Lichen sclerosus et atrophicus is a chronic inflammatory skin disease that typically affects prepubertal girls and peri- or post-menopausal women in genital and perineal areas. In some cases, it can also manifest as extragenital lesions. Extragenital Blaschkoid lesions have infrequently been reported. Here, we report a case of extragenital Blaschkoid lichen sclerosus et atrophicus in a child

Albornoz, Christian A

Wang, Jordan

Yang, Sherry

Journal of Dermatology Research and Therapy

English

Jefferson Digital Commons

Thomas Jefferson UniversityJefferson Digital CommonsDepartment of Dermatology and CutaneousBiology Faculty PapersDepartment of Dermatology and CutaneousBiology5-12-2018Extragenital Blaschkoid lichen sclerosus etatrophicus in a childChristian A. AlbornozTemple UniversityJordan WangThomas Jefferson University, jordan.wang@jefferson.eduSherry YangThomas Jefferson University, sherry.yang@jefferson.eduLet us know how access to this document benefits youFollow this and additional works at: https://jdc.jefferson.edu/dcbfpPart of the Dermatology CommonsThis Article is brought to you for free and open access by the Jefferson Digital Commons. The Jefferson Digital Commons is a service of ThomasJefferson University's Center for Teaching and Learning (CTL). The Commons is a showcase for Jefferson books and journals, peer-reviewed scholarlypublications, unique historical collections from the University archives, and teaching tools. The Jefferson Digital Commons allows researchers andinterested readers anywhere in the world to learn about and keep up to date with Jefferson scholarship. This article has been accepted for inclusion inDepartment of Dermatology and Cutaneous Biology Faculty Papers by an authorized administrator of the Jefferson Digital Commons. For moreinformation, please contact: JeffersonDigitalCommons@jefferson.edu.Recommended CitationAlbornoz, Christian A.; Wang, Jordan; and Yang, Sherry, "Extragenital Blaschkoid lichen sclerosus etatrophicus in a child" (2018). Department of Dermatology and Cutaneous Biology Faculty Papers. Paper105.https://jdc.jefferson.edu/dcbfp/105Albornoz et al. J Dermatol Res Ther 2018, 4:052Volume 4 | Issue 1DOI: 10.23937/2469-5750/1510052Journal ofDermatology Research and TherapyCitation: Albornoz CA, Wang JV, Yang S (2018) Extragenital Blaschkoid Lichen Sclerosus et Atrophicus in a Child. J Dermatol Res Ther 4:052. doi.org/10.23937/2469-5750/1510052Received: February 24, 2018: Accepted: May 10, 2018: Published: May 12, 2018Copyright: © 2018 Albornoz CA, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.Albornoz et al. J Dermatol Res Ther 2018, 4:052Open AccessISSN: 2469-5750• Page 1 of 3 •Extragenital Blaschkoid Lichen Sclerosus et Atrophicus in a ChildChristian A Albornoz1, Jordan V Wang2* and Sherry Yang21Lewis Katz School of Medicine, Temple University, Philadelphia, PA, USA2Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, PA, USA*Corresponding author: Jordan V Wang, MD, MBE, Department of Dermatology and Cutaneous Biology, Thomas Jefferson University Hospital, 833 Chestnut Street, Suite 740, Philadelphia, PA, 19107, USA, Tel: (215)-955-6680, E-mail: jordan.wang@jefferson.eduAbstractLichen sclerosus et atrophicus is a chronic inflammatory skin disease that typically affects prepubertal girls and peri- or post-menopausal women in genital and perineal areas. In some cases, it can also manifest as extragenital lesions. Extragenital Blaschkoid lesions have infrequently been re-ported. Here, we report a case of extragenital Blaschkoid lichen sclerosus et atrophicus in a child.KeywordsLichen sclerosus et atrophicus, Lichenoid eruptions, Der-matology, Blaschkoid, Pediatricsmuscle weakness, difficulty with movement, or oral or genital involvement. Her family history was unremar-kable.On the left upper back extending onto the axilla and chest in a linear distribution, there were multiple well-defined, ovoid, atrophic plaques with mottled hyperpigmentation and hypopigmentation (Figure 1). No induration or erythema was appreciated. Her shoul-der had normal range of motion. A biopsy showed com-pact orthokeratosis, loss of the normal rete ridge pat-tern, papillary dermal edema, and a sparse lymphocytic infiltrate (Figure 2). Clinical pathologic correlation was consistent with Blaschkoid LSA.The patient was treated with a regimen of clobeta-sol ointment alternating with tacrolimus ointment. Mild symptomatic improvement was noted after one month of treatment.DiscussionLSA is a chronic inflammatory disease that typically affects the genital and perineal areas, with extragenital spread occurring in 15-20% of patients [1]. Prepubertal girls and peri- or post-menopausal women have the