Disseminated hypertrophic lichen planus: relevant response to acitretin

Abstracts

O líquen plano hipertrófico é uma variante do líquen plano, com pronunciada hiperplasia epidérmica em resposta à coçadura persistente. Clinicamente, caracterizam-se por placas hiperceratósicas, simétricas, de coloração cinza-violácea, com predileção pela região pré-tibial. O prurido intenso, a refratariedade aos tratamentos convencionais e a possibilidade de associação de um carcinoma epidermoide às lesões de longa duração impõem um tratamento eficaz. Os corticoides são considerados o tratamento de primeira linha e podem ser aplicados topicamente ou empregados de forma sistêmica. Outras modalidades terapêuticas propostas são a fototerapia com UVB-NB ou PUVA, imunossupressores e retinoides sistêmicos, com destaque para a acitretina. Relatamos um caso com apresentação clínica exuberante e excelente resposta à acitretin, totalizando um seguimento de nove meses

Acitretina; Imunossupressores; Líquen plano; Prednisona

CASE REPORT

Disseminated hypertrophic lichen planus: relevant response to acitretin^*

Thais Jerez Jaime^I; Tatiana Jerez Jaime^II; Bianca de Mello Guaraldi^I; Daniel Fernandes Melo^III; Thiago Jeunon^IV; Claudio Lerer^V

^IThird-year post graduation student from the Dermatology course at the Marcílio Dias Naval Hospital - Rio de Janeiro (RJ), Brazil

^IIPreceptress at the Department of Dermatology from the University of Mogi das Cruzes (UMC) - Mogi das Cruzes (SP), Brazil

^IIIPreceptor of the Department of Dermatology at the Marcílio Dias Naval Hospital - Rio de Janeiro (RJ), Brazil

^IVPreceptor and Head of the Department of Dermatopathology of the Dermatology service from the Federal Hospital of Bonsucesso (HFB) - Rio de Janeiro (RJ), Brazil

^VPreceptor and head of the Dermatology Service at the Marcílio Dias Naval Hospital- Preceptor of the Professor Rubem David Azulay Dermatology Institute - Santa Casa da Misericórdia Hospital from Rio de Janeiro (IDPA - SCMRJ) - Rio de Janeiro (RJ), Brazil

^{Mailing address}

ABSTRACT

Hyperthrofic lichen planus is considered a variant of lichen planus with marked epidermal hyperplasia in response to persistent itch. It is clinically, characterized by symmetric hyperkeratotic plaques, of purplish-grey color, often located in the pretibial region. Intense pruritus, refractoriness to conventional treatments and the possibility of association of the long-term injuries with squamous cell carcinoma requires an effective treatment. The first-line treatment is corticosteroids which can be applied either topically or systemically. Other therapeutic modalities proposed are: NB-UVB phototherapy or PUVA, immunosuppressive drugs and systemic retinoids, notably acitretin. We report a case with exuberant clinical presentation of hyperthrofic lichen planus with excellent response to acitretin after nine months of treatment.

Keywords: Acitretin; Immunosuppressive drugs; Lichen planus; Prednisone

INTRODUCTION

Lichen planus (LP) is a chronic inflammatory thas a uniform distribution between the genders, with disease of unknown etiology that affects skin, attach-peak of incidence around 30 and 60 years of age. It is ments and occasionally ,mucosae. Most cases occur a frequently itchy dermatosis and it can present itself sporadically and the few cases of family involvement with annular, linear, bullous or atrophic lesions. ^1-3 seem to be related to the vertical transmission of hep-Hypertrophic lichen planus (HLP) represents the variatitis C or B viruses. Incidence of LP is higher in ety of LP in which the epidermis presents marked patients infected by these viruses that tend to have a hyperplasia, due to persistent itching, being considmore extense and resistant to treatment disease. LP ered by some authors as a superposition of a chronic simple lichen to the LP lesions.⁴ It is reported here a case of HLP with excellent response to oral treatment with acitretin.

