Print version ISSN 0365-0596
An. Bras. Dermatol. vol.86 no.4 supl.1 Rio de Janeiro July/Aug. 2011
Líquen escleroso e atrófico acral - relato de caso
Fernanda de Oliveira VianaI; Luíza Helena dos Santos CavaleiroI; Deborah Aben Athar UngerII; Mario Fernando Ribeiro de MirandaIII; Arival Cardoso de BritoIV
IMD, Resident, Dermatology Department, Federal University of Pará (UFPA), Belém, Brazil
IIMA in Tropical Diseases awarded by the Federal University of Pará (UFPA), Associate Professor of Dermatology at Federal University of Pará (UFPA), Belém, Brazil
IIISpecialist in Dermatopathology certified by the International Committee for Dermatopathology (ICDP) and the Union Européenne des Médecins Spécialistes (UEMS). Title of Specialist in Dermatology awarded by the Brazilian Association of Dermatology (SBD). Associate Professor of Dermatology at the Federal University of Pará (UFPA). Responsible for the dermatopathology laboratory at the Dermatology Department, Federal University of Pará (UFPA), Belém, Brazil
IVPh.D., Senior Professor of Dermatology, Federal University of Pará (UFPA). Professor in the Post-Graduate Tropical Diseases Program in the Tropical Medicine Institute, Federal University of Pará. Professor in the Biology of Infectious and Parasitic Agents Post-Graduate Program, Institute of Biological Sciences, Federal University of Pará. Head of Dermatology Department, Federal University of Pará, Belém, Brazil
Lichen sclerosus et atrophicus is a chronic disorder of the skin and mucosal surfaces, most commonly affecting the female genitalia. Few cases of lichen sclerosus et atrophicus confined to the feet and/or hands have been reported. We report a case of lichen sclerosus et atrophicus involving first the extremities and then the perigenital area.
Keywords: Sclerosis; Lichen sclerosus et atrophicus; Foot
O líquen escleroso e atrófico é uma doença crônica da pele e mucosas, que, usualmente, afeta a área genital de mulheres. Poucos casos de líquen escleroso e atrófico, confinados nos pés e/ou mãos, têm sido relatados. Relatamos um caso de líquen escleroso e atrófico envolvendo as extremidades e, posteriormente, a área perigenital.
Palavras-chave: Esclerose; Líquen escleroso e atrófico; Pé
Lichen sclerosus et atrophicus (LEA) is an uncommon chronic inflammatory disease which particularly affects the genital area of adult women. 1,2,3 Extragenital forms of the disease are rare, mainly affecting the thoracic region. 2 We describe a rare case of extragenital LEA which appeared initially in the palmoplantar region.
57-year-old female patient, born and resident in Belém, complaining of clear spots on the soles of the feet, accompanied by localized burning, which emerged two years previously. Patient had tried several topical medications such as desonide, calcipotriol, pimecrolimus and clobetasol oral prednisone, without improvement. A dermatological examination showed the presence of hypochromic papules located on the soles of the feet and also bilaterally in the paranasal sinuses (Figures 1 and 2). Some of the papules were isolated, while others were grouped in plaques, with areas of scaling. Similar lesions could also be seen in smaller numbers on the palms. The disorder was diagnosed as Flat Wart and a biopsy of the lesion was performed. Histopathology revealed epidermal hyperkeratosis, flattened interpapillary crests, scaling of interface areas, a band-like lymphocytic inflammatory infiltrate on the upper dermis and homogenization of the connective tissue with dilated vessels separating the epidermis from the infiltrate (Figure 3). Given that these findings were consistent with LEA, we prescribed tacrolimus 0.1% to be applied in the mornings and clobetasol cream at night. The patient returned three months later showing improved appearance of the lesions on the feet and less burning, but presenting erythematous, hypopigmented, shiny, well- defined, chaffed lesions of varying sizes in the inguinal region (Figure 4). We decided to biopsy the new lesions, the results of which were consistent with LEA.
