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A case of extragenital lichen sclerosus following Blaschko's lines
A case of extragenital lichen sclerosus following Blaschko’s lines Sung Woo Choi, MD, Jeong Eun Yang, MD, Hyun Jeong Park, MD, and Chung Won Kim, MD Seoul, Korea Extragenital lichen sclerosus is most common on the neck, shoulders, and upper portion of the trunk. A 20-year-old Korean man had asymptomatic, well-demarcated, linear violaceous plaques on the left lateral chest, in a pattern corresponding to the lines of Blaschko. We describe a case with a pattern of extragenital lichen sclerosus following Blaschko’s lines. (J Am Acad Dermatol 2000;43:903-4.)
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xtragenital lichen sclerosus developing in a pattern corresponding to the lines of Blaschko was first recognized by Libow and Coots in 1998.1 We describe a similar patient with a pattern of extragenital lichen sclerosus following Blaschko’s lines.
CASE REPORT A 20-year-old Korean man had asymptomatic, well-demarcated, linear violaceous plaques on the left lateral chest for 2 years. The plaques showed central atrophy with a violaceous border, and formed a discontinous band. The band extended from the left nipple in an arc-like configuration to the left lateral axillary line, consistent with the pattern of Blaschko’s lines (Fig 1). The genital, perianal, and mucosal areas were normal. A biopsy specimen showed a thinned epidermis devoid of rete ridges and vacuolar degeneration of the basal layer. Moderate papillary dermal edema was present, along with focal homogenization of the collagen and vascular ectasia. There was a superficial and deep periadnexal lymphohistiocytic infiltrate (Fig 2). The findings were consistent with lichen sclerosus.
This supplement is made possible through an educational grant from Ortho Dermatological to the American Academy of Dermatology. From the Department of Dermatology, College of Medicine, The Catholic University of Korea, Seoul. Reprint requests: Sung Woo Choi, MD, Department of Dermatology, Kangnam St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 505, Banpo-dong, Seocho-gu, Seoul, 137-040 Korea; e-mail: [email protected]. Copyright © 2000 by the American Academy of Dermatology, Inc. 0190-9622/2000/$12.00 + 0 16/4/101876 doi:10.1067/mjd.2000.101876
Fig 1. Lichen sclerosus along Blaschko’s lines on left chest.
DISCUSSION Extragenital lichen sclerosus is most common on the neck, shoulders, and upper portion of the trunk. It is generally asymptomatic, but occasionally pruritic.2 Less common presentations include infraorbital 903
904 Choi et al
A
J AM ACAD DERMATOL NOVEMBER 2000
B Fig 2. A, Effaced epidermis and homogenized papillary dermis consistent with lichen sclerosus. B, Higher power reveals vacuolar degeneration of basal layer. (A and B, Hematoxylin-eosin stain; original magnifications: A, × 64; B, ×200.)
lichen sclerosus, scarring alopecia, and involvement of the palms, soles, scalp, and face.3-5 Our patient had lesions on the trunk and was asymptomatic. Most lesions of extragenital lichen sclerosus present as flat, white, polygonal papules, and slight atrophic white plaques. This case, which shows well-demarcated, linear, shiny atrophic plaques, differs from most clinical presentations of extragenital lichen sclerosus. The arciform shape presented in this case was very similar to the first reported case of lichen sclerosus following the lines of Blaschko.1,6 Blaschko’s lines are most apparent on the trunk because of the arcs on the upper chest, the S-shapes on the abdomen, and a V-shape as the lesions approach the posterior midline.6 The first reported case presented with S-shapes on the abdomen, and in our case, the arcs were seen on the upper chest. We reviewed the literature and found that some lichen sclerosus that developed on the trunk also
showed well-circumscribed, linear, violaceous, shiny atrophic plaques.7 We could not exclude the possibility that lichen sclerosus in some of those cases also developed along the lines of Blaschko. REFERENCES 1. Libow LF, Coots NV. Lichen sclerosus following the lines of Blaschko. J Am Acad Dermatol 1998;38:831-3. 2. Meffert JJ, Davis BM, Grimwood RE. Lichen sclerosus. J Am Acad Dermatol 1995;32:393-416. 3. Petrozzi JW, Wood MG, Tisa V. Palmar-plantar lichen sclerosus et atrophicus. Arch Dermatol 1979;115:884. 4. Foulds IS. Lichen sclerosus et atrohicus of the scalp. Br J Dermatol 1980;103:197-200. 5. Purres J, Krull EA. Lichen sclerosus et atrophicus involving the palms. Arch Dermatol 1971;104:68-9. 6. Bolognia JL, Orlow SJ, Glick SA. Lines of Blaschko. J Am Acad Dermatol 1994;31:157-81. 7. Gross P. Generalized lichen sclerosus et atrophicus associated with band-like scleroderma (Koebner phenomenon and lichen sclerosus). Arch Dermatol 1958;77:752-3.
