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Zosteriform lichen planus

  • Author(s): Perry, Danielle
  • Fazel, Nasim
  • et al.
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Zosteriform lichen planus
Danielle Perry MD, Nasim Fazel MD DDS
Dermatology Online Journal 12: (5): 3

University of California Davis Department of Dermatology. nasim.fazel@ucdmc.ucdavis.edu

Abstract

A 95-year-old woman presented with a 4-month history of a pruritic eruption involving her trunk, medial thighs, and lesions limited to the C5 dermatome of the left upper extremity. Punch biopsy supported a clinical diagnosis of zosteriform lichen planus. Linear lichen planus refers to lichen planus with a unilateral linear distribution. This variant may present as an example of the Wolf isotopic response, or more rarely, a de novo eruption on previously-normal skin. In very rare instances linear lichen planus presents in a segmental fashion corresponding to a dermatome and is termed zosteriform lichen planus.



Clinical synopsis

A 95-year-old woman presented to the University of California Davis department of dermatology with a 4-month history of a pruritic eruption that started on the left shoulder and subsequently spread to the left arm. Initially, the patient had been diagnosed with herpes zoster and treated with acyclovir without improvement. Her condition worsened at which time she was referred to dermatology for further evaluation. Her past medical history was significant for diabetes mellitus and for hypertension controlled with lisinopril and furosemide. She denied any history of oral or genital lesions; family history was noncontributory. A review of systems failed to reveal any history of fevers, weight loss, chills, or pain.


Figure 1Figure 2
Well demarcated, raised, violaceous papules localized to the C5 dermatome of the left arm

Figure 3
Orthokeratosis with rare necrotic keratinocytes in the epidermis and band-like lymphohistiocytic infiltrate with melanophages below the dermo-epidermal junction

Physical examination revealed multiple slightly hyperpigmented, violaceous, discrete, raised papules and plaques in a linear distribution localized to the C5 dermatome of the left arm (Figs. 1 and 2). The patient had similar lesions scattered over the back and thighs. A punch biopsy revealed a histopathologic diagnosis of lichen planus (Fig. 3). The patient was treated subsequently with triamcinolone ointment (0.1 % twice daily). Complete resolution was noted after 4 weeks of treatment with residual post-inflammatory hyperpigmentation.


Comment

Lichen planus (LP) is a relatively common disorder affecting middle-aged individuals. The average age of onset is about 50 years. It is evenly distributed world wide and affects females more than males. Clinically, the disorder presents as pruritic, flat-topped, violaceous, polygonal papules most commonly on the flexural surfaces. The mucous membranes are involved in up to 65 percent of cases and occasionally nails can be affected as well. If biopsied, histopathologic examination shows a band-like infiltrate of lymphocytes at the dermal-epidermal junction. Other salient features include hyperkeratosis, wedge-shaped hypergranulosis, acanthosis with saw-toothed rete ridges, and vacuolar degeneration of the basal layer. Civatte bodies (colloid or cytoid bodies) are present at the dermal-epidermal junction and in the papillary dermis.

Lichen planus can be sub-classified by the morphology and distribution of the lesions. Many atypical presentations of LP have been described, including actinic lichen planus, hypertrophic lichen planus, and vesiculobullous lichen planus [1]. These variants are identified by their unique morphologic features. Only rarely does lichen planus present with typical lesions in an atypical distribution [2]. For example, inverse LP may affect the groin, axillae, and inframammary folds [1]. In very rare cases, it can have a unilateral, segmental, or linear distribution. There are many hypothetical causes for this type of presentation. For example, linear lichen planus can arise secondary to Koebnerization from trauma in association with more generalized disease [3]. Also, dermatomal lichen planus can erupt following healed herpes zoster of the same location, an example of the Wolf isotopic response [4, 5, 6]. This isotopic response is the occurrence of a new skin disorder exactly at the site of another, unrelated and already resolved skin disease.

In extremely rare cases, linear or segmental distributions appear de novo on previously normal, non-traumatized skin, as in our patient. Although case reports of de novo dermatomal LP have been reported [7, 8, 9, 10], this entity is controversial. Happle argued that the term zosteriform lichen planus has been applied inappropriately in cases that actually arose de novo in the lines of Blaschko, rather than in true dermatomes [11, 12]. Some authors believe that true zosteriform LP does not exist except in cases arising on the site of healed herpes zoster [13]. However, Lutz presented two cases of zosteriform (i.e., dermatomal) lichen planus without evidence of preceding viral disease [10].

In our patient, the distribution of lesions followed the C5 dermatome. The patient denied prior history of herpes zoster. The linear eruption seemed to follow a true dermatome rather than in the pattern the lines of Blaschko form on the upper arm [14]. In many of these cases, it is difficult to differentiate the two. So it remains unknown if there are two separate forms of unilateral, de novo lichen planus, one type arising in the lines of Blaschko (Blaschkoian lichen planus) [15, 16], and the other arising within one or more dermatomes. In either case, these atypical distribution patterns may well provide clues to the pathogenesis of a condition with currently unknown etiology.

A variety of treatment modalities has been used to relieve pruritus and induce remission of lichen planus. These modalities included topical and systemic steroids, dapsone, cyclosporine, retinoids, methotrexate, azathioprine, narrow-band ultraviolet-B phototherapy and psoralen plus ultraviolet A (PUVA) [1, 17]. In instances with limited involvement topical steroids may be sufficient as was the case with our patient.

References

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