Porphyria Cutanea Tarda: A Metabolic Human Blistering Disease

Lamkin, Barry C.; Bickers, David R.

doi:10.1016/s0733-8635(18)31043-x

Cited by 4 publications

(2 citation statements)

References 54 publications

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“…Pseudoporphyria is associated with patients who are on dialysis for chronic renal failure and patients who are being treated with nalidixic acid, tetracycline, or furosemide. The diagnosis is confirmed with histologic, immunopathologic, hematologic, and urine studies (95–97). Routine histology of a lesion demonstrates the presence of a subepidermal blister with little inflammatory infiltrate in the dermis.…”

Section: Porphyria Cutanea Tardamentioning

confidence: 94%

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Blistering disorders: diagnosis and treatment

et al. 2003

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Blistering diseases are a heterogeneous group of disorders that can affect either skin and mucous membrane, or both, varying in presentation, clinical course, pathohistology, immunopathology and treatment. Not infrequently the diagnosis is delayed. This can result in severe, and sometimes fatal consequences. Although these diseases are rare, it is very important to make an accurate diagnosis based on a combination of clinical profile and laboratory observations. A brief review is presented of the following bullous diseases: pemphigus, paraneoplastic pemphigus, bullous pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita, dermatitis herpetiformis, linear IgA bullous disease, porphyria cutanea tarda, and subcorneal pustular dermatitis. Their clinical, pathohistologic and immunopathologic features and recommendations for therapy are discussed.

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Section: Porphyria Cutanea Tardamentioning

confidence: 94%

“…Porphyria cutanea tarda (PCT) is a rare metabolic blistering disease with an incidence of 1 in 25,000 individuals (95,96). PCT patients have a dysfunctional enzyme in the liver called uroporphyrinogen decarboxylase.…”

Section: Porphyria Cutanea Tardamentioning

confidence: 99%

Blistering disorders: diagnosis and treatment

et al. 2003

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Porphyrias

Bickers¹

1990

Management of Blistering Diseases

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The Porphyrias

Meola

Lim

1993

Dermatologic Clinics

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The vesiculobullous porphyrias are a group of blistering diseases with systemic as well as cutaneous manifestations ranging from mild to disabling, secondary to endogenous photosensitizing porphyrins. The characteristic patterns of porphyrin accumulation in erythrocytes, plasma, urine, and feces are invaluable for differentiating the vesiculobullous porphyrias from other blistering diseases and from one another. Most importantly, these porphyrin profiles are essential for distinguishing the acute vesiculobullous porphyrias, HCP and VP, from PCT, which is not associated with acute attacks. This distinction may prevent the potentially fatal administration of porphyrinogenic drugs to patients with HCP or VP. Histopathology is not helpful in differentiating the vesiculobullous porphyrias, as all typically show subepidermal bullae. Avoidance of sun exposure and precipitating factors are the initial steps in both the prevention and treatment of these entities. In addition, more specific therapy may be indicated, as discussed in the preceding pages.

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Porphyria Cutanea Tarda: A Metabolic Human Blistering Disease

Cited by 4 publications

References 54 publications

Blistering disorders: diagnosis and treatment

Blistering disorders: diagnosis and treatment

Porphyrias

The Porphyrias

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