Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Five cases of malignant histiocytosis are reported. They associate specific cutaneous lesions with the other much more classical symptoms of the illness; fever, weight loss, lymphadenopathy, splenomegaly, hepatomegaly. The principal clinical and histological findings observed during the course of the illness in these patients are described. In a review of the literature concerning this rare but now well individualized illness, 34 cases of malignant histiocytosis associated with cutaneous lesions are found among which 24 were studied on a clinical and histological point of view. The principal characteristics of the cutaneous eruption are presented: clinical characteristics with the most frequent nodular aspect of the lesions, but also histological features with the relative specificity of the disposition of the infiltrate whose cytological features will not be forgotten to analize. Nevertheless, the diagnostic will often be established by the histological analysis of the lymph node biopsy or sometimes of biopsy of the other affected organs. These cutaneous lesions might also be in several cases of some prognostic interest.
Ann Dermatol Venereol 1979 May
PMID:[Cutaneous lesions of malignant histiocytosis. About five cases (author's transl)]. 49 10

The infant of a mother with systemic lupus erythematosus (SLE) developed an extensive cutaneous eruption at 5 weeks of age. Biopsy findings were consistent with cutaneous lupus erythematosus (LE). Splenomegaly, anemia, neutropenia, and depressed total hemolytic complemtnt levels were additional findings. The course was benign, and all manifestations disappeared by 4 months of age. Fifty-two previously reported infants with cutaneous lesions, congenital atrioventricular heart block, or hematologic manifestations of neonatal LE are reviewed.
J Am Acad Dermatol 1979 Nov
PMID:Neonatal lupus erythematosus. 51 88

The records of ten patients with Lucio's phenomenon showed clinical and histopathological changes similar to those described by others. Lucio's phenomenon is a syndrome distinct from erythema nodosum leprosum as indicated by an absence of fever, leukocytosis and tenderness, a failure to respond to thalidomide, and a restriction to patients with diffuse nonnodular lepromatous leprosy. Lymphopenia associated with splenomegaly in three patients and glomerulonephritis in one patient were unexpected findings of unknown relevance.
Arch Dermatol 1978 Jul
PMID:Lucio's phenomenon and diffuse nonnodular lepromatous leprosy. 68 19

Systemic lupus erythematosus (SLE) developed in as 23-year-old woman with psoriasis during treatment with psoralen-ultraviolet-A (PUVA). The connective tissue disease was characterized by an erythematous rash, hair loss, nephritis, splenomegaly, seizures, and coma. Serum antinuclear antibodies were present in high titer, and hypocomplementemia developed. Antibodies to native or ultraviolet-irradiated DNA were not demonstrated. While the association of psoriasis and lupus may have been fortuitous, the temporal relationships suggest that PUVA treatment in this case may have been of pathogenetic importance in the development of the connective tissue disease.
Arch Dermatol 1979 Jan
PMID:Systemic lupus erythematosus: association with psoralen--ultraviolet-A treatment of psoriasis. 76 Jun 58

A typical case of congenital erythropoietic porphyria in a 8 month old girl is reported (photosensitive blistering syndrome, hypertrichosis; erythrodontia; fluorescence of urine, teeth, erythrocytes and myeloblasts; splenomegaly; anaemia). Porphyrins, isomers I, were present in urine and blood. In faeces a porphyrin-X-like substance migrating as PX was detected; this porphyrin was finally identified as CP I unusually difficult to extract because of the presence of a banding and fluorescence quenching agent probably of exogenous origin. The usual methods are not always able to extract and to identify the faecal porphyrins.
Ann Dermatol Venereol 1977 Jan
PMID:[Gunther's congenital erythropoietic porphyria in an 8-month old girl (author's transl)]. 84 24

Four patients presented with a clinical picture of spiking fever, erythematous nodular subcutaneous skin lesions, anaemia and leucopenia. Inconstant features were weight loss (3/4), splenomegaly (3/4), thrombocytopenia (3/4), raised liver enzymes (3/4), hepatomegaly (2/4) and serosal effusions (2/4). The histopathological findings of lobular histiocytic panniculitis with 'bean-bag' cells were characteristic of cytophagic histiocytic panniculitis (CHP). No infectious agent could be detected, and there was no histological evidence of malignant neoplasia. Unlike most previously described patients with CHP, three of our four patients responded to treatment with immunosuppressive or cytostatic drugs.
Br J Dermatol 1992 Dec
PMID:Cytophagic histiocytic panniculitis: a report of four cases. 128 61

