Cutaneous manifestations of idiopathic hermochromatosis.Study of 100 cases

Chevrant-Breton, J.

doi:10.1001/archderm.113.2.161

Cited by 20 publications

(8 citation statements)

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“…Hyperpigmentation may be one of the earliest signs of disease and has been reported in up to 90% of patients in one study . Skin pigmentation occurs due to melanin and/or iron deposition in the basal layer of the epidermis and around sweat glands.…”

Section: Clinical Manifestations Of Iron Overloadmentioning

confidence: 94%

Review article: the iron overload syndromes

Siddique

Kowdley

2012

Aliment Pharmacol Ther

123

122

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Summary Background Iron overload syndromes encompass a wide range of hereditary and acquired conditions. Major developments in the field of genetics and the discovery of hepcidin as a central regulator of iron homeostasis have greatly increased our understanding of the pathophysiology of iron overload syndromes. Aim To review advances in iron regulation and iron overload syndrome with special emphasis on hereditary haemochromatosis, the prototype iron overload syndrome. Methods A PubMed search using words such as ‘iron overload’, ‘hemochromatosis’, ‘HFE’, ‘Non‐HFE’, ‘secondary iron overload’ was undertaken. Results Iron overload is associated with significant morbidity and mortality. Sensitive diagnostic tests and effective therapy are widely available and can prevent complications associated with iron accumulation in end‐ organs. Therapeutic phlebotomy remains the cornerstone of therapy for removal of excess body iron, but novel therapeutic agents including oral iron chelators have been developed for iron overload associated with anaemia. Conclusions Iron overload disorders are common. Inexpensive screening tests as well as confirmatory diagnostic tests are widely available. Increased awareness of the causes and importance of early diagnosis and knowledge of the appropriate use of genetic testing are encouraged. The availability of novel treatments should increase therapeutic options for patients with iron overload disorders.

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Section: Clinical Manifestations Of Iron Overloadmentioning

confidence: 94%

Review article: the iron overload syndromes

Siddique

Kowdley

2012

Aliment Pharmacol Ther

123

122

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“…Iron overload is a fairly common problem; yet, skin disease caused by iron overload rarely provokes visits to the dermatologist. A single report indicates an association between iron overload and thin, dry epidermis [61]. Another group has shown that iron overload in mice increases susceptibility to chemical carcinogenesis [18,62].…”

Section: Proposal To Harness Desquamation To Remediate Internal Toxinsmentioning

confidence: 99%

Epidermal desquamation

Milstone¹

2004

Journal of Dermatological Science

120

103

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Epidermal desquamation, a continuous but insensible bodily activity, is largely ignored unless the rate or amount of scale production becomes abnormal. It is the last topic to be considered in any serious discussion of epidermal growth and differentiation, but is becoming an increasingly fertile ground for investigation. This review summarizes: (a) methods for measuring desquamation; (b) variables that affect normal desquamation; (c) mechanisms of desquamation; (d) the role of desquamation in nutritional homeostasis; and (e) the role of desquamation as a first line of defense. Consideration is given to whether desquamation might be harnessed to eliminate or remediate toxins that have accumulated in the body.

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“…Skin from humans with hemochromatosis reportedly shows increased iron (Magnuson and Raulston, 1942), and an epidermis that is hyperkeratotic, atrophic, and pigmented (Cawley et al, 1969;Chevrant-Breton et al, 1977). These conclusions are drawn from surveys that made no distinction between genetic, dietary, or hemolytic causes for hemochromatosis.…”

mentioning

confidence: 99%

Iron in Skin of Mice with Three Etiologies of Systemic Iron Overload

Adams

Lazova

Andrews

et al. 2005

Journal of Investigative Dermatology

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In human hemochromatosis, tissue toxicity is a function of tissue iron levels. Despite reports of skin toxicity in hemochromatosis, little is known about iron levels in skin of individuals with systemic iron overload. We measured skin iron and studied skin histology in three mouse models of systemic iron overload: mice with a deletion of the hemochromatosis (Hfe) gene, mice fed a high iron diet, and mice given parenteral injections of iron. In Hfe(-/-) mice, iron content in the epidermis and dermis was unexpectedly the same as in Hfe(+/+) mice, and there were no histological abnormalities detected after 30 wk. A high iron diet produced increased iron in the epidermis of both normal and Hfe(-/-) animals; a high diet increased iron in the dermis only in Hfe(-/-) mice. Increased skin iron was not associated with other histological changes, even after 19 wk. Parenteral administration of iron produced increased iron in the epidermis and dermis, and gave the skin a bronze hue. These results show that the amount and distribution of iron in the skin depends on the etiology of iron overload. It appears that neither Hfe deletion nor elevated skin iron alone can account for cutaneous manifestations reportedly seen in humans with hereditary hemochromatosis.

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Cutaneous manifestations of idiopathic hermochromatosis.Study of 100 cases

Cited by 20 publications

References 0 publications

Review article: the iron overload syndromes

Review article: the iron overload syndromes

Epidermal desquamation

Iron in Skin of Mice with Three Etiologies of Systemic Iron Overload

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