Lichen sclerosus as a variant of limited scleroderma with damage to the anogenital region

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Abstract

Lichen sclerosus is one of the clinical variants of limited scleroderma, a feature of which is the defeat of the anogenital zone. The issues of the etiology and pathogenesis of lichen sclerosus remain not fully understood, despite numerous studies in which the polygenic nature of inheritance and multifactoriality in the implementation of dermatosis are noted.

With the progression of the scleroatrophic process and the absence of timely pathogenetic therapy, there is a high probability of the development of comorbid pathology with a whole range of symptoms, such as urinary (dysuria, recurrent urinary tract infections - cystitis, urethritis, cystourethritis), gastrointestinal (pain during defecation, chronic constipation), vaginal severe dryness and increased sensitivity of the vaginal mucosa, burning, itching).

Treatment of patients with lichen sclerosus with lesions of the anogenital zone causes great difficulties. In cases of isolated localization of scleroatrophic lesions of anogenital localization, difficulties may arise in the differential diagnosis with diseases of similar localization, such as vitiligo, atrophic lichen planus, basal cell carcinoma, contact dermatitis, nonspecific balanoposthitis, Keir's erythroplasia. Treatment of limited scleroderma, the clinical variant of which is lichen sclerosus, should be multi-course and complex, with the obligatory use of penicillin antibiotics, hyaluronidase-based drugs, drugs that improve microcirculation, vitamins, immunoregulators and physiotherapeutic methods.

Thus, early diagnosis and timely pathogenetic therapy contribute to the prevention of the risks of developing genitourinary syndrome, progression of tissue sclerosis, and psychoemotional disorders.

This article presents a clinical case of diagnosis and treatment of a patient with lichen sclerosus.

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About the authors

Gayane E. Bagramova

Peoples’ Friendship University of Russia

Email: bagramovaga@mail.ru
ORCID iD: 0000-0003-4615-7356
SPIN-code: 2687-8252

MD, Dr. Sci. (Med.), Professor

Russian Federation, Moscow

Elena S. Snarskaya

The First Sechenov Moscow State Medical University (Sechenov University)

Email: snarskaya-dok@mail.ru
ORCID iD: 0000-0002-7968-7663
SPIN-code: 3785-7859
Scopus Author ID: 8714450500

MD, Dr. Sci. (Med.), Professor

Russian Federation, 2 building 2 st. Bolshaya Pirogovskaya, 119992, Moscow

Lydia M. Shnakhova

The First Sechenov Moscow State Medical University (Sechenov University)

Email: Lika-slm@mail.ru
ORCID iD: 0000-0003-3000-0987

assistant

Russian Federation, 2 building 2 st. Bolshaya Pirogovskaya, 119992, Moscow

Yulia A. Semenchak

The First Sechenov Moscow State Medical University (Sechenov University)

Author for correspondence.
Email: y.semenchak@bk.ru
ORCID iD: 0000-0002-6608-0301
SPIN-code: 8580-1871

MD, Cand. Sci. (Med.), Assistant

Russian Federation, 2 building 2 st. Bolshaya Pirogovskaya, 119992, Moscow

References

  1. Snarskaya ES, Semenchuk YA. Scleroatrophic lichen of anogenital localization: clinical and morphological, differential diagnostic features and a comprehensive method of treatment. RMJ. Medical Review. 2019;3(12):9–12. (In Russ).
  2. Kirtschig G. Lichen sclerosus — presentation, diagnosis and management. Dtsch Arztebl. 2016;113:337–343. doi: 10.3238/arztebl.2016.0337
  3. Goldstein AT, Marinoff SC, Christopher K, et al. Prevalence of vulvar lichen sclerosus in a general gynecology practice. J Reprod Med. 2005;50(7):477–480.
  4. Kizer WS, Prarie T, Morey AF. Balanitis xerotica obliterans: epidemiologic distribution in an equal access health care system. South Med J. 2003;96(1):9–11. doi: 10.1097/00007611-200301000-00004
  5. Fistarol SK, Itin PH. Diagnosis and treatment of lichen sclerosus: an update. Am J Clin Dermatol. 2013;14(1):27–47. doi: 10.1007/s40257-012-0006-4
  6. Snarskaya ES, Semenchuk YA. Gender and clinical and topographic features of the anogenital zone lesion in patients with limited scleroderma. Russian Journal of Skin and Venereal Diseases. 2020;23(3):165–173. (In Russ). doi: 10.17816/dv48930
  7. Ignatovsky AV, Agakishizade NE. Scleroatrophic lichen of the vulva and genitourinary syndrome: issues of diagnosis and treatment. Medical Alphabet. 2019;1(7):39–41. (In Russ). doi: 10.33667/2078-5631-2019-1-7(382)-39-41
  8. Bleeker MC, Visser PJ, Overbeek LI, et al. Lichen sclerosus: Incidence and risk of vulvar squamous cell carcinoma. Cancer Epidemiol Biomark Prev. 2016;25(8):1224–1230. doi: 10.1158/1055-9965.epi-16-0019
  9. Weyers W. Hypertrophic Lichen sclerosus with dyskeratosis and parakeratosis ― a common presentation of vulvar Lichen sclerosus not associated with a significant risk of malignancy. Am J Dermatopatho. 2013;35(7):713–721. doi: 10.1097/dad.0b013e31827e7ea9
  10. Akel R, Fuller C. Updates in lichen sclerosis: British Association of Dermatologists guidelines for the management of lichen sclerosus 2018. Br J Dermatol. 2018;178(4):823–824. doi: 10.1111/bjd.16445
  11. Russian Society of Dermatovenerologists and Cosmetologists. Federal clinical guidelines. Dermatovenerology. 2020 [Accessed: 12.02.2021]. (In Russ). Available from: https://www.rodv.ru/klinicheskie-rekomendacii/

Supplementary files

Supplementary Files
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2. Fig. 1. Patient S., 49 years old. Diagnosis: lichen sclerosis of extragenital and anogenital localization. Multiple scleroatrophic foci on the skin of the mammary glands.

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3. Fig. 2. The same patient. Common scleroatrophic lesions of extragenital localization (skin of the back, lateral surfaces of the trunk, neck). Rodman skin score 19 points, 2 degree of severity of the process, activity index 3 points, index of the degree of damage and atrophy of the genitals and perianal region 13 points.

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4. Fig. 3. The same patient. Common scleroatrophic lesions of anogenital localization with lesions of the labia majora, labia minora, clitoris, posterior adhesions with the transition to the anus.

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5. Fig. 4. The same patient. Pathomorphological picture of scleroatrophic lesions of anogenital localization (staining with hematoxylin and eosin, ×40).

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