Dermatology Online Journal

Erosions on a prolapsed uterine in an old woman: an unusual manifestation of pemphigus vulgaris
Ali Ramezani MD¹, Narges Ghandi MD¹, Maryam Akhyani MD¹, Maryam Daneshpazhooh MD², Zahra S Naraghi MD³, Cheyda Chams-Davatchi MD¹
Dermatology Online Journal 15 (9): 9

1. Autoimmune Bullous Diseases Research Center, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
2. Vahdate-Eslami sq, Autoimmune Bullous Diseases Research Center, Razi Hospital, Tehran, Iran. daneshpj@sina.tums.ac.ir
3. Department of Pathology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran

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Abstract

Vaginal involvement in pemphigus vulgaris has previously been described. In all those cases a pelvic examination was needed to explore the lesions. We describe a patient with pemphigus vulgaris who had pemphigus erosions on a prolapsed uterus (i.e., on the everted surface of vagina). The patient had widespread lesions of pemphigus in other mucosal and cutaneous sites. Biopsy, antibodies against desmoglein 1 and 3, and direct and indirect immunofluorescence were confirming. The erosions on the prolapsed uterus were resistant to treatment; other mucosal and cutaneous lesions responded rapidly to prednisolone and azathioprine. After lowering the dose of prednisolone the patient was referred to a gynecologist for a vaginal hysterectomy. This case was unique because her vaginal lesions could be easily examined and followed.

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Introduction

Pemphigus vulgaris (PV) is a chronic autoimmune blistering disease of the skin and mucosa characterized by antibodies against desmoglein 3 and to a lesser extent desmoglein 1. The auto-antibodies directed against the keratinocyte cell surface lead to suprabasal acantholysis and blister formation. Although the oral cavity is the initial site of involvement in up to 70 percent of patients [1] and the most common site of involvement, lesions have been described at other mucosal sites including the pharynx, larynx, esophagus, conjunctiva, and anal mucosa [2]. Involvement of the female genital tract including the cervix, vagina, and vulva has also been described. This happens either concurrently with other affected sites or when other involved sites have been in remission [3, 4]. Herein we describe a PV patient with erosions on her prolapsed uterine.

Case report

Figure 1	Figure 2
Figure 1. Prolapsed uterine Figure 2. Pemphigus erosions on the prolapsed uterine and adjacent buttock

A 74-year-old woman presented to the Autoimmune Bullous Diseases Research Center, Razi Hospital, Tehran University of Medical Sciences, with typical oral and skin erosions of PV of two months duration. The diagnosis was confirmed by biopsy and direct immunofluorescence. Desmoglein 1 and 3 index values were 128 and 158, respectively (normal < 20 for both) and indirect immunofluorescence was positive at 1/40 dilution. When taking a detailed history, the patient complained of erosions on an old uterine prolapse that she had had for more than 20 years. These began simultaneously with her oral lesions and produced no pain or discomfort for the patient. On physical examination, typical pemphigus erosions were noted on the buttocks and prolapsed uterus (Figs. 1 & 2).

Figure 3	Figure 4
Figure 3. Hyperplastic epithelial mucosa of vagina with large adjacent ulcerative lesion (x40) Figure 4. Hyperplastic squamous epithelium with suprabasal clefting and tombstone appearance at the edge of biopsy (x100)

A biopsy was performed from the lesions and confirmed the diagnosis of PV showing acantholysis and suprabasal bulla (Figs. 3 - 5). The patient was treated with 2mg/kg/day of oral prednisolone (100 mg/day) and azathioprine (100 mg/day). After four weeks the oral and skin lesions improved and prednisolone tapering was started. However, the lesions on the prolapsed uterus were resistant to treatment and improved only slightly after eight weeks (Fig. 6). The patient was referred to a gynecologist for vaginal hysterectomy when the dose of prednisolone reached 30 mg per day.

Figure 5	Figure 6
Figure 5. Acantholytic epithelial cells within suprabasal bulla (x400) Figure 6. Slow healing lesion on the prolapsed uterine after 8 weeks of treatment

Discussion

Pemphigus vulgaris may involve the female genital tract. However, the frequency at which this occurs varies in different studies [5, 6, 7]. Whereas in the Marren et al. study, four out of nine female patients with PV, had vulvar involvement [6], Malik and Ahmed reported a prevalence of 35 percent for female genital involvement [8]. In a series of 15 PV patients examined by Sagher et al., 12 had evidence of a positive Nikolsky sign of the cervix after local trauma was applied [7]. In a previous study in our center, 39 of 77 female patients (51%) with PV had some degree of genital involvement, and this was the second most common mucosal site after the oral mucosa. The vagina and cervix were affected in 36 percent and 15 percent of patients, respectively [5].