Cutaneous T-cell lymphoma is a malignancy of T cells that express a clone-specific heterodimer T-cell receptor for antigen. The second recognized case of an epidermotropic malignancy of T-cells expressing gamma/delta T-cell receptor-expressing cells is reported. The immunophenotype of the malignant T-cells was CD3+, CD2+, CD7+, gamma/delta T-cell receptor+, CD4-, CD8-, and alpha/beta T-cell receptor-. The clinical features were remarkable for extreme epidermotropism with a scant dermal lymphomatous infiltrate. Profound keratinocyte necrosis occurred in areas of malignant skin infiltrates. Despite cutaneous lesions covering more than 50% of the skin surface of the patient, no adenopathy or splenomegaly was detected. The intense epidermotropism of this patient's gamma/delta T-cell receptor-expressing cells and the putative cytolytic properties of CD4- CD8- gamma/delta contributed to the destruction of epidermis. Remission was induced with a combination of electron beam and extracorporeal photochemotherapy.
J Am Acad Dermatol 1992 May
PMID:Correlations of unique clinical, immunotypic, and histologic findings in cutaneous gamma/delta T-cell lymphoma. 137 10

The ability of strains and fractions of killed propionibacteria suspensions to produce chronic rat ear inflammation after intradermal injection of 70-micrograms aliquots was highly correlated with production of splenomegaly in the mouse after i.p. injection of 1.4 mg Propionibacterium acnes strains CN 6134, VPI 0009, ATCC 11828, and UCLA SC and N1 produced a 2- to 3-fold increase in rat ear thickness and a 5- to 7-fold increase in mouse spleen weight 15 days post injection. In contrast P. granulosum CN 5888, P. acnes UCLA 6S and periodated, acetylated, or 12-h cultures of VPI 0009 were inactive or weakly active as stimulators of chronic ear inflammation and splenomegaly. Active strains produced in the rat ear a transepidermal elimination response characterized by follicular encapsulation and the formation of secondary comedones. These effects correlated with persistence of phagocytized bacteria within macrophages. Furthermore, when rats were first immunized and then challenged with active strains of P. acnes, an increased sensitivity to low doses of P. acnes and a chronic exacerbation of inflammation was observed.
J Invest Dermatol 1985 Sep
PMID:Acne-like chronic inflammatory activity of Propionibacterium acnes preparations in an animal model: correlation with ability to stimulate the reticuloendothelial system. 316 57

Prolidase deficiency occurred in two sisters suffering from recurrent leg ulcers that appeared in early childhood. The patients presented the typical clinical symptoms of the disease, including characteristic facies, dermatologic manifestations of the lower extremities, splenomegaly, and hematologic anomalies. Large amounts of iminodipeptides were excreted into the urine, and prolidase activity in their erythrocytes was virtually absent. Changes associated with a connective-tissue disorder were demonstrated by light and electron microscopic studies of the patients' apparently normal skin. Collagen fibers were smaller than in controls and were irregularly packed; the fibrils had normal aspect but were significantly smaller than in one age-matched control. Elastin fibers appeared altered both in size and structure.
Arch Dermatol 1987 Apr
PMID:Prolidase deficiency in two siblings with chronic leg ulcerations. Clinical, biochemical, and morphologic aspects. 382 81

A 7-year-old girl had fever, arthralgia, and a mild cutaneous vaculitis with papules, nodules, and livedo. A biopsy specimen of a papule showed lymphocytic small-vessel vasculitis, with some atypical lymphocytic nuclei. Splenomegaly developed, and results of subsequent studies supported a diagnosis of cytomegalovirus (CMV) infection, with atypical peripheral blood lymphocytes and a characteristic pattern of complement-fixing antibodies to CMV antigen. The CMV mononucleosis syndrome is rarely reported in children, and the cutaneous manifestations are usually rubelliform. Distinctive cutaneous pathologic characteristics have been described previously only in neonates ("blueberry muffin" syndrome) and in immunosuppressed patients (viral inclusion bodies in endothelial cells).
Arch Dermatol 1980 Oct
PMID:Vasculitis in cytomegalovirus infection. 625 54


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