Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This clinicopathologic study of patients with chronic graft-versus-host disease (GVHD) after allogeneic marrow transplantation emphasizes the most prominent feature of the syndrome, the cutaneous aspects, and describes the ophthalmic-oral sicca syndrome with sialoadenitis and the neurologic findings. Chronic cutaneous GVHD affected 19 of 92 recipients surviving 150 days or more. In 6 patients chronic GVHD presented as a continuation of acute GVHD; in 8 it occurred after the resolution of acute GVHD; and in 5 it arose without preceding acute GVHD, ie, de novo late onset. Two cutaneous types were distinguished. The generalized type affected 16 patients and ran a progressive course resulting in late complications of poikiloderma, diffuse dermal and subcutaneous fibrosis, and contractures. Microscopically, it resembled generalized morphea and lupus erythermatosus hypertrophicus et profundus. The local type affected 3 patients with a more variable picture of poikiloderma, dermal sclerosis, and contractures. Microscopically, it resembled lupus of erythematosus profundus and scleroderma. Guidelines for defining and subclassifying chronic cutaneous GVHD are proposed.
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PMID:Chronic cutaneous graft-versus-host disease in man. 2 21

Skin diseases associated with photosensitivity are numerous and may be divided into three main groups: photo-aggravated dermatoses, genophotodermatoses and metabolic photodermatoses. Photo-aggravated dermatoses are autonomous skin diseases in which exposure to sunlight may make the disease worse or precipitate its onset and/or its progressiveness; this group includes lupus erythematosus, autoimmune bullous diseases, acantolytic dyskeratoses, acne vulgaris, rosacea and cutaneous lymphoid infiltrates. To these must be added photosensitive forms of autonomous dermatoses such as atopic dermatitis, psoriasis, herpes labialis, erythema multiforme, granuloma and disseminated superficial actinic porokeratosis. Genophotodermatoses are genodermatoses which are made photosensitive by a recognized or as yet unidentified deficiency of the natural photoprotection system. In this group are albinism, vitiligo, xeroderma pigmentosum and poikiloderma. Metabolic photodermatoses are diseases in which photosensitization reactions, often revealing, are due to the accumulation in the skin of an endogenous chromophore as a result of a congenital (porphyria) or acquired (pellagra) enzymatic disorder.
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PMID:[Skin diseases with photosensitivity]. 152 48

The authors present a new classification for photodermatosis in five groups: 1. Primary toxic photodermatosis that means lesions produced in all human beings by non-ionized radiation. 2. Photodermatosis induced by drugs, with two subgroups--phototoxic and photoalergic--according to the mechanism of action of the drug. 3. Idiopathic photodermatosis in which the photonic effects are known but the chromophores are unknown; four conditions are included here polymorphous actinic eruption, solar urticaria, actinic reticuloid and hidroa vacciniforme. 4. Miscelanea group which includes several conditions of unknown mechanism which are not included in the other groups, such as: actinic cheilitis, actinic poikiloderma, actinic ceratoses, epitheliomas, melanomas and others. 5. Conditions precipitated or aggravated by solar radiation with two sub-groups: hereditary (xeroderma pigmentosum, Hartnup's syndrome and other) and acquired (lupus erythematosus, pellagra and other). In group 1, the authors propose the designation of actinic elastotic dermatosis to unify different conditions described by several authors such as: diffuse elastosis, citrein skin, cutis rhomboidalis, and others.
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PMID:[A new classification of photodermatoses]. 266 86

A 7-year-old girl developed recurrent febrile nodules and subcutaneous plaques on her arm, shoulder, and face. Primary acquired toxoplasmosis was diagnosed at onset, because of associated lymphadenopathy and detection of specific IgM. Skin lesions were responsive to therapy with systemic steroids but progressed to atrophy and poikiloderma within a few months. Subsequently, chloroquine therapy has been instituted and no relapse has occurred. Histopathologic findings and direct immunofluorescence studies were diagnostic of lupus erythematosus panniculitis. Electron microscopy showed lamellar inclusions within mast cells. Results of laboratory investigations disclosed leukopenia and hypocomplementemia with low C2 serum levels. A family study of the complement system, including C4 and Bf phenotyping and HLA A, B, and DR haplotyping, revealed the carriage of both C2 and C4A null alleles in the propositus. This observation suggests an additional link between lupus erythematosus panniculitis and the remainder of the lupus erythematosus clinical spectrum.
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PMID:Lupus erythematosus panniculitis with partial genetic deficiency of C2 and C4 in a child. 370 76

A 64-year-old woman experienced progressive generalized poikiloderma after an episode of sunburn 4 years earlier. The diagnosis of subacute cutaneous lupus erythematosus (SCLE) was confirmed by the presence of anti-Ro/SS-A and antinuclear antibodies, the histology, and the direct immunofluorescent findings (ie, positive lupus band test and "dust-like" epidermal IgG staining pattern). Poikiloderma has not been previously reported in the spectrum of SCLE. As a major pathomechanism of SCLE, photosensitivity might explain this uncommon clinical manifestation of the disease.
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PMID:Subacute cutaneous lupus erythematosus presenting with generalized poikiloderma. 1064 88

Subacute cutaneous lupus erythematosus (SCLE) is a type of lupus erythematosus having distinct characteristic clinical, serologic, and genetic features. Other than the commonly occurring papulosquamous and annular polycyclic lesion, rarely it may present as erythema multiformae, toxic epidermo necrolysis like lesion (Rowell syndrome), erythroderma, and generalized poikiloderma. Herein, we report a case of SCLE presenting as erythroderma.
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PMID:Subacute cutaneous lupus erythematosus presenting as erythroderma. 2548 33

The search for clinical signs suggestive of diseases and medical analysis in works of art and portraits is also known as iconodiagnosis. It raises discussions about underlying diseases and about whether the artist intended to represent them. We assessed the frequency of cutaneous signs in paintings on display in the permanent collections of the Ateneum and Sinebrychoff Art Museums, Finnish National Gallery in Helsinki. The most common feature was facial redness. Redness was mainly located on the cheeks with variable intensity according to paintings. Facial redness may be related to stylistic features, to make-up of the sitter, or the painter intended to depict an individual in good health or a specific emotion. It may be also related to rosacea, a common feature in individuals with fair skin. Lupus was not evoked in any of the cases. Additional specific findings included mainly sun-exposed skin lesions such as sun tan or chronic poikiloderma, skin aging (Milian's citrine skin), naevi, keratosis pilaris rubra, and ear piercing. We report here some specificities of the skin conditions displayed in the Finnish National Gallery. Examining from a dermatological point of view, works of art gives to a museum visit a twist.
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PMID:Skin abnormalities in the Finnish National Gallery. 3144 88