White sponge nevus

Regezi
Summary:
● Clinical Features
○ Asymptomatic
○ Bilateral
○ Dense
○ Shaggy
○ White or gray
○ Generalized opacification
○ Primarily buccal mucosa affected
○ Other membrane may be involved
○ Rare
● Cause
○ Hereditary
○ Autosomal dominant (keratin 4 and/or 13 genes)
● Significance
○ Remains indefinitely
○ No ill effects

White Sponge Nevus (Cannon’s Disease)

- White sponge nevus (WSN) is an autosomal-dominant inherited condition that is
due to point mutations for genes coding for keratin 4 and/or 13.
- It affects oral mucosa bilaterally and symmetrically, and treatment is
generally not required.

Clinical Features
● asymptomatic, folded, white lesion that may affect several mucosal sites
● Lesions tend to be thickened and have a spongy consistency
● Presentation intraorally is almost always bilateral and symmetric and usually
appears early in life, typically before puberty.
● characteristic clinical manifestations of this particular form of keratosis are
usually best observed on the buccal mucosa, although other areas such as the
tongue and vestibular mucosa may also be involved.
● conjunctival mucosa is usually spared, but mucosa of the esophagus, anus,
vulva, and vagina may be affected
● Skin is not affected because, unlike mucosa, skin does not contain keratins 4
and 13.

Histopathology
● Microscopically, the epithelium is greatly thickened, with marked spongiosis,
acanthosis, and parakeratosis
● Within the stratum spinosum, marked hydropic or clear cell change may be noted,
often beginning in the parabasal region and extending very close to the surface
● Perinuclear eosinophilic condensation of cytoplasm is characteristic of prickle
cells in WSN
 ● often possible to see columns of parakeratin extending from the spinous layer
to the surface.

Differential Diagnosis
● The differential diagnosis includes hereditary benign epithelial dyskeratosis,
lichen planus, lichenoid drug reaction, lupus erythematosus (LE), cheek
chewing, and possibly candidiasis (Table 3-1).
● Once tissue diagnosis is confirmed, no additional biopsies are necessary.

Treatment
● No treatment is necessary for this condition because it is asymptomatic and
benign.
Shafer’s

White Sponge Nevus

(Familial white folded dysplasia of mucous membrane, white folded gingivostomatitis,
oral epithelial nevus, congenital leukokeratosis, Cannon’s disease)

● Familial white folded dysplasia is a relatively uncommon condition of the oral

mucosa described by Cannon in 1935.
● The disease appears to follow a hereditary pattern as an autosomal dominant
trait but with irregular penetrance and no definite sex predilection.

Clinical Features
● This mucosal abnormality is congenital in many instances.
● In other cases it does not appear until infancy, childhood or even adolescence,
by which time it has generally reached the full extent of its severity.
● The oral lesions may be widespread, often involving the cheeks, palate, gingiva,
floor of the mouth and portions of the tongue.
● The mucosa appears thickened and folded or corrugated with a soft or spongy
texture and a peculiar white opalescent hue (Fig. 19-16).
● There is sometimes a minimal amount of folding present.
● Ragged white areas may also be present which can be removed sometimes by gentle
rubbing without any ensuing bleeding (Fig. 19-17A, B).
● The lesions themselves are almost invariably asymptomatic.
● Banoczy and her associates have provided a detailed review and discussion in
their report of 45 cases of this disease. In occasional cases reported in the
literature, the oral lesions were accompanied by similar lesions of other
mucosal surfaces, including the vagina and labia, anus, rectum and nasal
cavity.

Histologic Features
● The microscopic findings in familial white folded dysplasia are characteristic
but not entirely pathognomonic of the disease.
● epithelium is generally thickened, showing both hyperparakeratosis and
acanthosis,and the basal layer is intact.
● cells of the entire spinous layer, continuing to the very surface, exhibit
intracellular edema (Fig. 19-16).
● These vacuolated cells may show pyknotic nuclei.
● parakeratin plugs running deep into the spinous layer are typically found.
● submucosa may show a mild inflammatory cell infiltration, but this is not
consistent

Treatment and Prognosis

● no treatment for the condition, but since it is perfectly benign, the prognosis
is excellent. There are no serious clinical complications