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Genital vulvar lichen sclerosus in monozygotic twin women: a case report and review of the literature.

Doulaveri G, Armira K, Kouris A, Karypidis D, Potouridou I - Case Rep Dermatol (2013)

Bottom Line: Lichen sclerosus et atrophicus is a skin disease of multifactorial etiology which appears in patients with genetic or hormonal predisposition and autoimmune disease.Genetic predisposition is suggested by familial reports of the disease which involve twins, siblings, and mother-daughter/son series.This is a report of the occurrence of lichen sclerosus et atrophicus in monozygotic twin women, suggesting that inheritance is of relevance in the etiology of this disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology and Venereology, Hospital 'Andreas Sygros', Athens, Greece.

ABSTRACT
Lichen sclerosus et atrophicus is a skin disease of multifactorial etiology which appears in patients with genetic or hormonal predisposition and autoimmune disease. Genetic predisposition is suggested by familial reports of the disease which involve twins, siblings, and mother-daughter/son series. This is a report of the occurrence of lichen sclerosus et atrophicus in monozygotic twin women, suggesting that inheritance is of relevance in the etiology of this disease.

No MeSH data available.


Related in: MedlinePlus

White atrophic patches which extend onto the perineum in A.T.
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Figure 1: White atrophic patches which extend onto the perineum in A.T.

Mentions: A.T. and O.T. are clinically identical twin women aged 37 years. At the age of 34 years, O.T. developed white atrophic patches on her vulvar skin, which extended onto the perineum. Itching and dyspareunia were the dominant symptoms (fig. 1, fig. 2). The clinical diagnosis was lichen sclerosus et atrophicus. A.T., at the age of 35 years, developed similar changes due to lichen sclerosus et atrophicus affecting her vulva and perineum. Neither sister had any involvement of nongenital skin. Their medical history was unremarkable. Physical examination of internal organs, gynecological examination, and Pap smears were normal for both sisters. Neither sister had any history of trauma in the genital region. Both sisters had had uneventful pregnancy and childbirth, and their children had no perinatal problems. Their family history indicated that their mother suffered from lichen sclerosus et atrophicus and their father from balanitis xerotica obliterans. The results of laboratory studies (blood count, vitamin D3, vitamin B12, iron, folic acid, ferritin, C-reactive protein, glucose, urea, creatinine, uric acid, cholesterol, triglycerides, high-density lipoprotein, low-density lipoprotein, serum glutamic oxaloacetic transaminase, serum glutamic-pyruvic transaminase, alkaline phosphatase, bilirubin, triiodothyronine, thyroxine, and thyroid-stimulating hormone) and sex hormone level tests (follicle-stimulating hormone, luteinizing hormone, prolactin, and dehydroepiandrosterone sulfate) were normal. Antithyroid autoantibodies (anti-TPO and anti-Tg) were detected in serum from the twins and their parents. Moreover, only A.T. was positive for antinuclear antibodies. Antibodies to Borrelia were negative for both twins. The differential diagnosis involved genital lichen planus and lichen simplex chronicus. The diagnosis was supported by the skin biopsy specimen in the most mature area, which showed features of lichen sclerosus et atrophicus (fig. 3). The epidermis was thinned and the rete ridges were effaced. Compact hyperkeratosis and vacuolization of the focal basal cell layer were noted. A pale, widened papillary dermis with superficial perivascular and sparse diffuse lymphocytic infiltration of the upper reticular dermis were also observed.


Genital vulvar lichen sclerosus in monozygotic twin women: a case report and review of the literature.

Doulaveri G, Armira K, Kouris A, Karypidis D, Potouridou I - Case Rep Dermatol (2013)

White atrophic patches which extend onto the perineum in A.T.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3843921&req=5

Figure 1: White atrophic patches which extend onto the perineum in A.T.
Mentions: A.T. and O.T. are clinically identical twin women aged 37 years. At the age of 34 years, O.T. developed white atrophic patches on her vulvar skin, which extended onto the perineum. Itching and dyspareunia were the dominant symptoms (fig. 1, fig. 2). The clinical diagnosis was lichen sclerosus et atrophicus. A.T., at the age of 35 years, developed similar changes due to lichen sclerosus et atrophicus affecting her vulva and perineum. Neither sister had any involvement of nongenital skin. Their medical history was unremarkable. Physical examination of internal organs, gynecological examination, and Pap smears were normal for both sisters. Neither sister had any history of trauma in the genital region. Both sisters had had uneventful pregnancy and childbirth, and their children had no perinatal problems. Their family history indicated that their mother suffered from lichen sclerosus et atrophicus and their father from balanitis xerotica obliterans. The results of laboratory studies (blood count, vitamin D3, vitamin B12, iron, folic acid, ferritin, C-reactive protein, glucose, urea, creatinine, uric acid, cholesterol, triglycerides, high-density lipoprotein, low-density lipoprotein, serum glutamic oxaloacetic transaminase, serum glutamic-pyruvic transaminase, alkaline phosphatase, bilirubin, triiodothyronine, thyroxine, and thyroid-stimulating hormone) and sex hormone level tests (follicle-stimulating hormone, luteinizing hormone, prolactin, and dehydroepiandrosterone sulfate) were normal. Antithyroid autoantibodies (anti-TPO and anti-Tg) were detected in serum from the twins and their parents. Moreover, only A.T. was positive for antinuclear antibodies. Antibodies to Borrelia were negative for both twins. The differential diagnosis involved genital lichen planus and lichen simplex chronicus. The diagnosis was supported by the skin biopsy specimen in the most mature area, which showed features of lichen sclerosus et atrophicus (fig. 3). The epidermis was thinned and the rete ridges were effaced. Compact hyperkeratosis and vacuolization of the focal basal cell layer were noted. A pale, widened papillary dermis with superficial perivascular and sparse diffuse lymphocytic infiltration of the upper reticular dermis were also observed.

Bottom Line: Lichen sclerosus et atrophicus is a skin disease of multifactorial etiology which appears in patients with genetic or hormonal predisposition and autoimmune disease.Genetic predisposition is suggested by familial reports of the disease which involve twins, siblings, and mother-daughter/son series.This is a report of the occurrence of lichen sclerosus et atrophicus in monozygotic twin women, suggesting that inheritance is of relevance in the etiology of this disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology and Venereology, Hospital 'Andreas Sygros', Athens, Greece.

ABSTRACT
Lichen sclerosus et atrophicus is a skin disease of multifactorial etiology which appears in patients with genetic or hormonal predisposition and autoimmune disease. Genetic predisposition is suggested by familial reports of the disease which involve twins, siblings, and mother-daughter/son series. This is a report of the occurrence of lichen sclerosus et atrophicus in monozygotic twin women, suggesting that inheritance is of relevance in the etiology of this disease.

No MeSH data available.


Related in: MedlinePlus