A Boy With a Macular, Golden-Brown Patch

AUTHORS:
Kush S. Patel, BS; Richard H. Flowers, MD; and Barbara B. Wilson, MD
University of Virginia School of Medicine

A 4-year-old boy presented with a 1.5-year history of a rash on his right buttock. There was no associated tenderness, pruritus, or erythema, and his mother noted that the lesion had been growing slightly since its onset. A review of systems was negative for any constitutional or integumentary symptoms, and the boy’s family history was unremarkable. 

On physical examination, there was a 3.5 × 2 cm macular, golden-brown patch with scattered petechiae (“cayenne pepper”-like) on the right buttock (Figure 1). The results of the remainder of the examination were unremarkable. 

What is causing the boy’s rash? 

(Answer and explanation on the next page.)

Answer: Lichen aureus

The boy was diagnosed as having lichen aureus, a rare,
asymptomatic skin disease first described by Marten¹ in 1958, and later classified into the group of pigmented purpuric dermatoses (PPDs). Unlike the other PPDs, which are seen more frequently in adults, many cases of lichen aureus have been reported in children.^2-7  

Lichen aureus is characterized by the presence of petechiae, purpura, and increased skin pigmentation, which are found most commonly as localized lesions on the lower extremities.⁸ In children, lichen aureus has been reported more frequently in uncommon sites such as the trunk, abdomen, arms, and face.^2,4,7,9 The lesions tend to have an acute onset, presenting with distinct yellow-bronze or gold-colored circumscribed patches that often resemble a bruise.¹ 

The exact etiology of lichen aureus is unknown, though cases have been reported in association with vascular abnormalities of the skin,¹⁰ drugs,^11,12 energy drink consumption,⁵ and contact with allergens.¹³ The microscopic findings associated with PPDs include a perivascular lymphocytic infiltrate, erythrocyte extravasation, and hemosiderin deposition within the dermis. Lichen aureus differs from the other PPDs in that it is characterized by a dense lichenoid infiltrate within the superficial dermis, in addition to a marked accumulation of hemosiderin-laden macrophages.⁸ 

Diagnosis and Management

The diagnosis is made largely through clinical inspection, with biopsy reserved for uncertain cases. Additionally, biopsies may be useful in distinguishing lichen aureus from similar lesions such as cutaneous vasculitides or from simple bruises.  

Topical corticosteroids have often been used in the initial treatment of lichen aureus to varying degrees of success.^3,4,9,14,15 Other more recently employed treatment modalities include psoralen-UV-A,¹⁶ topical tacrolimus,¹⁷ and pentoxifylline/prostacyclin.¹⁸ However, many cases are left untreated due to the asymptomatic nature of the condition.²  

Disease in children tends to be self-limited; a case series of 8 patients found complete resolution of the lesion in 5 patients, reduced features in 2, and chronic persistence in one.² This is in contrast to adults, in whom lesions typically persist for several years and, in certain instances, may be progressive.^8,14 Lastly, an association between lichen aureus and mycosis fungoides (MF) has been documented in the past,^19-22 though recent findings suggest that most conventional cases of lichen aureus do not progress to MF.²³ 

Given our clinical certainty of the diagnosis, no biopsy was performed on our patient. The boy’s mother was asked to follow up in 1 year and monitor for new symptoms, or changes in the size or appearance of the lesion. This is especially critical in light of the proposed association between lichen aureus and MF. 

Kush S. Patel, BS, is a medical student at the University of Virginia School of Medicine in Charlottesville, Virginia.

Richard H. Flowers, MD, is a dermatology resident at the University of  Virginia School of Medicine in Charlottesville, Virginia.

Barbara B. Wilson, MD, is an associate professor of dermatology at the University of Virginia School of Medicine in Charlottesville, Virginia. 

REFERENCES

1. Marten RH. Case for diagnosis. Trans St Johns Hosp Dermatol Soc. 1958;40:98.

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14. Graham RM, English JS, Emmerson RW. Lichen aureus—a study of twelve cases. Clin Exp Dermatol. 1984;9(4):393-401.

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16. Ling TC, Goulden V, Goodfield MJ. PUVA therapy in lichen aureus. J Am Acad Dermatol. 2001;45(1):145-146.

17. Murota H, Katayama I. Lichen aureus responding to topical tacrolimus treatment. J Dermatol. 2011;38(8):823-825.

18. Lee H, Lee D, Chang S, et al. Segmental lichen aureus: combination therapy with pentoxifylline and prostacyclin. J Eur Acad Dermatol Venereol. 2006;20(10):1378-1380.

19. Ugajin T, Satoh T, Yokozeki H, Nishioka K. Mycosis fungoides presenting as pigmented purpuric eruption. Eur J Dermatol. 2005;15(6):489-491.

20. Martinez W, del Pozo J, Vázquez J, et al. Cutaneous T-cell lymphoma presenting as disseminated, pigmented, purpura-like eruption. Int J Dermatol. 2001;40(2):140-144.

21. Barnhill RL, Braverman IM. Progression of pigmented purpura-like eruptions to mycosis fungoides: report of three cases. J Am Acad Dermatol. 1988;19(1):25-31.

22. Toro JR, Sander CA, LeBoit PE. Persistent pigmented purpuric dermatitis and mycosis fungoides: simulant, precursor, or both? A study by light microscopy and molecular methods. Am J Dermatopathol. 1997;19(2):108-118.

23. Fink-Puches R, Wolf P, Kerl H, Cerroni L. Lichen aureus: clinicopathologic features, natural history, and relationship to mycosis fungoides. Arch Dermatol. 2008;144(9):1169-1173.