- Craig A Elmets, MD
Craig A Elmets, MD
- Section Editor — Photodermatology
- Professor and Chair of Dermatology
- University of Alabama at Birmingham
- Section Editors
- Jean-Claude Roujeau, MD
Jean-Claude Roujeau, MD
- Section Editor — Drug Eruptions
- Emeritus Professor of Dermatology
- Université Paris-Est Créteil (UPEC), Créteil, France
- Maja Mockenhaupt, MD, PhD
Maja Mockenhaupt, MD, PhD
- Section Editor — Drug Eruptions
- Professor of Dermatology
- Medical Center - University of Freiburg, Germany
Pseudoporphyria is a bullous photodermatosis with the clinical and histologic features of porphyria cutanea tarda (PCT) in the absence of abnormalities in porphyrin metabolism . Pseudoporphyria has been associated with medications, chronic renal failure and hemodialysis, and tanning beds. Patients typically present with skin fragility, bullae, milia, and scarring on the dorsum of the hands and other sun-exposed areas. Histologically, there is a noninflammatory subepidermal blister.
This topic will discuss the pathogenesis, clinical manifestations, diagnosis, and treatment of pseudoporphyria. PCT and other types of porphyrias associated with photosensitivity are discussed separately. (See "Porphyria cutanea tarda and hepatoerythropoietic porphyria: Clinical manifestations and diagnosis" and "Erythropoietic protoporphyria and X-linked protoporphyria".)
The incidence of pseudoporphyria is unknown. It occurs at any age and more frequently in women than in men. Pseudoporphyria has been reported in approximately 10 percent of children taking naproxen, a nonsteroidal antiinflammatory drug routinely used in the therapy of juvenile idiopathic arthritis. Among children treated with naproxen, risk factors for the development of pseudoporphyria include skin phototypes I or II (table 1), blue/gray eyes, and the concurrent use of chloroquine [2-4]. In one study of 363 hemodialysis patients, pseudoporphyria occurred in two (0.6 percent) .
ETIOLOGY AND PATHOGENESIS
Four factors have been implicated in the etiology of pseudoporphyria: ultraviolet (UV) radiation, medications, chronic renal failure and hemodialysis, and tanning beds.
UV radiation — Although the exact pathogenetic mechanism is unknown, drug-induced pseudoporphyria is considered a photodynamic phototoxic drug reaction. In this type of reaction, the photosensitizing compound, upon absorption of the appropriate wavelength radiation, changes to an excited state and reacts with oxygen to form reactive oxygen species (ROS) such as singlet oxygen, hydrogen peroxide, or superoxide anion. ROS can damage cell components such as nucleic acids, membranes, lipids, and proteins. Mediators of inflammation and inflammatory cells participate in tissue injury, including products of complement activation, proinflammatory cytokines, arachidonic acid metabolites, proteases, and polymorphonuclear leukocytes.
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- ETIOLOGY AND PATHOGENESIS
- UV radiation
- Hemodialysis and peritoneal dialysis
- Tanning beds
- CLINICAL MANIFESTATIONS
- CLINICAL COURSE
- DIFFERENTIAL DIAGNOSIS
- Porphyria cutanea tarda
- Epidermolysis bullosa acquisita
- Bullous pemphigoid
- Bullous lupus erythematosus
- Hydroa vacciniforme
- Erythropoietic protoporphyria
- SUMMARY AND RECOMMENDATIONS