Labial adhesion resulting from pemphigus vulgaris: A rare cause of hematometra
INTRODUCTION:
Pemphigus vulgaris (PV) incidence: 0.76- 16.1 cases/1,00,000 individuals per year
Autoimmune disease: desmoglein-1 & 3 protein
Organs Involved: mucosa of oral cavity (M/C), oesophagus, larynx, nose, nails, genital tract
Diagnosis: Clinical features, histology, immunofluorescence (IF)
Treatment: steroids, immunosuppressants
Here, we describe labial adhesion in reproductive age group female arising out of PV leading to secondary amenorrhea and hematometra
Never been reported before
CASE PRESENTATION:
A female in her 20’s, diagnosed case of PV presented with amenorrhea and cyclical abdominal pain for 2 months
No urinary/bowel complaints
No sexual trauma/ assault
Past menstrual history: unremarkable with menarche at 15 years
Past history: Onset of oral, nasal and genital erosions 8 months back, leading to difficulty in eating solids & liquids. Diagnosed with PV after histology and IF from oral mucosa biopsy
Treatment received: injection dexamethasone pulse therapy, tablet prednisolone 30 mg daily & tablet azathioprine 50 mg twice daily. Genital lesions had resolved at presentation. Oral and nasal lesions were in the healing phase.
Family history: Not significant
Examination:
General Physical Examination:
Crusted erosions on both the lips, hard and soft palate, nasal mucosa.
Normal secondary sexual characteristics
Abdomen: soft, non-tender, suprapubic bulge corresponding to 12 weeks pregnant uterus size appreciated
Genital Examination
Thick adhesion band between labia minora obliterating the vaginal introitus with only 1 cm unfused area anteriorly.
Clitoris visible and urethral orifice narrowed
No erosions or erythema visualized in the genital area (Figure 1)
Per-rectal examination: Same mass felt anteriorly
INVESTIGATIONS:
qRoutine tests- unremarkable
qAbdominal ultrasonogram (US)- hyperechoic collection in cervix -6.4 X 8.2 cm (hematocolpos) and in uterus 4.5 x3 X 2.3 cm (hematometra)
qTzanck smear from labia- acantholytic cells, neutrophils
TREATMENT:
Multidisciplinary approach (Gynaecologist, dermatologist)
Plan: Topical triamcinolone acetonide twice daily (0.1%) followed by surgical adhesiolysis in view of thick nature of fusion band
On day 2 of triamcinolone application, she started to bleed through vagina
On examination: labia were still fused, adhesion band had thinned out, bleeding seen coming through vagina
Repeat US: Empty uterine cavity and cervix
Surgical adhesiolysis cancelled. Manual separation of labial adhesions under local anaesthesia successfully attempted. Cervical opening felt. Blood observed draining. No erosions were visualized (Figure 3 & 4).
FOLLOW-UP:
Topical triamcinolone acetonide continued twice daily for 1 week followed by OD for 3 wks. At the end of 1 year follow up, labia were still separated, no recurrence
DISCUSSION:
Atypical presentations of genital pemphigus vulgaris described in literature include desquamative vaginitis and squamous cell carcinoma but labial adhesion and hematometra have not been reported.
Also, labial fusion is a rare clinical entity in reproductive age group and cause is unknown. Current case: could be due to genital area inflammation and superadded infection
In the current case, less possible causes include mucus membrane pemphigoid, erosive lichen planus and lichen sclerosus.
No guideline available for labial adhesion but few studies have recommended topical estrogen or steroid application for 4-6 weeks in prepubertal girls and surgical therapy in reproductive women as first line.
CONCLUSION
Ulcerative mucocutaneous lesions like pemphigus vulgaris should be kept as a differential diagnosis of labial adhesion in relevant clinical scenario.
Two-step procedure (topical therapy followed by manual separation of adhesion band) can be tried with thick adhesion band as it will avoid surgery related risk.
