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Case Reports

Abstract

INTRODUCTION

Vulvar mycosis fungoides (MF) is exceptionally rare. To the best of our knowledge this is the first reported case of vulvar granulomatous MF.

CASE DESCRIPTION

We describe a case of a 64-year-old lady with a 3 year history of scaly, eczematous swelling of the vulva. Initial biopsies showed granulomatous inflammation with a differentials that included infection, Crohn’s disease and sarcoidosis. The lesion gradually spread to the perineum, buttock and upper gluteal cleft, became indurated and ulcerated. Pendulous skin was absent. Her extensive investigations included a normal serum ACE, microbiological investigations, radiology and colonoscopy. The subsequennt incisional biopsy showed confluent interstitial, perivascular and periadnexal non-necrotizing granulomas extending through the dermis into the subcutis. There were multinucleated giant cells and scant lymphocytic cell infiltrate with minimal cytological atypia. There was no epidermotropism, folliculotropism or syringotropism. The lymphoid infiltrate was predominantly CD4 T cells with no loss of CD2, CD3 and CD5, but complete loss of CD7. Monoclonality was proven with T-cell gene rearrangement studies. The patient’s lesions responded well to 10 cycles of 20Gy VMAT radiotherapy.

DISCUSSION AND CONCLUSION

Granulomatous MF lacks specific clinical features. There may be no prominent epidermotropism or minimal cytological atypia, Furthermore, the granulomatous component may obscure the lymphomatous component. The differential to consider is granulomatous slack skin syndrome, a rare variant of MF characterised by pendulous skin folds in flexural areas and histologically indistinguishable from granulomatous MF. In addition, early treatment may arrest the development of hanging skin folds. This case highlights the need to consider lymphoma in granulomatous vulvar lesions.