CASE REPORT

Seventy-five year-old female patient, dark skinned, coming from Rio de Janeiro that five years before had presented sudden appearance of pruritic lesions exclusively on the back of the hands. The lesions became progressively verrucous, extending to the upper and lower limbs, lumbosacral region and buttocks. The patient reported previous treatment in another service with LCD 10% and steroids, with no improvement. Dermatological examination found verrucous plaques, confluent, with irregular borders and well defined limits, with purplish grey outskirts and hypochromic center, affecting upper and lower limbs and buttock. (Figures 1,2 and 3). The initial procedure consisted of skin biopsy and laboratory tests which showed an increase in erythrocyte sedimentation rate and a cicatricial seropositivity for hepatitis A. Histopathology revealed psoriasiform hyperplasia, with hyperkeratosis, hypergranulosis, vacuolar degeneration of basal layer and blurring of the dermoepidermal junction by lymphocytic infiltrate in the papillary dermis. (Figures 4 and 5 ). The confirmation of the hypothesis of hypertrophic lichen planus was firmed and the treatment was acitretin 40mg/day, hydroxyzine symptomatic and maintenance of topical LCD.

After 3 months of treatment it was observed partial improvement of the lesions, of the quality of life of the patient and pruritus. Today, in the ninth month of treatment there has been total resolution of the lesions on the upper limbs and partial resolution of the lesions on the lower limbs without recurrence up to this moment. The dose was decreased to 30 mg/day in the 5th month of treatment and 25 mg/day in the 7th month and it is being kept until the present date.

DISCUSSION

HLP usually presents itself clinically as papules and symetrical plaques, with lichenified and highly hyperkeratotic surface with coloration that varies from purple to gray having a predilection by the pretibial region. It is frequently associated to chronic venous insufficiency and it commonly causes residual hyper pigmentation or hypopigmentation when it evolves. There are reports of appearance of squamous cell carcinomas over HLP when they persist over time. Revision of the medical literature indicates the appearance of the disease approximately 12 years after the appearance of HLP. ^5-7

The diagnosis of HLP is morphologic, based on the clinical and histopathological aspects of the lesions. Microscopy reveals great psoriasiform hyperplasia, with bulbous epidermal cones blurred at the base by lymphocitic inflammatory infiltration, associated with keratinocytes with individual necrosis. Additionally, it is observed orthokeratotic hyperkeratosis, hypergranulosis and frequently vertical bundles of collagen in the dermal papillae denoting persistent itch.⁴ Its treatment is still unsatisfactory as it is only symptomatic. Systemical treatment of HLP is imperative when skin involvement is extensive being corticoid considered a drug of first choice.^1,5,8-12 In refractory cases or contraindication to the same acitretin, cutaneous cyclosporine, azathioprine, mycophenolate mofetil, ciclofosfamida and methotrexate and phototherapy are chosen. ^8-15

Acitretine is a half-life syntetic retinoid between 55 and 60 hours, time that can be increased in case of alcohol intake. Teratogenicity is among its most important side effects risk for which it is indicated oral contraception in childbearing women for 3 years after drug withdrawal. Hypertriglyceridemia as well as hypercholesterolemia and elevation of transaminases are also important changes caused by this medication being found that in 5 to 8% of the patients, but it is reversible with dose reduction. Mucocutaneous side effects are among the most prevalent as mucosa and skin xerosis, cheilitis and hair loss, but they are also dose dependent and reversible. Acitretine is used for different dermatological diseases having as its main effects the regulation of keratinization disorders such as psoriasis, ichthyosis, Darier's disease and palmoplantar keratodermas. More recently it was proposed to be used in very severe conditions and refractory cases of HLP and as an effective prevention of new cutaneous carcinomas, mainly the epidermoid ones, in predisposed patients as in the reported case beyoind its indication for carriers of xeroderma pigmentosum and immunosuppressed ones. ^5-8,9,13,14

It is reported here the case of an exuberant form of hypertrophic lichen planus for the extension of its dissemination besides its excellent response to the use of acitretine.

REFERENCES

Received on 17.08.2010.

Approved by the Advisory Board and accepted for publication on 28.09.2010.

Conflict of interest: None

Financial funding: None

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*

Work carried out at the Marcílio Dias Naval Hospital - Rio de Janeiro (RJ), Brazil.

Publication Dates

• Publication in this collection
  08 Nov 2011
• Date of issue
  Aug 2011

History

• Accepted
  28 Sept 2010
• Received
  17 Aug 2010