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At least 577 records · Page 32Linked to original sources
PubMed1992

[Liver transplantation in erythrohepatic protoporphyria].

A 51-year-old man had for 5 years been known to have erythropoietic protoporphyria. GPT levels were raised up to 40 U/l, gamma-GT up to 120 U/l. After lengthy exposure to sun radiation an erythema with blisters, abdominal discomfort and jaundice developed (total bilirubin 7.3 mg/dl) and biliary liver cirrhosis with portal hypertension and splenomegaly were diagnosed. Because the acute hepatobiliary complications were not improved by conservative treatment (daily 750 mg ursodeoxycholic acid and 12 g colestyramine), an orthotopic liver transplantation was performed without complication. The excised liver showed small nodular parenchymal transformation and contained reddish brown protoporphyrin pigment in the hepatocellular cytoplasm, the Kupffer cells, the canaliculi and in some biliary ducts. Bilirubin and transaminase levels in blood became normal after the transplantation, as did the urinary excretion of coproporphyrin. However, isomer I was still dominant. The protoporphyrin level in erythrocytes and plasma remained elevated. After a symptom-free interval of one year biochemical and histological tests demonstrated protoporphyrin-induced damage in the transplanted liver.

Cholestyramine Resin↗
PubMed2006

Successful treatment of nodular actinic reticuloid with tacrolimus ointment.

Actinic reticuloid (AR) is the severest clinical variant of chronic actinic dermatitis (CAD) and describes a persistent photoinduced skin disorder often associated with delayed-type hypersensitivity reactions. Histopathologically, AR resembles pseudolymphoma and may also show features of cutaneous lymphoma. In contrast to other UV-induced skin diseases, AR patients show persistent photosensitivity to UV radiation and visible light parallel to permanent changes of skin texture with infiltrated papules and thickened plaques. Therapeutically, CAD is a problematic condition. Daily application of sun protection has to be combined with local or systemic immunosuppression. However, various therapeutic approaches including systemic corticosteroids and other systemic immunosuppressive agents are limited by severe side effects. Tacrolimus, a Streptomyces-derived immunosuppressive macrolide antibiotic and inhibitor of calcineurin, has been proven successful in various inflammatory skin diseases including atopic eczema, allergic contact dermatitis and photodermatoses without undesired side effects. We present a case of severe recalcitrant and nodular chronic actinic dermatitis responding to topically applied tacrolimus (Protopic). To avoid further relapses we continued this therapy twice a day over a period of 2 years.

Administration, Cutaneous↗
PubMed1993

The clinical spectrum of mid-dermal elastolysis and the role of UV light in its pathogenesis.

BACKGROUND: We observed four patients with mid-dermal elastolysis (MDE) that was either precipitated or aggravated by UV light (UVL) exposure or was primarily confined to areas of UVL exposure. OBJECTIVE: Our purpose was to report four cases of MDE occurring after significant UVL exposure and to demonstrate why we suspect that MDE in some instances may be photoinduced or photoaggravated. We also wish to demonstrate the varied clinical presentation of this disorder. METHODS: Because all our patients had MDE involving skin exposed to UVL, biopsies were performed on clinically uninvolved sun-exposed and sun-protected skin in one patient to elucidate further the role of UVL in this process. RESULTS: On routine histopathologic examination we found that uninvolved sun-exposed skin but not sun-protected skin demonstrated early MDE. Ultrastructural examination revealed a spectrum of elastic fiber changes in involved and clinically uninvolved sun-exposed skin. Phagocytosis of elastic fibers was not present. CONCLUSION: We conclude that UVL exposure was a major causative or aggravating factor of MDE in our patients and that the clinical appearance of this disorder is variable.

Adult↗
PubMed1986

The erythropoietic porphyrias.

There are two well-characterized disorders of porphyrin-heme metabolism in which the bulk of the porphyrins that accumulate in excess are formed chiefly within juvenile erythroid elements of the bone marrow. The first is most often termed congenital erythropoietic porphyria, and the second is most often termed erythropoietic protoporphyria. The former is a rare disorder, whereas the latter is one of the two most common porphyrias (along with porphyria cutanea tarda) and has a good probability of being encountered in general dermatologic practice. Both are determined genetically, cause cutaneous photosensitivity and systemic complications of importance, and are amenable to various forms of therapy.

Diagnosis, Differential↗
PubMed1974

[Bloom's syndrome].

Explore the source record for details and available documents.

Abnormalities, Multiple↗
PubMed2004

Ultraviolet light and rosacea.

The general consensus among clinicians is that rosacea is a photoaggravated disorder. Pathophysiologic processes induced by UV radiation, which are processes similar to those seen in photoaging, contribute to the signs and symptoms of rosacea. Because of the purported role of solar radiation, clinicians may want to use photosensitizing antibiotics with discretion in patients with rosacea. In addition to topical and oral therapy for rosacea, clinicians should recommend that patients use sunscreens or sunblocks (inorganic chemicals such as zinc oxide or titanium dioxide).

Anti-Bacterial Agents↗
PubMed1982

Unusual complications of antipsychotic drugs.

Phenothiazines have been responsible for pigmentary changes in the skin (phototoxic reaction) and for pigment deposits in the cornea, lens and retina, which can result in blindness. Reversible agranulocytosis has also been reported with these agents. Psychotic exacerbation, including a form responsive to antiparkinsonian agents, may occur with high-potency antipsychotic drugs. Neuroleptic malignant syndrome, usually following high-dose therapy in young men, is a disorder of thermoregulatory mechanisms.

Adult↗