LEA is a chronic, benign and uncommon inflammatory disease. It is more prevalent in females (ratio of 6-10 women to one man). 1,2 Higher incidence has been observed in white women aged around 40. The etiology is unknown but it is thought that genetic susceptibility plays a role in the disease. LEA is however closely related to autoimmune diseases and immunogenetic studies have revealed a link with HLA-DQ74,5,6 Some authors have advanced the theory that the LEA and scleroderma could be facets of the same disease, but the subject remains controversial. 7 It occurs predominantly in the genital area (in 83-98% of all cases), with extragenital lesions occurring in between 8% to 20% of cases. 1,2,3,8
The genital form of the disease in women is called craurose vulvar. This manifests as extremely puriginous lesions forming painful erosions, vulvar atrophy with progressive narrowing of the vaginal opening and dispaurenia. 1, 3 In men, the genital form is known as balanitis obliterans xerotica, presenting as painful itching, erosions and fissures. 1 LEA presents clinically as macules, papules and shiny white plaques with follicular corneal plugs. The extragenital forms focus on the upper anterior torso, neck, arms and legs, ear, face, oral mucosa and nose. 2,9 Other even more unusual locations are the palmoplantar region, nipples and scalp. 1.10 LEA may be asymptomatic or cause itching and burning at the site. 2 Diagnosis is clinical and histopathological. Treatment is with high-potency topical steroids, immunomodulators, systemic retinoids, estrogen and progesterone. 1,3,11 Other options include cryosurgery, vulvectomy, CO2 laser, but these have high recurrence rates. 3 Our case of extragenital LEA, involving the palmoplantar region and proceeding to the groin area, is extremely rare. Only one other case has been reported in the literature. 10
1. Coelho WS, Diniz LM, Souza Filho JB. Líquen escleroso e atrófico - relato de dois casos de apresentação atípica. An Bras Dermatol. 2006;81(5Supl3):S297-300. [ Links ]
2. Preciado MM, Almeida JGD, Romo EM. Liquen escleroso y atrófico extragenital. Rev Cubana Med. 2003;42:36-3. [ Links ]
3. Smith RS, HaefnerHK. Vulvar Lichen Sclerosus: Pathophysiology and Treatment. Am J Clin Dermatol. 2004;5:105-25. [ Links ]
4. Powell J, Wojnarowska F, Winsey S, Marren P, Welsh K. Lichen sclerosus premenar che: autoimmunity and immunogenetics. Br J Dermatol. 2000;142:481-4. [ Links ]
5. Azurdia RM, Luzzi GA, Byren I, Welsh K, Wojnarowska F, Marren P, et al. Lichen scle rosus in adult men: a study of HLA associations and susceptibility to autoimmune disease. Br J Dermatol. 1999;140:79-83. [ Links ]
6. Marren P, Yell J, Charnock FM, Bunce M, Welsh K, Wojnarowska F. The association between lichen sclerosus and antigens of the HLA system. Br J Dermatol 1995; 132: 197-203. [ Links ]
7. Lampert A, Fortier-Beaulieve M, Thomine E, Young P, Laurent P. Association of lichen sclerosus and monomelic scleroderma. Ann Dermatol Venereol. 1995;122:102-4. [ Links ]
8. Meyrick Thomas RH, Ridley CM, McGibbon DH, Black MM. Lichen sclerosus et atrophicus and autoimmunity - a study of 350 women. Br J Dermatol. 1988;118:41-6. [ Links ]
9. Kaur S, Thami GP, Kanwar AJ, Mohan H. Linear oro-facial lichen sclerosus. Clin Exp Dermatol. 2002;27:467-70. [ Links ]
10. Steff M, Toulemonde A, Croue A, Lemerle E, Le Corre Y, Verret JL. Acral lichen sclerosus et atrophicus. Ann Dermatol Venereol. 2008;135:201-4. [ Links ]
11. Assmann T, Becker-Wegerich P, Grewe M, Megahed M, Ruzicka T. Tacrolimus ointment for the treatment of vulvar Lichen Sclerosus. J Am Acad Dermatol. 2003;48:935-7. [ Links ]
Mailing address: Received on 28.12.2010. * Study undertaken at the Department of Dermatology, Federal University of Pará (UFPA) - Belém, Brazil.
Fernanda de Oliveira Viana
Rua Tibúrcio Cavalcante - 2777 - Apt. 702
60125 101 Fortaleza CE, Brazil
Approved by the Advisory Board and accepted for publication on 14.02.2011.
Conflict of interest: None
Financial funding: None
Received on 28.12.2010.
* Study undertaken at the Department of Dermatology, Federal University of Pará (UFPA) - Belém, Brazil.