eMedicine Specialties > Dermatology > Bullous Diseases

Pseudoporphyria: Differential Diagnoses & Workup

Author: Elizabeth L Tanzi, MD, Co-Director, Laser Surgery, Washington Institute of Dermatologic Laser Surgery
Coauthor(s): Vincent A De Leo, MD, Clinical Professor of Dermatology, Department of Dermatology, College of Physicians and Surgeons of Columbia University; Chairman, Department of Dermatology, Director of Dermatology Residency Training Program, St Luke's-Roosevelt Hospital Center; Chairman, Department of Dermatology, Beth Israel Medical Center
Contributor Information and Disclosures

Updated: Jun 18, 2009

Differential Diagnoses

Bullous Pemphigoid
Epidermolysis Bullosa
Epidermolysis Bullosa Acquisita
Erythropoietic Protoporphyria
Lupus Erythematosus, Bullous
Porphyria Cutanea Tarda

Workup

Laboratory Studies

  • Of critical importance in the diagnosis of pseudoporphyria is the exclusion of true porphyria.
    • The most important test is a serum/plasma porphyrin assay. If this result is negative, the patient does not have a true porphyria.
    • If the serum/plasma porphyrin assay is unavailable, erythrocytes, urine, and stool may be evaluated for abnormal porphyrin levels.
  • Other causes of photosensitivity, such as connective tissue disease, must be excluded by obtaining a serum antinuclear antibody titer and more specific studies, such as antibodies to Ro, La, ribonucleoprotein, Smith, and double-stranded DNA.

Procedures

  • If the diagnosis of pseudoporphyria is suspected, biopsies for histologic evaluation with hematoxylin and eosin stains and direct immunofluorescence should be performed. Serum samples may also be obtained for indirect immunofluorescence evaluation to aid in the exclusion of bullous pemphigoid.

Histologic Findings

The histologic features of pseudoporphyria are similar to those of porphyria cutanea tarda (PCT) with subepidermal bullae and festooning of the dermal papillae. The thickness of the blood vessel wall may prove helpful in differentiating pseudoporphyria from porphyria cutanea tarda.

In a comparative histologic study from biopsy samples of patients with porphyria cutanea tarda and pseudoporphyria, Maynard and Peters found thickened blood vessel walls in 11 of 13 patients with porphyria cutanea tarda. In contrast, similar findings in only 1 of 9 patients with pseudoporphyria were present.49

Porphyria cutanea tarda and pseudoporphyria have similar, nonspecific direct immunofluorescence findings of granular deposits of immunoglobulins, mostly IgG, and C3 at the basement membrane zone and in the perivascular region. Although direct immunofluorescence is not a useful tool in distinguishing pseudoporphyria from porphyria cutanea tarda, it is helpful in the evaluation of other entities in the differential diagnosis of pseudoporphyria, specifically epidermolysis bullosa acquisita. Epidermolysis bullosa acquisita can be ruled out by the lack of intense, linear immunoreactants at the dermal-epidermal junction. Neither porphyria cutanea tarda nor pseudoporphyria has circulating autoantibodies detected by indirect immunofluorescence study.

More on Pseudoporphyria

Overview: Pseudoporphyria
Differential Diagnoses & Workup: Pseudoporphyria
Treatment & Medication: Pseudoporphyria
Follow-up: Pseudoporphyria
References
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References

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Further Reading

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Keywords

pseudoporphyria, drug-induced bullous photosensitivity, therapy-induced bullous photosensitivity, PCT, porphyria cutanea tarda

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Contributor Information and Disclosures

Author

Elizabeth L Tanzi, MD, Co-Director, Laser Surgery, Washington Institute of Dermatologic Laser Surgery
Elizabeth L Tanzi, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, and American Society for Laser Medicine and Surgery
Disclosure: Nothing to disclose.

Coauthor(s)

Vincent A De Leo, MD, Clinical Professor of Dermatology, Department of Dermatology, College of Physicians and Surgeons of Columbia University; Chairman, Department of Dermatology, Director of Dermatology Residency Training Program, St Luke's-Roosevelt Hospital Center; Chairman, Department of Dermatology, Beth Israel Medical Center
Vincent A De Leo, MD is a member of the following medical societies: American Academy of Dermatology, American College of Occupational and Environmental Medicine, American Contact Dermatitis Society, American Dermatological Association, American Medical Association, American Society for Photobiology, Dermatology Foundation, New York Academy of Medicine, New York County Medical Society, Photomedicine Society, Society for Investigative Dermatology, Society of Toxicology, and Women's Dermatologic Society
Disclosure: estee lauder Consulting fee Consulting; laroche posay Consulting fee Consulting; schering plough Consulting fee Consulting; pfizer Consulting fee Consulting; orfagen Grant/research funds study - clinical

Medical Editor

Maureen B Poh-Fitzpatrick, MD, Professor Emerita of Dermatology and Special Lecturer, Columbia University; Professor of Medicine (Dermatology), University of Tennessee
Maureen B Poh-Fitzpatrick, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and New York Academy of Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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