Non-classical congenital adrenal hyperplasia in association with porphyria cutanea tarda: co-incidence or trigger?

Ann Clin Biochem

Department of Chemical Pathology, Salford Royal NHS Foundation Trust, Salford M6 8HD, UK.

Published: September 2012

We present an interesting case of porphria cutanea tarda with hereditary haemochromatosis and non-classical congenital adrenal hyperplasia in a postmenopausal woman. This unusual combination of conditions does not appear to be reported in the literature. The exact mechanism causing porphyria in this patient is not clear. We consider the possibility that in addition to iron accumulation and excess alcohol, altered steroid milieu may have been a possible trigger for the development of bullous lesions in this patient.

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http://dx.doi.org/10.1258/acb.2011.011170DOI Listing

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  • - The case highlights the need to consider NCCAH in patients showing signs of hyperandrogenism, pointing to the complexity of his condition.
  • - Treatment with oral dexamethasone and contraceptive pills led to gradual improvement in the patient's symptoms, stressing the importance of proper clinical evaluation.
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