A case of cutaneous malakoplakia in a patient with chronic graft-versus-host disease

Background: Malakoplakia is a rare condition associated with infections, tumours and primary or secondary immunodeficiency.(1) Cutaneous malakoplakia is a rare variant most commonly seen in older males, often in the perianal or genital area in relation to immunosuppression.(1) We present a case of cutaneous malakoplakia in a patient with graft-versus-host-disease.
Main Observations: 62-year-old woman on oral immunosuppression for graft-versus-host-disease secondary to a bone marrow transplant for treatment of chronic lymphocytic leukaemia presented with yellow-coloured cutaneous nodules, some of them ulcerated on the genital and perianal region. An excisional biopsy was performed and the specimen was sent for histology and bacterial, mycobacterial and fungal culture. The haematoxylin and eosin stain showed a focal ulceration and acute inflammation in the epithelium. Numerous round calcospherites were seen in keeping with the diagnosis of malakoplakia. The calcospherites stained positively for von Kossa and Periodic-acid Schiff stain. The specimen had a positive bacterial culture showing heavy growth of Escherichia coli, and was negative for mycobacterium or fungi. A course of oral ciprofloxacin led to a significant reduction in the number and size of malakoplakia lesions, which was completed once azathioprine was ceased and prednisone reduced.
Conclusion: The diagnosis of cutaneous malakoplakia should be considered in immunosuppressed patients presenting with cutaneous nodules in the genital and perianal area. Directed antibiotic therapy in combination with local surgical excision has shown the highest chance for cure. Reduction or discontinuation of immunosuppressive drug therapy is also usually required.(2)
1. Coates M, Del Pero MM, Nassif R. A Case of Cutaneous Malakoplakia in the Head and Neck Region and Review of the Literature. Head Neck Pathol. 2016.
2. Shawaf AZ, Boushi LA, Douri TH. Perianal cutaneous malakoplakia in an immunocompetent patient. Dermatol Online J. 2010;16(1):10.

Dr. Yvonne Nguyen

1) Dermatology Department, Royal Prince Alfred Hospital 2) Discipline of Dermatology, Sydney Medical

Yvonne Nguyen is currently a dermatology research registrar at the Royal Prince Alfred Hospital after having previously completed her internship and residency at the Royal North Shore Hospital. She received Bachelor of Pharmacy (Hons), MBBS and MPhil (Medicine) degrees from The University of Sydney. Her main research experience is in the area of Chronic Vulvovaginal Candidiasis, which she undertook in fulfilment of her Master of Philosophy (Medicine) degree from The University of Sydney.