hi-ghest prevalence. While the etiology remains unclear, numerous hormonal and immunologic agents in addi-tion to genetic predisposition have been implicated as pathologic factors. Nearly 75% of women with LSA were found to have increased antibodies against extracellu-lar matrix protein 1, a significant building block of the dermis, thus supporting an autoimmune process [2]. However, this antibody is not routinely checked in pa-tients, and it was not checked in ours.CASe RepoRtCheck forupdatesIntroductionAlthough lichen sclerosus et atrophicus (LSA) is a common inflammatory skin disease affecting the geni-tal and perineal areas in prepubertal girls and peri- or post-menopausal women, extragenital lesions are less common, especially in a Blaschkoid distribution. Here, we present the case of a 12-year-old girl with extrage-nital Blaschkoid LSA that responded to high-potency topical steroids and also provide a brief review of the disease.Case ReportA 12-year-old African-American girl presented with a pruritic rash on her left back and axilla. This was diagno-sed clinically as lichen simplex chronicus about 2 years prior when there was a single, scaly, lichenified plaque in her left inferior axilla. However, the patient had been lost to follow-up, and her rash continued to spread. She denied any history of folliculitis, trauma, or burns to the area. She denied any associated myalgias, arthralgias, ISSN: 2469-5750DOI: 10.23937/2469-5750/1510052Albornoz et al. J Dermatol Res Ther 2018, 4:052 • Page 2 of 3 •red for atypical cases, including extragenital lesions that can mimic other disease processes. LSA characteristical-ly demonstrates an atrophic epidermis and a lichenoid infiltrate at the dermal-epidermal junction with loss of the normal rete ridges. In early lesions, papillary edema is found along with homogenization of collagen leading to eventual sclerosis. Scattered histiocytes, plasma cells, and mast cells can be present [4].LSA is a progressive disease that can be disfiguring. Ear-ly treatment should include high-potency topical cortico-steroids. Because of the side effects from long-term use of topical corticosteroids, calcineurin inhibitors can be utili-zed for continued management. Among other treatment options with limited efficacy, phototherapy and oral reti-The lesions of LSA usually present as ivory-colored and scar-like polygonal papules and plaques. Involve-ment of vulvar and perineal regions can produce a classic figure-of-eight configuration. Symptoms include vulvar pruritus, dyspareunia, and dysuria. However, extrageni-tal lesions are usually asymptomatic and can typically be found on the neck, trunk, and proximal extremities. In rare cases, a Blaschkoid pattern can been identified as in our case [3]. This is thought to be a product of genetic mosaicism, where two distinct cell clones are present. The abnormal clone is ultimately stimulated by an en-vironmental or immunologic factor, leading to its un-masking and the generation of segmental lesions.LSA can be diagnosed clinically, but a biopsy is requi-         Figure 1: Well-defined, ovoid, atrophic plaques with mottled hyperpigmentation and hypopigmentation.         A BFigure 2: Compact orthokeratosis, loss of the normal rete ridge pattern, papillary dermal edema, and a sparse lymphocytic infiltrate A) H&E, original magnification x4; B) H&E, original magnification x10.ISSN: 2469-5750DOI: 10.23937/2469-5750/1510052Albornoz et al. J Dermatol Res Ther 2018, 4:052 • Page 3 of 3 •References1. Cabanillas González M, Monteagudo B, de las Heras C, Cacharrón JM (2009) Blaschkoid, zosteriform linear lichen sclerosus et atrophicus. Actas Dermosifiliogr 100: 155-157. 2. Oyama N, Chan I, Neill SM, Hamada T, South AP, et al. (2003) Autoantibodies to extracellular matrix protein 1 in lichen sclerosus. Lancet 362: 118-123. 3. Pavlović MD (2004) Linear extragenital lichen sclerosus with underlying bony atrophy. J Am Acad Dermatol 50: e7-e10. 4. Meffert J, Zeichner JA, Talavera F, Chan EF, Quirk CM, et al. (1997) Lichen sclerosus et atrophicus. In: Elder D, Elenitsas R, Jaworsky C, Johnson B Jr, Lever's Histopathology of the Skin. Lippincott-Raven Publishers, Philadelphia, 280-283.5. Fistarol SK, Itin PH (2013) Diagnosis and treatment of li-chen sclerosus: An update. Am J Clin Dermatol 14: 27-47.noids have been shown to be helpful. Cryotherapy, carbon dioxide laser, and focused ultrasound have also been used with varying rates of success [5].Authorship StatementAll authors have contributed equally to this work.Funding SourcesNone.Conflict of Interest DisclosureNone.

Extragenital Blaschkoid lichen sclerosus et atrophicus in a child

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