Photo Quiz

Hyperpigmented Patches on the Dorsal Hands

 

Am Fam Physician. 2018 May 1;97(9):603-604.

A 50-year-old man presented with skin lesions on his upper extremities (Figures 1 and 2) that developed two months earlier. He thought they may have appeared following a spider bite. The lesions were located primarily over the dorsal aspects of the hands and consisted of hyperpigmented patches with one visible erosion. The patches were painful but not pruritic.

The patient's medical history included human immunodeficiency virus (HIV) infection, type 2 diabetes mellitus, alcohol use disorder, and tobacco use disorder. He was not taking any medications, including nonsteroidal anti-inflammatory drugs.

Physical examination revealed hyperpigmented patches on the malar region of the face with hypertrichosis (Figure 3). The patches were brownish and not raised.

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FIGURE 1


FIGURE 1

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FIGURE 2


FIGURE 2

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FIGURE 3


FIGURE 3

Question

Based on the patient's history and physical examination findings, which one of the following is the most likely diagnosis?

A. Epidermolysis bullosa acquisita.

B. Phytophotodermatitis.

C. Porphyria cutanea tarda.

D. Pseudoporphyria.

Discussion

The answer is C: porphyria cutanea tarda. Porphyria cutanea tarda is the most common type of porphyria and affects approximately one in 25,000 persons in the United States. It is a result of acquired inhibition of an enzyme (uroporphyrin decarboxylase or uroporphyrinogen decarboxylase) in the heme biosynthesis pathway of the liver.1,2 Porphyria cutanea tarda usually arises spontaneously, although accumulated iron, alcohol use, smoking, hepatitis C virus infection, excess estrogen exposure, and HIV infection are common inciting factors.

Reduced hepatic enzyme activity leads to accumulation of carboxylated porphyrinogens that are subsequently oxidized to photoactive porphyrins.3 Photodamage leads to the characteristic cutaneous features, including increased skin fragility, noninflammatory bullae and vesicles that scar, hyperpigmentation, and milia on sun-exposed areas.4 Increased fragility of the skin leads to erosions and blistering from trivial trauma, commonly on the hands.4 Hirsutism of the face and forearms is

Author disclosure: No relevant financial affiliations.

Address correspondence to Steven Feldman, MD, PhD, at sfeldman@wakehealth.edu. Reprints are not available from the authors.

References

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1. Green JJ, Manders SM. Pseudoporphyria. J Am Acad Dermatol. 2001;44(1):100–108....

2. Jalil S, Grady JJ, Lee C, Anderson KE. Associations among behavior-related susceptibility factors in porphyria cutanea tarda. Clin Gastroenterol Hepatol. 2010;8(3):297–302.

3. Anderson KE, Sassa S, Bishop DF, Desnick RJ. Disorders of heme bio-synthesis: X-linked sideroblastic anemias and the porphyrias. In: Scriver CR, Beaudet AL, Sly WS, Valle D, Stanbury JB, eds. The Metabolic and Molecular Basis of Inherited Disease. 8th ed. New York, NY: McGraw-Hill; 2001:2991.

4. Elder GH. Porphyria cutanea tarda and related disorders. In: Kadish KM, Smith KM, Guilard R, eds. The Porphyrin Handbook. San Diego, Calif.: Academic Press; 2003:67.

5. Khayat R, Dupuy A, Pansé I, Bagot M, Cordoliani F. Sclerodermatous changes in porphyria cutanea tarda: six cases [in French]. Ann Dermatol Venereol. 2013;140(10):589–597.

6. Iranzo P, Herrero-González JE, Mascaró-Galy JM, Suárez-Fernández R, España A. Epidermolysis bullosa acquisita: a retrospective analysis of 12 patients evaluated in four tertiary hospitals in Spain. Br J Dermatol. 2014;171(5):1022–1030.

7. Luke MC, Darling TN, Hsu R, et al. Mucosal morbidity in patients with epidermolysis bullosa acquisita. Arch Dermatol. 1999;135(8):954–959.

8. Cather JC, Macknet MR, Menter MA. Hyperpigmented macules and streaks. Proceedings (Bayl Univ Med Cent). 2000;13(4):405–406.

This series is coordinated by John E. Delzell Jr., MD, MSPH, Associate Medical Editor.

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