numerous nodular lesions. Microscopic findings revealed a histiocytic proliferation without foam cells and giant cells. Immunohistochemical studies showed that the histiocytes belonged to the monocyte-macrophage system and not to the Langerhans cell system. This case is an unusual variant of juvenile xanthogranuloma that, when congenital, may be difficult to differentiate from Hashimoto-Pritzker disease.
Histiocytes↗
S100 protein and neuron-specific enolase on monocytic leukemic CD1+ cells, probable precursors of Langerhans cells.
Langerhans cells are characterized by specific markers, such as Birbeck granules and CD1a antigen. S100 protein and neuron-specific enolase are less specific but are expressed only on Langerhans cells among the cells of the phagocytic mononuclear system. In this study, the expression of these two antigens on many monocytic leukemic cells is shown. These cells could be precursors of Langerhans cells which have been transformed into malignant cells.
Antigens, CD↗
BCG-vaccine-induced lupus vulgaris and urticarial vasculitis.
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Adult↗
[Systemic urticaria disclosing post-vaccine lupus tuberculosis].
The authors report a case of association between lupus vulgaris and urticarial vasculitis after BCG vaccination. Lupus vulgaris is a rare classical complication of BCG vaccination. Anaphylactoid reactions after BCG are very rare. This is the first report of association between the two diseases after BCG and the third report of urticaria after BCG. The pathogenesis of this is discussed.
Adult↗
CD1-reactive leukemic cells in bone marrow: presence of Langerhans cell marker on leukemic monocytic cells.
Langerhans cells originate in bone marrow and probably belong to the monocyte-macrophage lineage. CD1 is a specific marker of Langerhans cells. By immunofluorescence and immunoelectron microscopy, CD1a antigen and myeloid markers (CD11, CD13, CD14, CD15, CD33, HLA-DR) were studied in 53 cases of acute myeloid leukemias (AML) and 3 acute lymphoblastic leukemias (ALL). The 11 ANLL without monocytic component were CD1a negative. 2/5 of acute myelomonocytic leukemias (AML4) and 9/37 of acute monocytic leukemias (AML5) were positive. All 3 ALL were negative. No correlation was found between CD1a and myeloid markers. CD1a+ AML did not differ from CD1a- AML with regard to cytogenetics or response to therapy. The CD1a positive cells may originate from an abnormal proliferation of CD1a positive cells which are present in bone marrow and which may differentiate into Langerhans cell precursors.
Antigens, CD↗
[In virtuo model for research on allergic reaction].
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Computational Biology↗
Expression of PGP9.5 on Langerhans' cells and their precursors.
Langerhans' cells are epidermal dendritic cells, derived from blood precursors. Their main function is antigen presentation to T-cells. They are able to express neuronal proteins, such as neuron-specific enolase or substance P-receptor. They are closely associated with nerve fibres. PGP9.5 is the most specific neuronal protein in the epidermis. Epidermal Langerhans' cells can express PGP9.5 if denervated. Using flow cytometry, we found that cultured CD34+ precursors did not express PGP9.5, whereas suspensions of fresh or cultured Langerhans' cells could express this neuronal protein. Precursors of Langerhans' cells are not able to express PGP9.5, suggesting that they are not mature enough or that the capacity to express PGP9.5 may be acquired only in the epidermis. The function of PGP9.5 on Langerhans' cells and mature dendritic cells remains unknown. PGP9.5 might be related to dendritic cell maturation or to the lack of contacts with nerve endings.
Antigens, Differentiation↗
[Localized epidermal necrolysis after intravenous injection of vinorelbine].
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Antineoplastic Agents↗
[Papulo-erythematous eruption of neonatal lupus].
INTRODUCTION: Neonatal lupus is rare and cutaneous lesions are usually suggestive of the diagnosis. We describe the case an infant with atypical clinical and histological aspects. CASE REPORT: A female newborn, 6 weeks of life, presented since 3 weeks a papulo-erythematous eruption involving the trunk and the 4 limbs. Cutaneous biopsy showed a dermal infiltrate of mononuclear cells, which corresponded morphologically to lymphocytes. But myelo-monocytic origin was proved by immunostaining. This result could be seen in hematodermia and macrophagic activation syndrome, but we had no clinical sign for these diagnosis. Later, she presented an erythema of the eyelids and erythematous papules of the face leading to suspicion of neonatal lupus. Questioning the mother revealed that she had Raynaud's syndrome since 1995. Antinuclear factors were positive in the patient and her mother, corresponding to antiSSA and antiSSB antibodies. She exhibited cytolytic hepatitis but no congenital heart block. Direct immunofluorescence was negative. The clinical evolution was good with complete clearing of the eruption at the age of 3 months. At 10 months, clinical and biological examinations were normal, with no arguments for haematological disease. DISCUSSION: This observation is original because of the initial papulo-erythematous eruption which is rare in neonatal lupus. Diagnosis was confirmed when specific secondary cutaneous lesions appeared. The biopsy of the first eruption showed a myelo-monocytic infiltrate which has never been described in neonatal lupus. However cutaneous biopsies are rarely performed in neonatal lupus and immunostaining is lacking in the literature.
Age Factors↗
[Subcutaneous mycosis due to Scopulariopsis brevicaulis in an aplasic patient].
INTRODUCTION: Among the filamentous fungi of the Hyphomycete class, Annellospore group, the Scopulariopsis genera presently includes 16 species considered as opportunist pathogens. We report some unusual cutaneous manifestations due to Scopulariopsis brevicaulis in an immunodepressed patient. OBSERVATION: A 67 year-old man presented with a lymphomatous form of Waldenström's macroglobulinemia. During chemotherapy with medullar aplasia, inflammatory cutaneous nodules appeared on the forearm and leg. Microbiological and histological examinations identified Scopulariopsis brevicaulis. The patient improved with rapid remission from aplasia using hematopoietic growth factors and treatment with itraconazole. COMMENTS: In a patient in medullar aplasia, the rapid onset of an extra-ungual cutaneous localization (forearm and leg) of a Scopulariopsis brevicaulis infection, without systemic involvement, suggests that the cutaneous inoculation occurred through scratching (from the nails) or from another cutaneous origin. Prolonged neutropenia enhanced the pathogenicity of this mycosis. The mycological and histological examinations are fundamental to confirm the diagnosis.
Aged↗
Cutaneous manifestations of primary Sjögren's syndrome are underestimated.
The association of kerato-conjunctivitis sicca and xerostomia has been termed Sjogren's syndrome (SS). Although this disease is referred to as a non-organ-specific autoimmune condition, the vast majority of the deleterious effects of primary SS are restricted to the exocrine glands. Among them, the lacrymal and salivary glands are at the foreground, owing to the severity of the objective consequences and the importance of the subjective manifestations. As a result, cutaneous manifestations are minimized, albeit relatively common. We have carefully analyzed the literature to draw up an inventory of the possible skin complications of this syndrome. In addition to xerosis and epidermal IgG deposits, they include vasculitis and cutaneous B cell lymphoma. Alopecia, vitiligo and papular lesions have also been reported to be associated with primary SS.
Humans↗
Sweet's syndrome associated with cutaneous T cell lymphoma.
Although the association of Sweet's syndrome with haemoproliferative disorders or solid malignant tumors is well known, only two previous observations associated with T lymphoma have been reported. We report the first observation of Sweet's syndrome associated with cutaneous T cell lymphoma. The role of a cytokine cascade released by an abnormal T cell clone is discussed as an initiating event responsible for secondary infiltration with neutrophils.
Aged↗