Acessibilidade / Reportar erro

Lichen sclerosus associated with localized scleroderma: dermoscopy contribution* * Study performed at Hospital Universitário Pedro Ernesto -Universidade do Estado do Rio de Janeiro (HUPE-UERJ) - Rio de Janeiro (RJ), Brazil.

Abstract:

Lichen sclerosus is an uncommon inflammatory dermatosis with preferential involvement of the urogenital region. The extragenital involvement is uncommon and is characterized by small rounded macules or papules, pearly white in color. The coexistence of lichen sclerosus and scleroderma plaques in most cases with extragenital location has been reported in the literature. We report a case of lichen sclerosus associated with scleroderma in children, highlighting the importance of dermoscopy in diagnosis.

Keywords:
Dermoscopy; Lichen sclerosus et atrophicus; Scleroderma localized

Female patient, 9 years old, white, with asymptomatic hypochromic lesions initially on the hands with subsequent progression to feet and knees, for about 8 months. Dermatological examination revealed polygonal, pearly papules, with parchment-like surface, which agglomerated on plaques in hands, feet and knees (Figure 1). Some of these lesions presented central blackish points. On the right wrist and dorsum of the feet there were poorly defined areas of hyperpigmentation and skin atrophy, intermingled with hypochromic lesions described above (Figure 2). General physical and laboratory examination showed no alterations. Dermoscopy of hand, feet and knees lesions showed multiple whitish areas, some surrounded by an erythematous halo, with the presence of whitish amorphous areas and pseudocomedones (Figure 3). On the clinical and dermoscopic findings the diagnosis of extragenital lichen sclerosus associated with localized scleroderma was established.

Figure 1
Pearly papules lesions, polygonal, confluent, with parchment-like surface, on the back of the hand

Figure 2
White-pearly papules surrounded by hyperchromic halo, poorly delimited, associated with areas of atrophy, on the back of the foot

Figure 3
Dermoscopy of the lesions on the back of the hand: multiple whitish areas, some surrounded by an erythematous halo, with the presence of whitish amorphous areas and pseudo-comedones

Lichen sclerosus (LS) was first described in the late nineteenth century by Hallopeau. 11 Coelho WS, Diniz LM, Souza Filho JB. Líquen escleroso e atrófico - relato de dois casos de apresentação atípica. An Bras Dermatol. 2006;81:S297-300. It is considered a chronic inflammatory dermatosis of the dermal papilla, which is characterized by the presence of asymptomatic white polygonal papules that coalesce to form a plaque with points corresponding to follicular pseudo-aperture. 22 Garrido-Ríos AA, Alvarez-Garrido H, Sanz-Muñoz C, Aragoneses-Fraile H, Manchado-López P, Miranda-Romero A. Dermoscopy of extragenital lichen sclerosus. Arch Dermatol. 2009;145:1468.

3 Sauder MB, Linzon-Smith J, Beecker J. Extragenital bullous lichen sclerosus. J Am Acad Dermatol. 2014;71:981-4.
-44 Stavrianeas NG, Katoulis AC, Kanelleas AI, Bozi E, Toumbis-Ioannou E. Extragenital lichen sclerosus of childhood presenting as erythematous patches. Indian J Dermatol Venereol Leprol. 2008;74:59-60. With time these lesions become increasingly atrophic with rough surface. 55 Fistarol SK, Itin PH. Diagnosis and treatment of lichen sclerosus: an update. Am J Clin Dermatol. 2013;14:27-47. Classically it occurs in the genital area, but it also has extragenital manifestations, with a variety of clinical presentations. 33 Sauder MB, Linzon-Smith J, Beecker J. Extragenital bullous lichen sclerosus. J Am Acad Dermatol. 2014;71:981-4. LS can exist in isolation in 6% of cases, or in association with genital LS in 15-20% of presentations. 33 Sauder MB, Linzon-Smith J, Beecker J. Extragenital bullous lichen sclerosus. J Am Acad Dermatol. 2014;71:981-4.

4 Stavrianeas NG, Katoulis AC, Kanelleas AI, Bozi E, Toumbis-Ioannou E. Extragenital lichen sclerosus of childhood presenting as erythematous patches. Indian J Dermatol Venereol Leprol. 2008;74:59-60.
-55 Fistarol SK, Itin PH. Diagnosis and treatment of lichen sclerosus: an update. Am J Clin Dermatol. 2013;14:27-47. The disease affects people of any age but is rare in childhood, with an estimated prevalence of at least 1 in 900 children. When present in children, the appearance of symptoms occurs between two and five years of age. 44 Stavrianeas NG, Katoulis AC, Kanelleas AI, Bozi E, Toumbis-Ioannou E. Extragenital lichen sclerosus of childhood presenting as erythematous patches. Indian J Dermatol Venereol Leprol. 2008;74:59-60.

The etiology is still not clear, but there is evidence of association with HLA Class I antigens and with HLA-A29/B44, infections by human papillomavirus (HPV) and spirochete Borrelia burgdorferi, autoimmune and hormonal phenomena, and trauma (through the Koebner phenomenon). 44 Stavrianeas NG, Katoulis AC, Kanelleas AI, Bozi E, Toumbis-Ioannou E. Extragenital lichen sclerosus of childhood presenting as erythematous patches. Indian J Dermatol Venereol Leprol. 2008;74:59-60.,66 Rose AE, Boyd KP, Meehan SA, Latkowski JA. Lichen sclerosus et atrophicus. Dermatol Online J. 2013;19:20714. There may be an overlap between LS and morphea, considered as two distinct entities. 44 Stavrianeas NG, Katoulis AC, Kanelleas AI, Bozi E, Toumbis-Ioannou E. Extragenital lichen sclerosus of childhood presenting as erythematous patches. Indian J Dermatol Venereol Leprol. 2008;74:59-60. A recent study with 472 patients indicates that there is a higher frequency of LS in patients with localized scleroderma than in the general population, suggesting a common physiopathological autoimmune mechanism.77 Kreuter A, Wischnewski J, Terras S, Altmeyer P, Stücker M, Gambichler T. Coexistence of lichen sclerosus and morphea: a retrospective analysis of 472 patients with localized scleroderma from a German tertiary referral center. J Am Acad Dermatol. 2012;67:1157-62.

