Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 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

A 10-month-old male infant had keratosis follicularis spinulosa decalvans, an X-linked dominant disorder. His cutaneous abnormalities consisted of generalized hyperkeratosis, spiny follicular papular lesions, universal alopecia, and hypoplastic nails. Ocular changes characteristic of the disease were also present. Unusual findings included deafness, failure to thrive, predisposition to bacterial infections without demonstrable immune defect, and transient hepatomegaly with abnormal liver function studies.
Arch Dermatol 1978 May
PMID:Keratosis follicularis spinulosa decalvans. An infant with failure to thrive, deafness, and recurrent infections. 64 99

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

A male patient presented with a congenital ichthyosis clinically characterized by generalized erythroderma, fine scaling on the trunk and palmoplantar hyperkeratoses with severely affected nails. The acanthotic epidermis was characterized by hyperproliferation with a large quantity of mitoses and extremely suppressed keratinization without a normal granular layer. The horny layer was parakeratotic and contained remnants of cell debris and lipid droplets. Ultrastructurally the prickle cell layer was characterized by binuclear cells, oedematization of the keratinocytes and isolated dyskeratotic cells. Some suprabasal cells showed unusual morphological features, containing nuclei with cytoplasmic pseudoinclusions, sometimes leading to a complete disintegration of the nuclear structure, and bowl- and lens-shaped accumulations of a filamentous material. Instead of normal tonofibrils, the aggregated material consisted of fine interlacing filaments. The latter are compared with the filamentous shells in ichthyosis hystrix Curth-Macklin and congenital reticular ichthyosiform erythroderma. The clinical symptomatology--congenital ichthyosis, growth retardation, secondary hypogonadism, hepatomegaly--and the ultrastructural characteristics of the keratinization disorder indicate that the present case cannot be considered as a subtype of the recessively inherited ichthyosis congenita group, but suggest a new syndrome as a separate nosologic entity.
Arch Dermatol Res 1992
PMID:Congenital ichthyosis with hypogonadism and growth retardation--a new syndrome with peculiar ultrastructural features. 141 65

Acne fulminans is a rare form of acne characterized by ulcerative nodules and associated with systemic complications. We report the case of a 17-year-old boy with cystic acne who experienced sudden onset of acne fulminans associated with fever, weight loss, hepatosplenomegaly, and erythema nodosum migrans. To the best of our knowledge, this is the first reported case of acne fulminans manifested by hepatomegaly and erythema nodosum migrans.
J Am Acad Dermatol 1991 May
PMID:Acne fulminans with hepatosplenomegaly and erythema nodosum migrans. 182 16

Hairless mice were exposed to UVB irradiation from a Philips T1 12 light source. Mice that were exposed to UV-light and not protected with 5% p-aminobenzoic acid (PABA) showed a significantly higher number of peripheral blood granulocytes (P less than 0.001) and a significantly higher mean weight of both the spleen and the liver (P less than 0.001) than the non-irradiated controls. Light microscopy of the histology of the enlarged liver and spleen showed a proliferation of granulocytes and the reticuloendothelial cells. Treatment with topical PABA during the whole period of UV-exposure prevented the peripheral blood granulocytosis. These protected mice also had a significantly (P less than 0.001) lower mean weight for the liver and spleen than UV-exposed and non-protected mice.
Br J Dermatol 1991 Sep
PMID:Ultraviolet light induction of peripheral granulocytosis with splenomegaly: protection of mice with topical p-aminobenzoic acid (PABA). 191 13

We report two patients with chronic, recurrent, nodular panniculitis lesions that later developed areas of lipoatrophy. Histologically, there was a consistent lobular lipophagic replacement of fat cells with lipophagic giant cells. The clinical appearance was that of tender, erythematous, superficial or subcutaneous, symmetrical nodules and plaques of 1-2 weeks' duration. The lesions could occur with episodes of fever. One patient had hepatomegaly and the other had an increased sedimentation rate and leucocytosis. The histology and the clinical pattern of the panniculitus syndrome resembled those of lipophagic lipoatrophy of childhood. This is a panniculitis of unknown cause in which the principal inflammatory cell response in the subcutaneous tissue is the macrophage.
Br J Dermatol 1991 Mar
PMID:Adult lipophagic atrophic panniculitis. 201 38

Six cases of telangiectatic clinical variant of mastocytosis (TMV) are reported. Patients' age varied from 33 to 62, there were 2 men and 4 women. A number of clinical features of TMV were detected in the course of follow-up: a generalized type of the skin process; abundant maculopapular eruptions colored brownish, sometimes with a cyanotic shade, liable to fuse and form diffuse foci of involvement; development of telangiectasias on the surface of these eruptions; symptoms of elevated blood histamin levels, i.e. regular blood congestion, enhanced itching, tachycardia, gastrointestinal events, etc.; systemic mastocytosis (3 of the 6 patients developed lymphadenopathy and hepatomegaly). The detected features of the condition permit considering TMV as an individual clinical variety of mastocytosis.
Vestn Dermatol Venerol 1990
PMID:[A telangiectatic clinical variant of mastocytosis]. 228 58

We describe an infant who presented at birth with numerous haemorrhagic and encrusted skin lesions, hepatomegaly, lymphadenopathy, raised hepatic transaminases, leucopenia and thrombocytopenia. The diagnosis of Langerhans cell histiocytosis was confirmed by immunohistochemistry, which demonstrated the presence of CD1, S-100 and DR positive cells in the skin infiltrate. The skin lesions resolved spontaneously after 6 weeks but recurred at 3 months and again were self involuting with resolution by 9 months. Persistent circulating T-cell abnormalities, including T-cell lymphopenia and the presence and persistence of peripheral blood CD1 + cells were noted throughout the first year of life.
Br J Dermatol 1990 Apr
PMID:Congenital self-healing Langerhans cell histiocytosis with persistent cellular immunological abnormalities. 233 23

We have studied 26 patients presenting with a symmetrical papular or papulovesicular acrolocated eruption of more than 10 days duration. Mean age at onset was 2 years (range 10 months to 5.75 years). Lymphadenopathy was noted in eight cases, and hepatomegaly in one case. In 12 cases, histopathology and direct immunofluorescence were non-contributory. Cytolytic hepatitis occurred in one case and was associated with HBs antigenemia. A history of recent immunization was given in two cases. There was serological evidence of recent Epstein-Barr virus infection in seven out of 13 cases tested. Coxsackie B viruses were isolated from three patients, and cytomegalovirus was probably associated with the syndrome in one case. We conclude that the Gianotti-Crosti syndrome is not rare in France, and that non-hepatitis B virus (HBV)-associated cases are more frequent than the classical HBV-associated papular acrodermatitis of childhood.
Br J Dermatol 1986 Jul
PMID:Gianotti-Crosti syndrome: a study of 26 cases. 301 87


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