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CORRESPONDENCE
Year : 2022  |  Volume : 67  |  Issue : 6  |  Page : 765-768
A case of Hewitt-Pelisse syndrome (vulvovaginal-gingival lichen planus) with ocular and duodenal involvement


Department of Dermatology, All India Institute of Medical Sciences, Patna, India

Date of Web Publication23-Feb-2023

Correspondence Address:
Swetalina Pradhan
Department of Dermatology, All India Institute of Medical Sciences, Patna
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_507_21

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How to cite this article:
Sinha A, Pradhan S. A case of Hewitt-Pelisse syndrome (vulvovaginal-gingival lichen planus) with ocular and duodenal involvement. Indian J Dermatol 2022;67:765-8

How to cite this URL:
Sinha A, Pradhan S. A case of Hewitt-Pelisse syndrome (vulvovaginal-gingival lichen planus) with ocular and duodenal involvement. Indian J Dermatol [serial online] 2022 [cited 2023 Mar 29];67:765-8. Available from: https://www.e-ijd.org/text.asp?2022/67/6/765/370309




Sir,

Lichen planus (LP) is a chronic mucocutaneous disorder affecting up to 1% to 4% of the population, severely affecting the patient's quality of life.[1] Vulvovaginal-gingival LP (VVG-LP) or Hewitt-Pelisse syndrome, is an underreported, difficult to treat condition, and sometimes associated with concomitant disease in other mucosal sites. We report a case of VVG-LP, which also presented with ocular involvement, duodenal ulcer and cutaneous lesions and could be successfully managed.

A 40-year-old female, presented to the dermatology outpatient department with complaints of progressive redness and burning sensation in oral and vaginal mucosa of 6 months duration, dark coloured, itchy skin lesions of 3 months duration and redness with burning sensation in both eyes of 1 month duration, progressing to bilateral blurred vision. She also complained of dyspareunia, post coital bleed and increased vaginal discharge. One month ago, she also started having epigastric discomfort and retrosternal pain following food intake, which was partially relieved with proton pump inhibitors.

On dermatological examination, there were multiple discrete violaceous to hyperpigmented papules, macules and plaques over bilateral upper and lower extremities and buttocks [Figure 1]a. Mucosal examination revealed, bright erythema, swelling with whitish streaks over both upper and lower gingiva [Figure 1]b. Violaceous plaques with erosions and Wickham striae were also present on bilateral buccal mucosa and tongue [Figure 1]c. The vaginal mucosa was glazed and erythematous with violaceous streaks at periphery [Figure 2]a. Ophthalmological examination showed violaceous plaques over the lid margins and medial canthus of both the eyes with conjunctival congestion [Figure 2]b.
Figure 1: (a) Hyperpigmented papules, macules and plaques over bilateral lower extremities and buttocks. (b) Erythema and whitish streaks over upper gingiva. (c) Erosions, Wickham's striae over tongue, buccal mucosa along with violaceous pigmentation of lips

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Figure 2: (a) Erythematous vaginal mucosa with violaceous streaks at periphery. (b) Violaceous plaques over the lid margins and medial canthus of both the eyes with conjunctival congestion

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Routine hematological and biochemical investigations were within normal limits. Anti-nuclear antibody profile, viral markers for human immunodeficiency virus, hepatitis B surface antigen and antihepatitis C virus were negative. Abdominal and pelvic ultrasound was normal. Biopsy from cutaneous lesions revealed saw-toothing of the rete ridges, basal cell vacuolation and an extensive band-like infiltrate in the upper dermis along the dermo-epidermal junction. The infiltrate was predominantly lymphocytic in nature. Melanin incontinence was also noted [Figure 3]a and [Figure 3]b. Biopsy from buccal mucosa revealed ulcerated epidermis, and an extensive inflammatory infiltrate [Figure 3]c. Ocular examination by a slit lamp revealed distinct central corneal opacity bilaterally [Figure 4]a. Fluorescent stain was positive in the centre of opacity in right cornea suggestive of epithelial defect [Figure 4]b. Endoscopic examination revealed a normal oesophagus, but congested duodenal mucosa with ulcerative, inflammatory changes.
Figure 3: (a and b) 40× and 100× hematoxylin and eosin stain: saw-toothing of the rete ridges, basal cell vacuolation, an extensive band-like predominantly lymphocytic infiltration in the upper dermis along the dermo-epidermal junction and melanin incontinence. (c) 40× showing band-like lymphocytic infiltrate in dermo-epidermal junction

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Figure 4: (a) Slit lamp showing distinct central corneal opacity. (b) Positive fluorescent stain in the centre of opacity in right cornea suggestive of epithelial defect

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Based upon clinico-histopathological corelation and other supportive investigations, final diagnosis of VVG-LP with associated ocular and duodenal mucosal involvement was made. The patient was started on oral cyclosporine 3 mg/kg/day. Frequent lubrication, lid hygiene and topical fluorometholone 0.1% every 6 hours were prescribed for the eyes. Within 3 months, the patient showed 90% improvement in all her symptoms involving oral and genital mucosal pain and discomfort, clearing of conjunctival congestion, improvement in vision, cutaneous itching and relief of epigastric pain, following which cyclosporine was tapered [Figure 5].
Figure 5: Post treatment follow-up. (a) Flattening of papules and plaques over the buttocks and lower extremities. (b) Improvement in erythema and disappearance of whitish streaks on gingiva. (c) Subsidence of erythema and disappearance of violaceous streaks on vaginal mucosa. (d) Improvement in conjunctival congestion and violaceous plaques over lid margins

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Lichen planus is a chronic mucocutaneous disorder affecting up to 1% to 3% of the population badly affecting the quality of life of the patient particularly with its erosive mucosal variants.[2] Oral lesions are present as the only lesions in 15% to 20% of all patients with LP, whereas two thirds of patients with cutaneous LP have oral lesions. A unique type of oral and genital mucosal LP was described by Pelisse et al.,[2] in 1982 with the triad of erosive or desquamative vulvitis, vaginitis and gingivitis in women, and was detailed in a series of publications in the 1980s, and called as the Hewitt-Pelisse syndrome. The vaginal lesions are seen as velvety red erosions or bright red, glazed erythema, which are friable and bleed on touch. There may be increased vaginal discharge, dysuria, dyspareunia and postcoital bleeding. Vaginal synechiae and adhesions can develop, followed by vaginal stenosis in some cases. Oral lesions may occur at any site, characteristic finding, being an intense erythema of the gingiva, which may sometimes be asymptomatic. Unusual extraoral and extragenital lesions have been reported in the conjunctiva, lacrimal gland canal, esophagus and auditory canal, scarring alopecia and nail changes. Cutaneous lesions seem to be associated in 16% to 18% of cases.[3]

In last two decades, about 50 cases of ocular LP have been reported.[4],[5] Thorne et al.,[4] studied 5 patients with cutaneous and mucosal LP. Most of these patients presented with complaints of chronic conjunctivitis after their third decade of life. Cicatrizing conjunctivitis, lacrimal duct stenosis and even corneal involvement are possible manifestations of LP in ocular surface disorders. Corneal lesions in LP are reported to be exceptionally rare and limited to corneal epithelial defects and ulcers associated with severe dry eye. It is not yet known that it is a part of the natural course of the LP or a secondary morbidity due to the conjunctival cicatrization and dry eye.[5] Sanli et al.,[6] reported, the most common findings on endoscopy in people having gastrointestinal symptoms with LP, as antral gastritis (35%), oesophagitis (20%) and bulbitis (15%). Therapeutic benefit in erosive mucosal LP has been reported with topical as well as systemic corticosteroids, retinoids, dapsone, cyclosporine and mycofenolate mofetil.[1]

Our case started with oral and genital symptoms followed by skin lesions, and eventually developed conjunctival congestion followed by corneal dryness, opacity, epithelial defect along with duodenal erosions on endoscopy. We are reporting this case for the rarity of ocular and duodenal involvement in LP, their rare association with the VVG syndrome of LP, which has very rarely been reported, and the debilitating morbidity, which responded dramatically to cyclosporine.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Vasudevan B, Neema S, Verma R, Deb P, Kharayat V, Sethumadhavan T. Vulvovaginal-gingival syndrome and esophageal involvement in lichen planus. Indian J Dermatol Venereol Leprol 2016;82:209-11.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Pelisse M, Leibowitch M, Sedel D, Hewitt J. A new vulvovaginogingival syndrome. Plurimucous erosive lichen planus. Ann Dermatol Venereol 1982;109:797-8.[in French]  Back to cited text no. 2
    
3.
Eisen D. The evaluation of cutaneous, genital, scalp, nail, esophageal, and ocular involvement in patients with oral lichen planus. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;88:431-6.  Back to cited text no. 3
    
4.
Thorne JE, Jabs DA, Nikolskaia OV, Mimouni D, Anhalt GJ, Nousari HC. et al. Lichen planus and cicatrizing conjunctivitis: Characterization of five cases. Am J Ophthalmol 2003;136:239-43.  Back to cited text no. 4
    
5.
Soleimani M, Tabatabaei SA, Mirshahi R, Nozarian Z, Matini AH. Corneal Involvement by Lichen planus pigmentosus. Ocul Immunol Inflamm 2018;26:897-9.  Back to cited text no. 5
    
6.
Sanli H, Cetinkaya H, Türsen U, Kaya M, Kuzu I, Gürler A. Upper gastrointestinal findings in oral lichen planus. Turk J Gastroenterol 2002;13:31-4.  Back to cited text no. 6
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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