Literature DB >> 19756434

[Solarium-induced pseudoporphyria and variegate porphyria as rare differential diagnoses of porphyria cutanea tarda].

C Kochs1, E Mühlenstädt, N J Neumann, S Hanneken.   

Abstract

Three patients presented with typical porphyria cutanea tarda-like vesicles, erosions and scars as well as increased fragility, primarily on the back of the hands. In two of the three, porphyrin workup was normal. Skin biopsy was compatible with porphyria cutanea tarda (PCT) or pseudoporphyria. The common aspect in the patients' history was the frequent use of solaria for many years, so that UV-induced pseudoporphyria was diagnosed. Treatment was strict abstention from UV radiation and regular dermatologic controls for signs of skin damage. Porphyrin analysis in the third patient showed normal excretion of total urine porphyrins and precursors; however, fecal porphyrins were elevated with dominating coproporphyrins in HPLC and the plasma fluorescence scan yielded a peak at 625 nm. Subsequent mutation analysis showed a mutation in the protoporphyrinogen oxidase gene, thereby confirming the diagnosis of variegate porphyria. Five months after the initial diagnosis the patient presented with the first acute attack. Further investigations revealed a metastasized carcinoma of the colon, which probably triggered the acute attack. Our cases show rare differential diagnoses in patients presenting with typical PCT-like skin lesions. The discrimination between porphyria cutanea tarda and its differential diagnoses is very important since it has an important impact not only on the treatment modality but also on the course and the prognosis of the disease.

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Year:  2009        PMID: 19756434     DOI: 10.1007/s00105-009-1846-6

Source DB:  PubMed          Journal:  Hautarzt        ISSN: 0017-8470            Impact factor:   0.751


  7 in total

Review 1.  Pseudoporphyria.

Authors:  J J Green; S M Manders
Journal:  J Am Acad Dermatol       Date:  2001-01       Impact factor: 11.527

2.  Metastatic colon cancer triggering an acute attack of variegate porphyria.

Authors:  S Hanneken; V Kuerten; M Hoernke; N J Neumann
Journal:  Int J Colorectal Dis       Date:  2008-07-30       Impact factor: 2.571

3.  Pseudoporphyria or porphyria cutanea tarda? Diagnostic and treatment difficulties.

Authors:  Sheila A M Fevang; Susanne Kroon; Øyvind Skadberg
Journal:  Acta Derm Venereol       Date:  2008       Impact factor: 4.437

4.  Sunbed-induced pseudoporphyria.

Authors:  G M Murphy; J Wright; D S Nicholls; P H McKee; A G Messenger; J L Hawk; G M Levene
Journal:  Br J Dermatol       Date:  1989-04       Impact factor: 9.302

Review 5.  Variegate porphyria.

Authors:  R E Kirsch; P N Meissner; R J Hift
Journal:  Semin Liver Dis       Date:  1998       Impact factor: 6.115

6.  Pseudoporphyria and sunbeds.

Authors:  A Stenberg
Journal:  Acta Derm Venereol       Date:  1990       Impact factor: 4.437

7.  Identical twins with sunbed-induced pseudoporphyria.

Authors:  C L Wilson; S S Mendelsohn
Journal:  J R Soc Med       Date:  1992-01       Impact factor: 18.000

  7 in total
  1 in total

1.  Pseudoporphyria induced by hemodialysis.

Authors:  Beata Bergler-Czop; Ligia Brzezińska-Wcisło
Journal:  Postepy Dermatol Alergol       Date:  2014-02-25       Impact factor: 1.837

  1 in total

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