E
xtragenital lichen sclerosus developing in a pattern corresponding to the lines of Blaschko was first recognized by Libow and Coots in 1998.1 We describe a similar patient with a pattern of extragenital lichen sclerosus following Blaschko’s lines.
CASE REPORT A 20-year-old Korean man had asymptomatic, well-demarcated, linear violaceous plaques on the left lateral chest for 2 years. The plaques showed central atrophy with a violaceous border, and formed a discontinous band. The band extended from the left nipple in an arc-like configuration to the left lateral axillary line, consistent with the pattern of Blaschko’s lines (Fig 1). The genital, perianal, and mucosal areas were normal. A biopsy specimen showed a thinned epidermis devoid of rete ridges and vacuolar degeneration of the basal layer. Moderate papillary dermal edema was present, along with focal homogenization of the collagen and vascular ectasia. There was a superficial and deep periadnexal lymphohistiocytic infiltrate (Fig 2). The findings were consistent with lichen sclerosus.
This supplement is made possible through an educational grant from Ortho Dermatological to the American Academy of Dermatology. From the Department of Dermatology, College of Medicine, The Catholic University of Korea, Seoul. Reprint requests: Sung Woo Choi, MD, Department of Dermatology, Kangnam St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 505, Banpo-dong, Seocho-gu, Seoul, 137-040 Korea; e-mail: [email protected]. Copyright © 2000 by the American Academy of Dermatology, Inc. 0190-9622/2000/$12.00 + 0 16/4/101876 doi:10.1067/mjd.2000.101876
Fig 1. Lichen sclerosus along Blaschko’s lines on left chest.
DISCUSSION Extragenital lichen sclerosus is most common on the neck, shoulders, and upper portion of the trunk. It is generally asymptomatic, but occasionally pruritic.2 Less common presentations include infraorbital 903
904 Choi et al
A
J AM ACAD DERMATOL NOVEMBER 2000
B Fig 2. A, Effaced epidermis and homogenized papillary dermis consistent with lichen sclerosus. B, Higher power reveals vacuolar degeneration of basal layer. (A and B, Hematoxylin-eosin stain; original magnifications: A, × 64; B, ×200.)
lichen sclerosus, scarring alopecia, and involvement of the palms, soles, scalp, and face.3-5 Our patient had lesions on the trunk and was asymptomatic. Most lesions of extragenital lichen sclerosus present as flat, white, polygonal papules, and slight atrophic white plaques. This case, which shows well-demarcated, linear, shiny atrophic plaques, differs from most clinical presentations of extragenital lichen sclerosus. The arciform shape presented in this case was very similar to the first reported case of lichen sclerosus following the lines of Blaschko.1,6 Blaschko’s lines are most apparent on the trunk because of the arcs on the upper chest, the S-shapes on the abdomen, and a V-shape as the lesions approach the posterior midline.6 The first reported case presented with S-shapes on the abdomen, and in our case, the arcs were seen on the upper chest. We reviewed the literature and found that some lichen sclerosus that developed on the trunk also
showed well-circumscribed, linear, violaceous, shiny atrophic plaques.7 We could not exclude the possibility that lichen sclerosus in some of those cases also developed along the lines of Blaschko. REFERENCES 1. Libow LF, Coots NV. Lichen sclerosus following the lines of Blaschko. J Am Acad Dermatol 1998;38:831-3. 2. Meffert JJ, Davis BM, Grimwood RE. Lichen sclerosus. J Am Acad Dermatol 1995;32:393-416. 3. Petrozzi JW, Wood MG, Tisa V. Palmar-plantar lichen sclerosus et atrophicus. Arch Dermatol 1979;115:884. 4. Foulds IS. Lichen sclerosus et atrohicus of the scalp. Br J Dermatol 1980;103:197-200. 5. Purres J, Krull EA. Lichen sclerosus et atrophicus involving the palms. Arch Dermatol 1971;104:68-9. 6. Bolognia JL, Orlow SJ, Glick SA. Lines of Blaschko. J Am Acad Dermatol 1994;31:157-81. 7. Gross P. Generalized lichen sclerosus et atrophicus associated with band-like scleroderma (Koebner phenomenon and lichen sclerosus). Arch Dermatol 1958;77:752-3.