Uterine prolapse is the herniation of the uterus into or beyond the vagina as a result of failure of the ligamentous and fascial supports. It often coexists with prolapse of the vaginal walls, involving the bladder or rectum. According to the pelvic organ prolapse quantification system [9] our patient suffered from stage IV uterine prolapse (complete eversion of the vagina) and the erosions were located on the everted surface of the vagina. The presence of acantholysis and suprabasal clefting of the lesional biopsy specimen was in favor of PV and against other infective and non-infective diagnoses.

There are some reports that show that female genital tract lesions tend to become recalcitrant, persisting after generalized cutaneous PV was controlled by steroid or immunosuppressive therapy [10, 11]. Vaginal erosions in our case were likewise persistent after eight weeks of treatment despite healing of other mucosal and cutaneous lesions.

Female genital tract involvement in PV may cause discomfort, discharge, dyspareunia, and pain [5, 8]. In addition it may create diagnostic problems: non-malignant Papanicolaou smears taken from PV patients have been misinterpreted as neoplasia; conversely neoplastic changes may be falsely regarded as benign acantholytic changes in some PV patients' smears. Considering these two over and under diagnoses, pelvic examination and detection of any vaginal or cervical lesions is of practical importance [12, 13].

Physicians are usually reluctant to perform pelvic examinations, especially in patients with generalized disease or in those whose diagnoses are made easily by examining other more accessible cutaneous or mucosal surfaces. This patient was unique, because her genital involvement could be easily examined and followed.

References

1. Meurer M, Millns JL, Rogers RS, Jordan RE. Oral pemphigus vulgaris.A report of ten cases. Arch Dermatol 1977; 113: 1520-4. [PubMed]

2. Korman NJ. Pemphigus. Dermatol Clin 1990; 8: 689-700. [PubMed]

3. Chams-Davatchi C, Valikhani M, Daneshpazhooh M et al. Pemphigus: analysis of 1209 cases. Int J Dermatol 2005; 44:470-6. [PubMed]

4. Chan E, Thakur A, Farid SL et al. Pemphigus vulgaris of the cervix and upper vaginal vault: a cause of atypical Papanicolaou smears. Arch Dermatol 1998; 134:1485-6. [PubMed]

5. Akhyani M, Chams-Davatchi C, Naraghi Z et al. Cervicovaginal involvement in pemphigus vulgaris: a clinical study of 77 cases. British Journal of Dermatology 2007; 158:478-482. [PubMed]

6. Marren P, Wojnarowska F, Venning V et al. Vulvar involvement in autoimmune bullous diseases. J Reprod Med 1993; 38: 101-7. [PubMed]

7. Sagher F, Bercovici B, Romem R. Nikolsky sign on cervix uteri in pemphigus. Br J Dermatol 1974; 90:407-11. [PubMed]

8. Malik M, Ahmed AR. Involvement of the female genital tract in pemphigus vulgaris. Obstet Gynecol 2005; 106:1005-12. [PubMed]

9. Doshani A, Teo REC, Mayne CJ, Tincello DG.Uterine prolapse: Clinical Review . BMJ 2007;335:819-823. [PubMed]

10. Friedman D, Haim S, Paldi E. Refractory involvement of cervix uteri in a case of pemphigus vulgaris. Am J Obstet Gynecol 1971; 110: 1023-4. [PubMed]

11. Zosmer A, Kogan S, Frumkin A et al. Unsuspected involvement of the female genitalia in pemphigus vulgaris. Eur J Obstet Gynecol Reprod Biol 1992; 47: 260-3. [PubMed]

12. Lonsdale RN, Gibbs S. Pemphigus vulgaris with involvement of the cervix. Br J Dermatol 1998 Feb; 138(2):363-5. [PubMed]

13. Lobo AZ, de Sousa JX Jr, Aoki V et al. Pemphigus vulgaris of the uterine cervix: misinterpretation of papanicolaou smears. Acta Derm Venereol. 2006; 86(4):355-6. [PubMed]

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