1/6. 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 = skin disease (Clic here for more details about this article) |
2/6. antibodies to the borrelia burgdorferi flagellum in patients with scleroderma, granuloma annulare and porphyria cutanea tarda.It is generally accepted that cutaneous Lyme borreliosis comprises erythema chronicum migrans, lymphadenosis benigna cutis, and acrodermatitis chronica atrophicans. In recent years the tick-borne spirochete borrelia burgdorferi has been associated with a number of other cutaneous disorders. We therefore investigated sera from 175 patients with localized scleroderma (morphea) (n = 64), systemic sclerosis (n = 74), granuloma annulare (n = 16) and porphyria cutanea tarda (n = 21) with the new, highly sensitive and specific borrelia burgdorferi flagellum ELISA assay. As controls (n = 297) served normal healthy volunteers and patients with other skin diseases. It was found that the distribution of individual antibody values and the median antibody levels were identical in controls and in patients with scleroderma, granuloma annulare and porphyria cutanea tarda. These data do not support the hypothesis of an etiological association between borrelia burgdorferi infection and scleroderma, granuloma annulare or porphyria cutanea tarda.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
3/6. Homozygous variegate porphyria. A severe skin disease of infancy.A boy exhibited severe bullous skin disease a few days after birth, followed by increased fragility of the exposed skin in spring and summer. Examination at 2 1/2 years of age led to characteristic biochemical findings: increased excretion of fecal porphyrins (coproporphyrin 121 to 131 and protoporphyrin 467 to 576 nmol/g dry weight), and increased erythrocyte protoporphyrin concentration (3643 to 4840 nmol/l). Lymphocyte protoporphyrinogen oxidase activity was very low in the patient (0.4 nmol/mg protein/h) and half-normal (2.7 and 2.3 nmol/mg protein/h) in the parents, suggesting that the patient had homozygous variegate porphyria. Severe skin symptoms and a high concentration of red cell protoporphyrin concentration in an infant should prompt suspicion of homozygous acute hepatic porphyria.- - - - - - - - - - ranking = 5keywords = skin disease (Clic here for more details about this article) |
4/6. A case of porphyria cutanea tarda with hemangioma of the liver.The clinical incidence of porphyria cutanea tarda is apparently less in japan than in European countries and the U.S.A.. In the last 7 years, porphyria cutanea tarda was encountered in the dermatologic clinic of Tokai University Hospital at the rate of one in twelve thousand patients with various kinds of skin diseases. This case is the first case in our medical clinic at Tokai University Hospital in the past 7 years, among 2,456 cases of liver diseases in which laparoscopy with needle biopsy of the liver was performed. The physiopathological aspects remain obscure. Unexpectedly, a hemangioma was disclosed under laparoscopic observation but it was clear that the hemangioma had no significant relation to the thrombopenia-hemangioma syndrome in this case.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
5/6. Treatment of hemodialysis-related porphyria cutanea tarda with plasma exchange.A patient with hemodialysis-related porphyria cutanea tarda was treated with plasma exchange. A rapid clinical response occurred coincidentally with a significant fall in the plasma porphyrin level. The level fell further over the following few months without additional therapeutic intervention, whereafter a slow rise occurred without recurrence of skin disease. We suggest that this form of treatment may be ideal for the patient with porphyria cutanea tarda and chronic renal failure in whom no alternative therapy is available for the cutaneous problem.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
6/6. Severe cutaneous porphyria in a 12-year-old boy: hepatoerythropoietic or symptomatic porphyria?Deforming cutaneous porphyria developed in a 12-year-old boy; the severity of his skin disease resolved spontaneously as he grew older. The clinical and biochemical aspects of this case clearly fit the descriptions in the medical literature of both hepatoerythropoietic porphyria (HEP) and infantile symptomatic porphyria (SP). Further porphyrin analyses indicated that our patient suffered from infantile SP. The lack of unquestionably unique features diagnostic for HEP raises strong doubts that it exists as a separate disease entity substantially different from infantile SP.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |