Case Of White Sponge Nevus Research Articles

18781 A familial case of white sponge nevus and review of the literature

White sponge nevus (WSN) is an autosomal dominant skin disorder characterized by white corrugated and diffuse plaques mainly affecting the non-keratinised stratified squamous epithelium. Although WSN is usually seen in the oral mucosa, other sites, such as the nasal, esophageal, laryngeal, and anogenital mucosa, may also be involved. We describe a case a 11-year-old girl who presented with asymptomatic rough, diffuse, white patches in the bilateral buccal mucosa gingiva with extension to the esophagus that was evident on esophageal gastroduodenoscopy. A biopsy of esophageal tissue confirmed the presence of WSN. Her father had a similar biopsy-proven but more widespread appearance in the buccal mucosa and esophagus as well. WSN is caused by germline variants of the keratin genes KRT4 or KRT13, located, respectively, at chromosomes 12q13 and 17q21-q22. Extra-oral affected sites have been shown to arise only from KRT13 variants. It is important to recognize this benign entity and to differentiate it from other acquired causes of intra-oral white plaques such as leukoplakia, candidiasis and lichen planus. A thorough history and physical examination would help to exclude other genodermatoses like pachyonychia congenita, hereditary benign intraepithelial dyskeratosis, Darier disease, and dyskeratosis congenita.

A case and familial cases of white sponge nevus successfully treated by long term-low dose macrolide therapy

A case and familial cases of white sponge nevus successfully treated by long term-low dose macrolide therapy

A Familial Case of White Sponge Nevus in a Korean Family

A Familial Case of White Sponge Nevus in a Korean Family

White Sponge Nevus: Clinical Suspicion and Diagnosis

White sponge nevus is a rare, autosomal-dominant disorder that affects the noncornified stratified squamous epithelia. Clinically, the presence of white, spongy plaques mostly in the buccal, labial, and gingival mucosa and the floor of the mouth characterize the lesions. The differential diagnosis of the lesion may be difficult and it is best diagnosed by biopsy. We report a case of white sponge nevus in the oral cavity of a 16-year-old boy and review of the literature.

頰粘膜に発生した白色海綿状母斑の2例

White sponge nevus is a rare disease that develops in the oral mucosa, which becomes edematous and spongy. We report two cases of white sponge nevus of the oral mucosa. Patient 1 was a 25-year-old woman. Her chief complaint was a bilateral sense of incongruity of the buccal mucosa and upper and lower lips. Her father and older brother had similar lesions. She also had a similar lesion on the perineum. The symptoms improved in response to treatment with a cephem antimicrobial agent, which had been prescribed after the biopsy. Patient 2 was a 44-year-old man. He requested a detailed examination of bilateral white lesions of the buccal mucosa. A biopsy yielded a diagnosis of a white sponge nevus. In this patient, lesions were present in only the oral cavity. His symptoms responded to treatment with macrolide antibiotics, which had been prescribed after the biopsy.

White sponge nevus in a patient with EEC syndrome

A 10-year-old boy, affected by cleft lip and palate and the Ectrodactyly-Ectodermal Dysplasia-Clefting (EEC) syndrome, presented with hypodontia, microdontia, enamel hypoplasia and caries, as well as white macules and plaques on the buccal mucosa bilaterally. Verrucous plaques were noted on the right side and a smooth diffuse white macule was present on the left side. Samples of each were taken by incisional biopsy. Microscopic analysis revealed oral mucosa comprised of hyperplastic stratified squamous epithelium with either hyperparakeratosis or lack of keratinization. There was extensive hydropic degeneration and sparse cells with perinuclear eosinophilic condensation were identified. The underlying fibrous connective tissue exhibited blood vessels with mild subepithelial mononuclear inflammatory infiltrate, bundles of skeletal striated muscle fibers, and areas of hemorrhage at deeper regions. White sponge nevus was diagnosed. To our knowledge, no other case of white sponge nevus has been reported in a patient with EEC syndrome. Considering the low prevalence of this disorder and also of the EEC syndrome, future studies on the etiology of the white sponge nevus might look at the common etiopathogenic aspects of both the white sponge nevus and the EEC syndrome.

White sponge nevus. Seven cases in the same family

AbstractSeven cases of white sponge nevus in seven women of the same family are reported. At birth the patients had lesions consisting of asymptomatic white spongy plaques located on the oral mucosa; in two patients the lesions also involved the vaginal mucosa. The lesions improved after antibiotic treatment. Histology was performed in four cases and an ultrastructural study in one. The findings were consistent with the diagnosis of white sponge nevus in all cases. Differential diagnosis was made with other conditions presenting as white lesions of the oral mucosa.

White sponge nevus. Seven cases in the same family

Seven cases of white sponge nevus in seven women of the same family are reported. At birth the patients had lesions consisting of asymptomatic white spongy plaques located on the oral mucosa; in two patients the lesions also involved the vaginal mucosa. The lesions improved after antibiotic treatment. Histology was performed in four cases and an ultrastructural study in one. The findings were consistent with the diagnosis of white sponge nevus in all cases. Differential diagnosis was made with other conditions presenting as white lesions of the oral mucosa.

The white sponge nevus

A case of white sponge nevus, the first reported from India, is described. There was evidence of an autosomal dominant inheritance.

The electron microscopic examination of congenital keratoses of the oral mucous membranes: I. White sponge nevus

A review of the literature concerning the reported cases of white sponge nevus and a discussion of other congenital white lesions of the oral mucous membranes have been presented. Five cases of white sponge nevus were examined by means of the light microscope and the electron microscope. One of the patients was a Negro, the first reported as such in the literature. The tonofilaments were observed in four patterns: (1) loosely dispersed throughout the cytoplasm, (2) perinuclear in distribution, (3) at opposite poles of the cytoplasm from the nucleus, or (4) in a circular mass in the center of an anucleoid cell. The cells of the spinous and more superficial layers often showed crystallization of the residual nuclear material and a loss of the nuclear membrane. Membrane-bound lamellated structures were noted near the outer cell membranes of many of the cells. The attachment plaques (desmosomes) appeared to have a normal morphology in most instances, although a number of altered attachments in the superficial cells were noted.

White sponge nevus in the oral regions

A review of the literature revealed 28 previously reported cases of white sponge nevus of the oral mucosa. The 28 lesions showed various clinical appearances leading to disagreement as to the proper name for the lesion. The lesion exhibited a familial incidence in all instances when attempts were made to correlate it with a genetic factor. The histopathology of the 28 white sponge nevi revealed the same characteristics of hyperplasia, acanthosis, dyskeratosis, elongation and fusion of the rete pegs, intact basement membrane and no inflammation. The two patients reported in this article and the 28 reported in the literature were identical from a histopathologic standpoint, although various clinical criteria were represented. The latter patients were unrelated.

White sponge nevus: Report of a case

A case of white sponge nevus has been presented. The lesion requires no treatment, and it should therefore be differentiated from leukoplakia, lichen planus, and moniliasis. The histologic and microscopic findings are distinctive.