A S H VI N G A R L A P A T I , D . O .P R O G R A M D I R E C T O R : S T A N L E Y S K O P I T , D O , M S E , F A O C D

L A R K I N C O M M U N I T Y H O S P I T A L / N S U - C O M

A O C D M I D Y E A R M E E T I N G

A P R I L 2 3 - 2 6 2 0 1 5

ZOSTERIFORM LICHEN PLANUS: AN UNUSUAL

CLINICAL VARIANT

DISCLOSURES

No Financial Relations

with

Commercial Interests

OBJECTIVES

• Case presentation

• Introduction

• Epidemiology

• Clinical

• Pathogenesis

• Differential Diagnosis

• Histopathology

• Treatment and Prognosis

CASE PRESENTATION

• 52-year-old woman w/ six-week history of a pruritic

eruption on her right leg

• PMHX: Discoid Lupus Erythematosus (DLE). No prior

hx of herpes zoster

• No significant family or medication history

• Hepatitis C serology was negative

PHYSICAL EXAMINATION

• Purple pruritic papules and plaques with an

overlying white scale present on her right thigh

• The lesions were arranged in a linear pattern in the

L1, L2, and L3 dermatomal distribution

• A lacy white patch was seen on her left buccal

mucosa

• There was no scalp or nail involvement

DIAGNOSIS

• Histopathology: Wedge shaped hypergranulosis,

acanthosis, saw-tooth rete ridges, and a lichenoid

infiltrate

• A diagnosis of Zosteriform Lichen Planus was made

based on the clinical and pathological correlation

TREATMENT

• Intramuscular injection of Kenalog 40mg

• Tacrolimus ointment 0.1%, which was applied to the

cutaneous and oral lesions twice daily

INTRODUCTION

• Lichen Planus(LP): Papulosquamous skin disorder

with several morphological variants.

• Zosteriform LP may arise:

De novo

At sites of trauma (koebnerization)

Wolf’s isotopic response at the site of healed zoster

• Zosteriform type is an uncommon variant of lichen

planus with dermatomal or zonal distribution.

EPIDEMIOLOGY

• Prevalence of LP is 0.22% to 5% worldwide

• Average age of onset is 50 years old

• No racial predilection

• Females slightly more affected often than males

• The mucous membranes are affected in 65% of cases

• Oral LP: Wickham's striae may develop into squamous cell carcinoma(SCC) in 0.2% of cases

• Zosteriform LP is an atypical presentation of linear LP. Linear LP accounts for less than 1% of cases

CLINICAL

• Purple polygonal pruritic flat-topped papule and plaques. May have wickham's striae on papules

• Over 20 variants of lichen planus

• Linear LP refers to lichen planus with a unilateral linear distribution

• Can present in segmental distribution corresponding to a dermatome referred to as zosteriform lichen planus

• Zosteriform lichen planus: Wolf’s isotopic response at areas of healed zoster

Secondary to Koebnerization from trauma

De novo eruption on previously normal skin

PATHOGENESIS

• Lesions arranged in band several centimeters wide

and run along the course of a peripheral cutaneous

nerve and its branches

Triggered by neural factors

Suggested that the lesions in zosteriform LP actually follow

the Lines of Blaschko rather than an actual dermatome

Blaschkoid lines are invisible lines in the skin that are

believed to trace the migration of embryonic cells

Some believe that true zosteriform LP only occurs if lesions

develop at sites of healed herpes zoster

DIFFERENTIAL DIAGNOSIS

• Linear psoriasis

• Lichen striatus

• Linear epidermal nevus

• Linear darier’s disease

• Inflammatory linear verrucous epidermal

nevus(ILVEN)

• Lichen simplex chronicus

• Lichenoid drug eruption

• Lichenoid mycosis fungoides

HISTOPATHOLOGY

• Band-like lymphohistiocytic infiltrate at the dermal-epidermal junction(DEJ) and upper dermis. Wedge-shaped hypergranulosis and acanthosis with saw-toothed rete ridges

Wickham’s Striae seen in areas of hypergranulosis

• Max Joseph Spaces: Vacuolar degeneration at the basal layer leading to focal subepidermal clefts

• Civatte Bodies: Eosinophilic remnants of anucleateapoptotic basal cells found in the dermis

• Squamatization: Maturation and flattening basal layer cells

DIRECT IMMUNOFLUORESCENCE

• Direct Immunofluorescence(DIF):

Shaggy fibrin, cytoid bodies, and deposition of IgM immunoglobulins at the DEJ

Can distinguish LP from hypertrophic lupus erythematosus

(continuous granular band of IgG, IgM, IgA, and C3 at the

DEJ on DIF)

TREATMENT AND PROGNOSIS

• Treatments aimed to induce remission and relieve

associated pruritus

• First line therapy:

High potency topical steroids

• Systemic corticosteroids can be used as a second

line treatment or in those with more extensive

disease

TREATMENT AND PROGNOSIS

• Alternative treaments include

Immunosuppressive agents

Cyclosporine

Methotrexate

Azathioprine

Dapsone

Topical Tacrolimus

Narrow-band Ultraviolet B phototherapy. Psoralen plus ultraviolet A (PUVA)

Enoxaprarin Sodium

Oral Metronidazole

TREATMENT AND PROGNOSIS

• Treatment of pruritus• Oral antihistamines

• Diphenhydramine • Hydroxyzine

• Topical antipruritic agents • Menthol• Camphor• Pramoxine • Doxepin

• Prognosis: • Often resolves over average period of 18 months

• Approximately 20% of patients will have a second occurrence.In a subset of patients, the disease may persist for many years.

• Oral LP more therapy resistant, and close follow up advised given increased risk for SCC

REFERENCES

• 1. Pai K, Pai S. Zosteriform Lichen Planus: Case report of a rare variant of lichen planus. Our Dermatol Online. 2013; 4(2): 183-184.

• 2. Turel A, Ozturkcan 5, Sahin MT, TUrkdogan P. Wolf's isotopic response: a case of zosteriform lichen planus.J Dermatol 2002; 29: 339-42.

• 3. Scully C, Beyli M, Ferreiro MC, et al. Update on oral lichen planus: etiopathogenesis and management. Crit Rev Oral Biol Med 1998;9:86-122.

• 4. Boyd AS, Neldner KH. Lichen planus. J Am Acad Dermatol. 1991 Oct; 25(4): 593-619

• 5. Happle R: Zosteriform’ Lichen planus: Is it Zosteriform?Dermatology. 1996;192:385-6.

• 6. Turel A, Ozturkcan S, Ozturkcan S, Sahin MT: Wolf’s isotopic response: a case of zosteriform lichen planus. J Dermatol.2002;29:339-42.

• 7. Braun RP, Barua D, Masouye I: Zosteriform lichen planus after herpes zoster. Dermatology. 1998;197:87-8.

• 8. Perry D, Fazel N. Zosteriform lichen planus. Dermatology Online Journal 2006 Sep 8;12: (5): 3

• 9. Elston, Dirk M., and Tammie Ferringer. “Interface Dermatitis.” Dermatopathology. Edinburgh: Saunders/Elsevier, 2009. 135-139. Print.

• 10. Taneja A, Taylor CR. Narrow-band UVB for lichen planus treatment. Int J Dermatol. 2002 May;41(5):282-3.

• 11. Sugerman PB, Savage NW. Oral lichen planus: Causes, diagnosis and management. Australian Dental Journal 2002;47:(4):290-297.

• 12. James WD, Berger TG, Elston DM, eds. Andrews' Diseases of the Skin: Clinical Dermatology. 11th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 12, Lichen Planus and Related Conditions; p. 213-218.