Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

A primary cutaneous lesion in a 5-year-old boy who had recently received chemotherapy for acute lymphocytic leukemia was found to be caused by a dematiaceous fungus, Drechslera spicifera. The lesion was an erythematous macule that rapidly developed necrotic ulcerations. The fungus, which is commonly found in soil and as a plant pathogen, was isolated from cultures of the lesion and from an excisional biopsy specimen. Hyphae and swollen hyphal cells resembling chlamydospores were observed in the biopsy specimen. Septate pigmented hyphae were found in the tissue, which is consistent with phaeohyphomycosis. Resolution of the infection occurred following excisional biopsy and systemic amphotericin B therapy. There was a concomitant recovery from neutropenia.
Arch Dermatol 1977 Jun
PMID:Primary cutaneous phaeohyphomycosis caused by Drechslera spicifera. 55 73

Large granular lymphocytic leukemia refers to a clonal expansion of lymphocytes that have abundant cytoplasm and azurophilic granules. The disease is characterized clinically by chronic neutropenia and it may be associated with recurrent pyogenic infections. Except for these infections, cutaneous manifestations of this disease have not been well characterized. We describe a patient with large granular lymphocytic leukemia, which was confirmed by molecular genetics studies, who had a pyoderma gangrenosum-like ulcer on his leg. Results of an evaluation of the histologic characteristics and the leukocytic immunophenotype of a skin biopsy specimen from the ulcer demonstrated large granular lymphocytes within the blood vessels. Cutaneous ulceration may be a manifestation of large granular lymphocytic leukemia, and this disease should be considered when diagnosing patients with otherwise unexplained pyoderma gangrenosum-like ulcers of the skin.
J Am Acad Dermatol 1992 Nov
PMID:Pyoderma gangrenosum-like ulcer in a patient with large granular lymphocytic leukemia. 146 48

Deep cutaneous lesions are seldom encountered in lupus erythematosus. The disease described in the literature as lupus erythematosus profundus or lupus erythematosus panniculitis usually occurs in middle-aged women. The authors report a case of deep lupus erythematosus which was exceptional in that it occurred in an 11-year old girl. The lesions were situated on the face and consisted of solid subcutaneous nodules and clear-cut ulcerations leaving atrophic pigmented scars. The histological image of a nodule was one of lobular lymphocytic panniculitis with homogeneous hyalinization of adipose nodules, to which must be added periadnexal and perivascular dermo-epidermal lymphocytic infiltrates. Granular IgM deposits arranged along the dermo-epidermal junction were observed at direct cutaneous immunofluorescence. Laboratory examinations showed leucopenia (3,300/mm3) with neutropenia (1,100/mm3) and the presence of antinuclear antibodies at 1/100 speckled fluorescence, as well as antibodies directed against native DNA. Studies of renal function and for complement gave normal results. The other causes of lobular panniculitis were excluded. The lesions regressed within 3 weeks under hydroxychloroquine; this drug was also successful in arresting a relapse consecutive to withdrawal of treatment. The authors have analysed the 17 paediatric cases of deep lupus erythematosus and were able to determine their main characteristics: 1. The lesions occur mostly in girls (70 p. 100). They are located electively on the face and the lateral aspect of the shoulders. They consist of well-limited, solid or hard subcutaneous nodule which may congregate to form plaques. The epidermis may be normal or pathological, poikilodermic, looking like a discoid or ulcerated lupus erythematosus. The lesions regress, leaving a characteristic atrophic scar.(ABSTRACT TRUNCATED AT 250 WORDS)
Ann Dermatol Venereol 1992
PMID:[Deep lupus erythematosus in children]. 148 54

