Penis & scrotum
Inflammatory
Lichen sclerosus (balanitis xerotica obliterans)


Topic Completed: 21 August 2020

Minor changes: 24 August 2020

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PubMed Search: (Lichen sclerosus[TI] OR balanitis xerotica obliterans[TI]) penis[TIAB]

Diego F. Sanchez, M.D.
Antonio L. Cubilla, M.D.
Page views in 2019: 16,849
Page views in 2020 to date: 10,782
Cite this page: Sanchez DF, Cubilla AL. Lichen sclerosus (balanitis xerotica obliterans). PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/penscrotumbxo.html. Accessed October 28th, 2020.
Definition / general
Essential features
  • Chronic inflammatory and sclerotic benign condition
  • Clinical diagnosis is usually straightforward
  • Middle aged men
  • Unknown etiology
  • Associated with penile intraepithelial neoplasia (PeIN) and penile carcinomas
Terminology
  • Also called lichen sclerosus et atrophicus
  • Balanitis: inflammation of glans, from Greek ("acorn")
  • Xerotica: unable to determine origin of term but used by Stuhmer in 1928 (see also Arch Dermatol Syph 1941;44:547)
ICD coding
  • ICD-10: N48.0 - leukoplakia of penis
  • ICD-10: L90.0 - lichen sclerosus et atrophicus, for other nongenital sites
  • ICD-11: EB60.1 - lichen sclerosus of penis
Epidemiology
Sites
  • Inner foreskin, coronal sulcus and glans mucosae
  • Urethra may be affected
  • Rarely extends to the shaft
Pathophysiology
  • Unknown at this time
Etiology
Clinical features
Diagnosis
  • Peniscopy: grayish white plaques in foreskin, coronal sulcus or glans
  • Biopsy all suspected cases (J Urol 2007;178:2268)
Prognostic factors
Case reports
Treatment
Clinical images

Images hosted on other servers:

Lichen sclerosus and melanosis

Lichen sclerosus and phimosis

Depigmentation, glans atrophy and ecchymoses

Severe sclerosis, scarring and ulceration

Gross description
  • Grayish, bluish to white irregular geographic foci of atrophy
  • Erosion, ulceration and raised pearly white areas
  • In advanced cases, inner preputial folds may disappear due to replacement of elastic fibers by fibrous tissue (Am J Clin Dermatol 2013;14:27)
Microscopic (histologic) description
  • Topographical evaluation from surface epithelia to deep penile layers (dartos in foreskin and corpus spongiosum in glans) is recommended as follows (Int J Surg Pathol 2020;28:468)
  • Squamous epithelium:
    • Normal, atrophic or hyperplastic, the latter most common, with more than 10 cell epithelial layers
    • Hyperkeratosis associated with hyperplasia
    • Vacuolar degeneration of basal layer, landmark lesions in all types of lichen
    • Tissue separation at the basal layer, which indices late epithelial ulceration
  • Lamina propria:
    • Thickening and loss of structures
    • Edema, hypervascularity and typically sclerosis
    • Distinctive sclerotic patterns
      • At the surface: perivascular, globular or linear sclerosis
      • Diffuse sclerosis in deeper lamina propria tissues
  • Presence of a sclerotic globule is sufficient for diagnosis
  • Sclerotic changes spares corpus spongiosum of glans and foreskin dartos
  • Lymphocytic infiltration:
    • Landmark lesion with variable presentation
    • At the interface of epithelium and lamina propria
    • Distant from basal layer deep in lamina propria
  • Lymphocytes are the basis for subtyping
    • Lichenoid: lymphocytes at the basal layer
    • Band-like or classic: lymphocyte in deep lamina propria
    • Lymphocytic depletion: only few lymphocytes present
  • Depletion of lymphocytes is typical of cancer associated lichen sclerosis
  • During progression sclerosis increases; fully developed lesions are characterized by:
    • Epithelial thinning and ulceration
    • Wide hyalinized band in the upper dermis
    • Lymphocytic infiltrate below the hyalinized band
    • Penile intraepidermal neoplasia and/or carcinoma can be associated
Microscopic (histologic) images

Contributed by Diego F. Sanchez, M.D. and Antonio L. Cubilla, M.D.

Atrophy and sclerosis

Hyperplasia and sclerosis

Linear sclerosis

Vacuolization and perivascular sclerosis

Edema and hyalinization


Classic lichen sclerosus

Lichenoid lichen sclerosus

Lymphocytic depleted lichen sclerosus

PeIN and lichen sclerosus

Pseudohyperplastic carcinoma, lichen sclerosus

Positive stains
Molecular / cytogenetics description
  • Some reports regarding RNA expression are (Int J Biol Sci 2019;15:1429):
    • Upregulation of miR-155, TNF, IL6, galectin 7, collagen (type I, III, V), p53
    • Downregulation of FOXO3, CDKN1B, IL10 and endothelial ECM1
    • CDKN2A and p53 epigenomic modifications
Videos

Lichen sclerosus et atrophicus

Sample pathology report
  • Foreskin, circumcision:
    • Lichen sclerosus (see comment)
    • Comment: Epithelial hyperplasia and hyperkeratosis was noted. Scarce lymphocytic infiltrate was seen in the lamina propria and between sclerotic tissue suggesting lymphocytic depleted variant. This subtype has been reported associated with preneoplastic lesions and carcinoma which were not observed in this specimen.
Differential diagnosis
  • Lichen planus:
    • Dense lymphoid infiltrate is the hallmark
    • Absence of hyalinization / sclerosis
    • This later can be difficult to differentiate with early lichen sclerosus
  • Zoon balanitis:
    • Plasma cell balanitis
    • Usually absence of granular and cornified layers
    • Very rare
Board review style question #1

    A 30 year old man went to the urologist complaining about difficulty with coitus. He has a history of untreated phimosis from childhood. At the clinical examination, a buried penis is found. Circumcision is performed and the following image shows the findings. What is your diagnosis?

  1. Acute postitis
  2. Amyloidosis
  3. Lichen planus
  4. Lichen sclerosus
  5. Zoon balanitis
Board review answer #1
D. Lichen sclerosus. Atrophic epithelium, dense hyalinized fibrotic tissue replacing lamina propria and subjacent lymphocytic inflammatory infiltrate.

Comment Here

Reference: Lichen sclerosus (balanitis xerotica obliterans)
Board review style question #2
    Regarding lichen sclerosus, which of the following is correct?

  1. Circumcision is always curative
  2. HPV is the most common etiology
  3. Immunohistochemistry is needed for accurate diagnosis
  4. It is associated with neoplastic lesions
  5. Only seen in anogenital area
Board review answer #2
D. It is associated with neoplastic lesions. Although it is a benign chronic inflammatory condition, this lesion is associated with non-HPV related PeIN and carcinomas.

Comment Here

Reference: Lichen sclerosus (balanitis xerotica obliterans)
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