Diagnosis is usually clinical and may be supplemented with dermoscopy, with no need for histopathological examination when the lesions are typical. 55 Fistarol SK, Itin PH. Diagnosis and treatment of lichen sclerosus: an update. Am J Clin Dermatol. 2013;14:27-47.

In dermoscopy recent LS lesions are characterized by homogeneous whitish areas with erythematous halo (Figure 4), with the presence of yellow circles (follicular pseudo-apertures - comedo-like openings), which complies with the histopathologic features of ortohyperkeratosis and follicular plugging. 88 Lacarrubba F, Pellacani G, Verzì AE, Pippione M, Micali G. Extragenital lichen sclerosus: clinical, dermoscopic, confocal microscopy and histologic correlations. J Am Acad Dermatol. 2015;72:S50-2. Older lesions present no halo, with poorly-defined diffuse margins, corresponding in histopathology to epidermal atrophy with flattening of the rete ridges, fibrosis in upper dermis with lichenoid lymphocytic infiltrate and collagen homogenization.22 Garrido-Ríos AA, Alvarez-Garrido H, Sanz-Muñoz C, Aragoneses-Fraile H, Manchado-López P, Miranda-Romero A. Dermoscopy of extragenital lichen sclerosus. Arch Dermatol. 2009;145:1468.,88 Lacarrubba F, Pellacani G, Verzì AE, Pippione M, Micali G. Extragenital lichen sclerosus: clinical, dermoscopic, confocal microscopy and histologic correlations. J Am Acad Dermatol. 2015;72:S50-2.

Figure 4
Dermoscopy showing disease activity: amorphous whitish central area and erythematous halo in the lesion on the back of the hand

Currently there is still no effective treatment for LS, and the majority of patients are initially treated with potent topic corticosteroids. Alternatives are topic calcineurin inhibitors, topic and systemic retinoid, antimalarial drugs and phototherapy. 44 Stavrianeas NG, Katoulis AC, Kanelleas AI, Bozi E, Toumbis-Ioannou E. Extragenital lichen sclerosus of childhood presenting as erythematous patches. Indian J Dermatol Venereol Leprol. 2008;74:59-60.,55 Fistarol SK, Itin PH. Diagnosis and treatment of lichen sclerosus: an update. Am J Clin Dermatol. 2013;14:27-47.

Dermoscopy is a practical optical tool, easy to handle, which, associated with typical clinical features, can assist in the early diagnosis of LS, with no need for invasive examinations.

  • *
    Study performed at Hospital Universitário Pedro Ernesto -Universidade do Estado do Rio de Janeiro (HUPE-UERJ) - Rio de Janeiro (RJ), Brazil.
  • Financial Support: None.

REFERENCES

  • 1
    Coelho WS, Diniz LM, Souza Filho JB. Líquen escleroso e atrófico - relato de dois casos de apresentação atípica. An Bras Dermatol. 2006;81:S297-300.
  • 2
    Garrido-Ríos AA, Alvarez-Garrido H, Sanz-Muñoz C, Aragoneses-Fraile H, Manchado-López P, Miranda-Romero A. Dermoscopy of extragenital lichen sclerosus. Arch Dermatol. 2009;145:1468.
  • 3
    Sauder MB, Linzon-Smith J, Beecker J. Extragenital bullous lichen sclerosus. J Am Acad Dermatol. 2014;71:981-4.
  • 4
    Stavrianeas NG, Katoulis AC, Kanelleas AI, Bozi E, Toumbis-Ioannou E. Extragenital lichen sclerosus of childhood presenting as erythematous patches. Indian J Dermatol Venereol Leprol. 2008;74:59-60.
  • 5
    Fistarol SK, Itin PH. Diagnosis and treatment of lichen sclerosus: an update. Am J Clin Dermatol. 2013;14:27-47.
  • 6
    Rose AE, Boyd KP, Meehan SA, Latkowski JA. Lichen sclerosus et atrophicus. Dermatol Online J. 2013;19:20714.
  • 7
    Kreuter A, Wischnewski J, Terras S, Altmeyer P, Stücker M, Gambichler T. Coexistence of lichen sclerosus and morphea: a retrospective analysis of 472 patients with localized scleroderma from a German tertiary referral center. J Am Acad Dermatol. 2012;67:1157-62.
  • 8
    Lacarrubba F, Pellacani G, Verzì AE, Pippione M, Micali G. Extragenital lichen sclerosus: clinical, dermoscopic, confocal microscopy and histologic correlations. J Am Acad Dermatol. 2015;72:S50-2.

Publication Dates

  • Publication in this collection
    Jul-Aug 2016

History

  • Received
    08 June 2015
  • Accepted
    02 Oct 2015
Sociedade Brasileira de Dermatologia Av. Rio Branco, 39 18. and., 20090-003 Rio de Janeiro RJ, Tel./Fax: +55 21 2253-6747 - Rio de Janeiro - RJ - Brazil
E-mail: revista@sbd.org.br