Erythematous patches on the hands

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Erythematous patches on the hands

J Fam Pract. 2012 February;61(2):101-104

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References

1. Michaels BD, Del Rosso JQ, Mobini N, et al. Erythropoietic protoporphyria: a case report and literature review. J Clin Aesthet Dermatol. 2010;3:44-48.

2. Wahlin S, Floderus Y, Stål P, et al. Erythropoietic protoporphyria in Sweden: demographic, clinical, biochemical and genetic characteristics. J Intern Med. 2011;269:278-288.

3. James WD, Berger TG, Elston DM. Errors in metabolism. In: James WD, Berger TG, Elston DM, eds. Andrews’ Diseases of the Skin: Clinical Dermatology. 11th ed. Philadelphia, Pa: Elsevier; 2011:511–515.

4. Murphy GM. Porphyria. In: Bolognia JL, Jorizzo JL, Rapini R, eds. Dermatology. 1st ed. Philadelphia, Pa: Elsevier; 2003:679–686.

5. Herrero C, To-Figueras J, Badenas C, et al. Clinical, biochemical, and genetic study of 11 patients with erythropoietic protoporphyria including one with homozygous disease. Arch Dermatol. 2007;143:1125-1129.

6. Usatine RP, Riojas M. Diagnosis and management of contact dermatitis. Am Fam Physician. 2010;82:249-255.

7. Yeh SW, Ahmed B, Sami N, et al. Blistering disorders: diagnosis and treatment. Dermatol Ther. 2003;16:214-223.

8. Kedrowski DA, Warshaw EM. Hand dermatitis: a review of clinical features, diagnosis, and management. Dermatol Nurs. 2008;20:17-25.

9. Gambichler T, Al-Muhammadi R, Boms S. Immunologically mediated photodermatoses: diagnosis and treatment. Am J Clin Dermatol. 2009;10:169-180.

10. Murphy GM. Diagnosis and management of the erythropoietic porphyrias. Dermatol Ther. 2003;16:57-64.

11. Lecluse AL, Kuck-Koot VC, van Weelden H, et al. Erythropoietic protoporphyria without skin symptoms–you do not always see what they feel. Eur J Pediatr. 2008;167:703-706.

12. Holme SA, Anstey AV, Finlay AY, et al. Erythropoietic protoporphyria in the U.K.: clinical features and effect on quality of life. Br J Dermatol. 2006;155:574-581.

13. Sies C, Florkowski C, George P, et al. Clinical indications for the investigation of porphyria: case examples and evolving laboratory approaches to its diagnosis in New Zealand. NZ Med J. 2005;118:U1658.-

Confirm your suspicions with testing

There should be a high degree of suspicion for EPP when any child or adult presents with photosensitivity.^10,11 The absence or delayed onset of visible lesions can make the diagnosis difficult and cause the patient considerable suffering.¹²

FAST TRACK

Suspect erythropoietic protoporphyria when any child or adult presents with photosensitivity.

If EPP is suspected, order lab work for confirmation. The most diagnostically useful test is the erythrocyte protoporphyrin level, which will be increased in patients with EPP. These levels can range from several hundred to several thousand μg/100 mL of packed red blood cells (normal= <35 μg/100 mL).³ In addition, elevated levels of protoporphyrin are found in the feces. However, unlike other porphyrias, the level of urine porphyrins is normal (because protoporphyrin IX is water-soluble).^1,3

Due to the incidence of hepatobiliary disease in EPP, liver enzymes should be drawn and the proper imaging performed if abnormal values are obtained. Biopsy of the involved skin, with histologic review, will also aid in the diagnosis if other testing is inconclusive.^1,4

Treatment: Protection from sun is key

While complete sun exposure avoidance would prevent most sequelae in EPP, this is not always a feasible option. Covering the skin with sun-protective clothing and using sunscreen that contains titanium oxide or zinc oxide are acceptable alternatives to overt avoidance^1,3,10,13 (strength of recommendation [SOR]: A). Beta-carotene at doses of 60 to 180 mg/d for adults or 30 to 90 mg/d for children to achieve a serum level of 600 mcg/dL has also been shown to be effective in reducing photosensitivity in some cases ^1,2,5 (SOR: B). Cysteine, antihistamines, phototherapy, pyroxidine, and vitamin C have also been used to treat EPP, but have demonstrated limited efficacy^1,3,10 (SOR: B).

Hepatobiliary complications. Treating hepatobiliary complications is dependent upon the degree of dysfunction. Cholestyramine can be used to increase the fecal excretion of protoporphyrins in liver dysfunction^1,3,10 (SOR: A). In severe liver dysfunction, blood transfusions can be utilized until a liver transplant can be performed^1,3,10 (SOR: A). Liver transplantation, however, does not correct the metabolic error, and damage to the transplanted liver can occur.¹

Erythematous patches on the hands

References

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