1/5. Pseudoporphyria induced by propionic acid derivatives.BACKGROUND: Pseudoporphyria is a photosensitive bullous skin disease that is distinguished from porphyria cutanea tarda (PCT) by its normal porphyrin profile. Drugs are a major cause of this disease, and the list of culprits is continually expanding. Nonsteroidal antiinflammatory agents (NSAIDs), especially naproxen and other propionic acid derivatives, appear to be the most common offenders. OBJECTIVE: The study was carried out to increase awareness about the etiology and characteristic features of pseudoporphyria. methods: We report two cases of pseudoporphyria caused by naproxen and oxaprozin. We review the current English language literature on this entity and discuss its clinical features, histology, ultrastructure, etiology, and pathophysiology. RESULTS: A 44-year-old man taking naproxen for chronic low back pain and a 20-year-old woman on oxaprozin for rheumatoid arthritis presented with tense bullae and cutaneous fragility on the face and the back of the hands. In both, skin biopsy showed a cell-poor subepidermal vesicle with festooning of the dermal papillae. Direct immunofluorescence revealed staining at the dermal-epidermal junction and around blood vessels with IgG in the first case and with IgG, IgA, and fibrin in the second case. urine collections and serum samples yielded normal levels of uro- and coproporphyrins. CONCLUSIONS: Most cases of pseudoporphyria are drug-induced. naproxen, the most common offender, has been associated with a dimorphic clinical pattern: a PCT-like presentation and one simulating erythropoietic protoporphyria in the pediatric population. Other NSAIDs of the propionic acid family can also cause pseudoporphyria.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/5. erythema elevatum diutinum mimicking porphyria cutanea tarda.A case of erythema elevatum diutinum (EED) closely resembling porphyria cutanea tarda (PCT) is reported. The initial skin biopsies were suggestive for PCT but porphyrin levels in the urine, stool and plasma were normal. A further biopsy from an early cutaneous lesion showed a leucocytoclastic vasculitis with fibrinoid necrosis of the vessel walls.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/5. optic atrophy in acute intermittent porphyria.A 24-year-old woman developed bilateral blindness after recovery from coma secondary to acute intermittent porphyria. Gradual return of vision in the right eye with a permanent unilateral visual field defect and optic atrophy followed. We believe the pathophysiologic mechanism was spasm of the vessels supplying the optic disk leading to ischemia and infarction of the optic nerve.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/5. Retinal branch vessel occlusion in acute intermittent porphyria.Three patients with acute intermittent porphyria were noted to have retinal branch vessel occlusion. Branch "vein" occlusion, segmental optic atrophy, and soft exudate were the most common ocular manifestation. Two patients had labile elevated hypertension. When patients present with retinal branch vessel occlusion and a constellation of bizarre symptoms that might include hypertension, abdominal pain, acute psychotic behavior and/or cutaneous photosensitivity, the diagnosis of acute intermittent porphyria should be considered.- - - - - - - - - - ranking = 6keywords = vessel (Clic here for more details about this article) |
5/5. Lipoid proteinosis of the oral mucosa: case report and review of the literature.We describe a 37-year-old woman who presented with progressive mouth dryness. physical examination revealed long-standing plaques on the face and upper limbs, papular lesions of the oral cavity and tongue firmness. A lower lip biopsy was performed. light microscopy demonstrated accumulation of PAS-positive material around blood vessels, capillaries and salivary gland canaliculi as well as focally massive hyaline deposits in the submucosa. immunohistochemistry revealed widespread presence of type IV collagen in the hyaline material and around thickened blood vessels. laminin immunoreactivity was particularly strong at thickened basement membranes. The above findings were compatible with lipoid proteinosis, which is likely to involve primary perturbation of collagen metabolism and production of glycoproteins.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |