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1/4. 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.
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2/4. Actinic superficial folliculitis.

    Actinic superficial folliculitis was first described in 1985, and since then only three reports have been published. Clinically and histopathologically this disease is very particular and has been suggested to be considered as an entity. We report on a 30-year-old man who presented with an extensive superficial follicular pustulosis on his back, shoulders and upper chest after exposure to intense heat and subsequent sweating on a sunny day. The pustules arose within 24-36 h afterwards. histology and immunohistochemistry revealed subcorneal pustules, suppurative folliculitis and an infiltrate consisting of T cells, macrophages and neutrophils around the hair follicle, sebaceous glands and small vessels. To the best of our knowledge this is the fourth report on actinic superficial folliculitis and the first on which a characterization of the inflammatory infiltrate has been performed. Because of the impressive, unique symptoms and the characteristic histology we agree with those who have suggested that actinic superficial folliculitis is a new entity.
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3/4. 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.
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4/4. chlorella photosensitization. New phytophotodermatosis.

    Swelling followed by erythematopurpuric lesions on sun-exposed areas of the body developed in five patients. All patients were found to have ingested chlorella. The histopathologic changes consisted of swelling of endothelial cells and thrombosis of small blood vessels in the dermis and the subcutaneous fatty tissue. The photosensitizing agent contained in chlorella tablets was proved to be pheophorbide -a and its ester.
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