2016
DOI: 10.1590/abd1806-4841.20164398
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Extensive bullous lichen sclerosus et atrophicus

Abstract: Lichen sclerosus et atrophicus is a chronic disease of unknown etiology characterized by atrophic and sclerotic plaques in both genital and extragenital regions. Extensive bullous lichen sclerosus et atrophicus (BLSA) is a severe variant of the disease with no widely accepted treatment. We present a 63-year-old woman with extensive extragenital, ivory-colored, atrophic plaques on her trunk and extremities and disseminated hemorrhagic bullae. The patient was unsuccessfully treated with standard topical corticos… Show more

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Cited by 9 publications
(12 citation statements)
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“…This feature is also closely associated with marked papillary dermal edema and even with subepidermal vesiculation and hemorrhage. Although the etiology of bullous LSA is unknown, loss of androgen receptor expression, random inactivation of the androgen receptor gene, autoimmunity with type II diabetes mellitus, and Borrelia infection are considered possible causes [1,2]. The extragenital form of LSA occurs in approximately 20% of the patients, and the isolated extragenital lichen sclerosus being even rarer tends to develop unusual clinical presentation, such as the development of vesiculobullous lesions [3].…”
Section: Discussion Pathogenesismentioning
confidence: 99%
See 1 more Smart Citation
“…This feature is also closely associated with marked papillary dermal edema and even with subepidermal vesiculation and hemorrhage. Although the etiology of bullous LSA is unknown, loss of androgen receptor expression, random inactivation of the androgen receptor gene, autoimmunity with type II diabetes mellitus, and Borrelia infection are considered possible causes [1,2]. The extragenital form of LSA occurs in approximately 20% of the patients, and the isolated extragenital lichen sclerosus being even rarer tends to develop unusual clinical presentation, such as the development of vesiculobullous lesions [3].…”
Section: Discussion Pathogenesismentioning
confidence: 99%
“…Extensive vacuolar degeneration weakens the integrity of the dermoepidermal junction, which contributes to the development of marked edema in the papillary dermis and subepidermal vesiculation. With increased fragility of dermal capillaries, hemorrhage can accumulate within the bullae [1].…”
Section: Introductionmentioning
confidence: 99%
“…Extragenital bullous LS can also be localized or generalized. Although the cause is unknown, possible theories include genetic predilection, hormonal abnormalities, autoimmunity and Borrelia infection …”
Section: Discussionmentioning
confidence: 99%
“…Evidence for the use of hydroxychloroquine in lichen sclerosis is largely anecdotal. Among three reported cases of bullous lichen sclerosus et atrophicus treated with hydroxychloroquine, two were unresponsive and one showed a positive response …”
Section: Clinical Applications In Dermatologymentioning
confidence: 99%