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Yugal K. Sharma, Neha C. Virmani, Kedar N. Dash, Kirti S. Deo, Vidya Mave, Amita Gupta, Arjun Kakrani, A 23-Year-Old Man Presenting With Fluid-Filled Skin Lesions, Patchy Pigmentation, and Skin Breakage After Trivial Trauma, Clinical Infectious Diseases, Volume 56, Issue 6, 15 March 2013, Pages 898–899, https://doi.org/10.1093/cid/cis996
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(See page 851–2 for the Photo Quiz.)
Diagnosis: Porphyria cutanea tarda in a newly diagnosed AIDS patient.
Histopathological examination of the bulla revealed a subepidermal cleavage with “festooning” of dermal papillae, mild dermal lymphocytic infiltrate, and linear eosinophilic 1globules (“caterpillar bodies”) in the blister roof resulting from degenerating basement membrane material and keratinocytes (Figure 1). Periodic acid-Schiff staining accentuated the caterpillar bodies as well as thickened upper dermal capillary walls. Wood's light examination of the urine revealed coral pink fluorescence. Results of a 24-hour urine porphobilinogen were within normal limits. Absence of any neurovisceral involvement along with the above-mentioned clinical and histopathological findings confirmed the diagnosis of porphyria cutanea tarda (PCT).
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Porphyrias, a group of rare acquired or hereditary diseases, occur due to deficiency or decreased activity of one of the 8 enzymes in the heme biosynthesis pathway [1]. PCT, first described in 1911, is the commonest of all porphyrias and occurs due to the deficiency of the fifth enzyme, uroporphyrinogen decarboxylase. Type I PCT is sporadic, has adult onset with enzymatic deficiency limited to the liver, and is more common than the rare familial type II, which commences during childhood and has enzymatic deficiency that involves all tissues including red cells [1–3]. The most recognized factors that precipitate sporadic PCT are ethanol abuse, hepatic siderosis, exposure to toxins (eg, polychlorinated biphenyls, hexachlorobenzene, and 2,3,7,8-tetrachlorodibenzo-p-dioxin), several medications (anticonvulsants, iron supplements, sulfonamides, estrogens, zidovudine, or indinavir) and viral infections (hepatitis C virus and probably human immunodeficiency virus [HIV]) [2, 3].
