Dermatology Online Journal

The evolution of clinicopathologic features in eruptive lichen planus: A case report and review of literature
Kuei-Chung Liu, Julia Yu-Yun Lee, Mark Ming-Long Hsu, Chao-Kai Hsu
Dermatology Online Journal 19 (1): 8

Department of Dermatology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University

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Abstract

“Eruptive” or “exanthematous” lichen planus (LP) is a rare variant of lichen planus. Here we report a middle-aged woman with a 6-month history of episodic eruptive LP presenting as generalized, erythematous, flat-topped, round, polygonal, or umbilicated papules and hyperpigmented macules. The disease was under good control with continuous low-dose prednisolone over one year. We had the opportunity to correlate the clinical and pathologic findings based on histopathologic examination of three separate skin lesions that appeared to represent different stages of evolution. Recognition of these 3 chronological manifestations (polygonal papule in active inflammation, centrally umbilicated papule in involution, and hyperpigmented macule in resolution) can aid the diagnosis.

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Introduction

Lichen planus is a chronic inflammatory mucocutaneous disease of unknown etiology [1]. It typically manifests as pruritic, faintly erythematous to violaceous, flat-topped, polygonal papules distributed mainly over the flexural areas of wrists, arms, and legs. The coalescence of individual papules into plaques, the presence of fine white lines on the surface of skin lesions, and the hyperpigmentation after resolution are also characteristic [1]. Herein we report an adult female presenting with eruptive lichen planus who manifested a chronological evolution of the clinical and pathological findings.

Case report

Figure 1

A 41-year-old woman presented with numerous asymptomatic erythematous papules over the trunk and four extremities for 6 months (Figure 1). Examination revealed the presence of lesions of different morphology that appeared to represent three stages of evolution. In the first stage, the lesions consisted of erythematous flat-topped or dome-shaped papules (Figure 2a). In the second stage, the lesions became umbilicated with dusky erythematous or violaceous hue (Figure 2b). In the third stage, the lesions became flattened with hyperpigmentation (Figure 2c). The patient reported recurrent episodes and each episode lasted for 3 to 4 weeks. The medication history was unremarkable and she denied any constitutional symptoms such as fever, malaise, arthralgia, or body weight loss. There were no mucosal lesions found in the oral or genital areas.

The results of laboratory examinations including biochemistry, hemogram, and thyroid function were within normal range; serology tests of hepatitis B and C virus also showed negative results. Skin biopsy was performed on three lesions representing the chronological evolution of the eruption as described above.

Figure 2

Histopathological examination of first stage and the border of the second stage skin lesions (Figures 2d and 2e) showed epidermal hyperplasia with wedge-shaped hypergranulosis and a saw-toothed appearance of the undersurface of the epidermis in which there were necrotic keratinocytes and vacuolar alteration in the basal layer. In the dermis there was a dense, band-like lymphocytic infiltrate. The umbilicated portion of the second stage of skin lesion (Figure 2e, arrowhead) showed resolution of interface and lichenoid dermatitis with mild epidermal atrophy. The pathological findings of the final stage of skin lesion (Figure 2f) revealed an atrophic epidermis and melanophages in the papillary dermis with a sparse superficial perivascular lymphocytic infiltrate. Based on the clinicopathologic findings, the diagnosis of eruptive lichen planus was made. After a one-week treatment with oral prednisolone 10 mg twice daily and strong-potency topical steroid agents, the papules subsided gradually, leaving mild hyperpigmentation. Recurrence was prevented with prednisolone 10 mg daily in the following 1 year.

Discussion

Lichen planus is a chronic mucocutaneous disease with several variants defined by the configuration of lesions, the morphological appearance, and the site of involvement [1, 2]. To our knowledge, the eruptive or exanthematous variant of lichen planus is rarely reported in the English-language literature, especially among adults (Table 1) [3-9].

In the present case, the diagnosis of LP was confirmed by the typical pathologic features of LP in the actively inflamed lesion with a dense lichenoid lymphohistiocytic infiltrate (Figure 2a). Interestingly, the central portion of the umbilicated papule revealed much less inflammatory infiltrate (Figure 2b), suggesting that resolution of eruptive LP started from the center of the papule. The clinicopathological findings of the third stage (Figure 2c) were those of a resolved lesion with post-inflammatory hyperpigmentation. The findings observed in the 3 lesions in the present case demonstrated well “the lives of lesions” of eruptive LP and recognition of these sequential changes is important to make the correct diagnosis as emphasized by Ackerman [10].

The differential diagnoses of the disseminated and discrete papules or macules in this case include papular umbilicated granuloma annulare (GA), lichenoid drug reaction, atypical pityriasis rosea, verruca plana, pityriasis lichenoides and lichen scrofulosorum. The lesions of papular umbilicated GA are mostly localized on the dorsa of the hands and feet and the pathological finding of the central umbilication is focal collagen degeneration, which was not seen in our case [11, 12].

Various precipitating factors, including drugs and viral infections, are known to play an important role in the pathogenesis of forms of LP. Recently, Fleming J et al. reported a 41-year-old healthy woman who developed a widespread eruptive lichen planus following acupuncture treatment [9]. However, no obvious precipitating factors could be elicited in our patient.

Treatment of generalized lichen planus includes systemic corticosteroid, retinoids, cyclosporine, photochemotherapy, hydroxychloroquine, azathioprine, or other immunosuppressants [1, 3, 6]. Systemic steroid in doses greater than 20 mg/day (e.g., 30 to 80 mg prednisolone) for 4 to 6 weeks with a subsequent taper over 4 to 6 weeks is recommended as the first line therapy [1]. Other choices may be reserved for refractory lesions. Our patient’s LP was controlled well by low-dosed oral prednisolone, but the rash had a tendency to flare up after stopping steroid treatment.

In conclusion, we described clinical and histologic evolution of the skin lesions of eruptive LP. Recognition of these chronological changes will help us to make correct diagnosis.

Acknowledgement: We would like to thank Dr. Heather Ann Long, Institute of Cellular Medicine, Newcastle University, England, for helping us revise the grammar of the manuscript.

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