Disseminated fusariosis occurred in a pediatric patient with acute lymphocytic leukemia in relapse. The patient had fever and neutropenia, and scattered violaceous papules and vesicles with central erosions that rapidly progressed to generalized, painful, violaceous, papulovesicular lesions with central necrosis. Severe myalgias were associated. The diagnosis was suspected by noting hyphae on a smear of vesicular contents, and confirmed by culture. The clinical course was rapidly fatal despite early institution of amphotericin B therapy. Disseminated Fusarium infection should be suspected in immunocompromised patients with fever and neutropenia who have generalized, eroded, violaceous papules, vesicles, and pustules, particularly with associated myalgias.
Pediatr Dermatol 1992 Mar
PMID:Cutaneous Fusarium infection in an adolescent with acute leukemia. 157 78

A case of haemorrhagic pompholyx occurring in a 29-year-old man with linear IgA disease is described. There were several features in our patient that are usually seen in chronic bullous disease of childhood. Treatment with dapsone cleared the eruption but induced a progressive yet reversible neutropenia.
Br J Dermatol 1991 Aug
PMID:Linear IgA disease with haemorrhagic pompholyx and dapsone-induced neutropenia. 183 28

The Shwachman syndrome comprises exocrine pancreatic insufficiency, growth retardation, and bone marrow hypoplasia resulting in neutropenia. Clinical, morphological, and ultrastructural studies, as well as hair analysis, were performed in a patient with Shwachman's syndrome and severe ichthyosis. Clinical findings were lamellar ichthyosiform desquamation on the extremities. The hair was scanty and short on the scalp, in the eyelashes, and in the eyebrows. The nails were hyperkeratotic. Morphologic findings were slight, regular acanthosis and severe diffuse hyperkeratosis with variable parakeratosis. The granular layer was thickened. The papillary dermis showed very slight perivascular lymphocyte infiltration. The most prominent ultrastructural finding was the presence of solitary or multiple droplets of varying size in the cytoplasm of the keratinocytes. Hair analysis revealed no abnormalities; the cystine concentration in hair specimens was normal.
Arch Dermatol 1991 Feb
PMID:Ichthyosis, exocrine pancreatic insufficiency, impaired neutrophil chemotaxis, growth retardation, and metaphyseal dysplasia (Shwachman syndrome). Report of a case with extensive skin lesions (clinical, histological, and ultrastructural findings) 189 16

Trichothiodystrophy is a feature of several diseases that consist of characteristic hair shaft abnormalities and a wide spectrum of other developmental defects. Detection of sulfur-deficient hairs identifies this disorder and separates it from other similar ectodermal dysplasias with normal sulfur content. Detection of low sulfur hair syndrome is also important for genetic counseling, because the disease appears to be an autosomal recessive trait. We report a patient with chronic neutropenia, mild mental retardation, and low sulfur content in hair. Our case expands the spectrum of disorders associated with trichothiodystrophy.
J Am Acad Dermatol 1991 Feb
PMID:Trichothiodystrophy with chronic neutropenia and mild mental retardation. 199 47

A 19-year-old man with severe acne developed extensive scalp folliculitis and later superficial pyoderma gangrenosum following treatment with isotretinoin. A cyclical neutropenia was noted and bone marrow findings suggested myelodysplasia. We believe that isotretinoin was implicated in the development of overt symptoms in this patient whose haematological condition was previously asymptomatic.
Br J Dermatol 1990 May
PMID:Development of folliculitis and pyoderma gangrenosum in association with abdominal pain in a patient following treatment with isotretinoin. 214 Dec 75

A 40-year-old white woman with acute nonlymphocytic leukemia, which relapsed despite bone marrow transplantation and various chemotherapeutic regimens, developed fever and neutropenia. Her fever was unresponsive to broad-spectrum antibiotics, and on hospital day 53 she developed purpuric macules with necrotic centers on her left hand and forearm. Frozen sections of lesional skin were stained with Grocott's methenamine-silver and showed hyphae consistent with a species of Aspergillus; culture of the skin biopsy specimen yielded a pure culture of Aspergillus flavus. Localization of the emboli to the left upper extremity was subsequently explained by magnetic resonance imaging scan of the chest demonstrating invasion of the left subclavian artery by a pulmonary aspergilloma.
Arch Dermatol 1990 Sep
PMID:Unilateral cutaneous emboli of Aspergillus. 239 39


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