A Rare Case of Cutaneous T-Cell Lymphoma in the Foot: Woringer-Kolopp Disease

I received a consult for an 84-year-old male with worsening appearance and malodor of a full-thickness ulcer on his right medial foot. The patient had dementia and had been at the hospital’s behavioral unit for several weeks. The patient and hospital staff could not give any history about the wound, such as the chronicity, or any previous treatment. Surprisingly, his lab work was unremarkable. He did have positive methicillin-sensitive Staphylococcus aureus (MSSA) blood cultures, most likely from the foot wound. A magnetic resonance imaging (MRI) study was contraindicated due to metal present from a hip replacement. Computed tomography (CT) with and without contrast did not show any bone involvement, subcutaneous emphysema or abscess. For infection source control, I planned to excise the entire ulcer plus some normal skin margins and send it to pathology. I contacted the patient’s daughter to obtain more history of the wound and to obtain consent for the surgery.

The history was convoluted due to the patient’s daughter being out of state and her often only receiving second-hand information from the patient’s caregiver. A rash on the medial foot and lip appeared at approximately the same time. Dermatology evaluated the patient and performed a shave biopsy of the lip first. The pathology results were inconclusive. The patient had more complaints about the lip lesion which appeared similar to the foot lesion–an erythematous, plaque and nodular-type lesion. Within one month, the right foot lesion began to bleed and drain with wound cultures being positive for MSSA. He was placed on cephalexin and trimethoprimsulfamethoxazole without improvement. His dermatology provider then took a shave biopsy of the right foot skin lesion, the results of which were also inconclusive.

Subsequently, he was referred to an infectious disease physician who was suspicious of a malignant process and ran some blood work, however, the patient did not follow up. From the information that I gathered from the dermatology, infectious disease and wound care offices, the skin lesion began as a 3.0 x 3.0 cm nodule with an excoriated center. By the time I joined the case, the lesion on his right foot had been present for approximately 6 months with progression to a full-thickness infected ulcer that measured 7.0 x 5.0 x 0.5 cm with exposed muscle, a wound bed that contained nodules and slough necrosis, and heavy malodorous exudate (see first photo). After obtaining consent for surgery, I performed a wide excision of the wound and sent it to pathology. The patient had tunneling, which was why there were ancillary incisions from the wound of origin (see second photo).

The preliminary pathology report stated that the sample was consistent with T-cell lymphoma, and the pathologist consulted an expert hematopathologist outside of the hospital system. The final pathology and molecular genetics reports revealed that the findings were consistent with a cutaneous T-cell lymphoma that was positive for rearrangement of the T-cell gamma and beta receptor genes, most likely representing a variant of mycoides fungoides called pagetoid reticulosis, or Woringer-Kolopp disease. Due to the ongoing gradual decline of the patient, the family decided to place the patient under at-home hospice care. Unfortunately, he passed away two days later.

So, what exactly is Woringer-Kolopp disease, or pagetoid reticulosis? This disease is a malignant and rare lymphoproliferative condition that is a variant of mycosis fungoides, which is the more common type of cutaneous T-cell lymphoma or non-Hodgkin lymphoma.¹ While mycosis fungoides account for roughly 1 percent of all cutaneous T-cell lymphomas, Woringer-Kolopp disease only accounts for 0.6 percent of reported mycosis fungoides cases.^1,2 In other words, this malignant skin lesion is rare. The skin lesion is often clinically described as a solitary lesion with underlying plaque formation found mostly on the extremities.^1,3 Immunohistochemical and genotype evaluation of Woringer-Kolopp disease displays unique epidermotropism patterns that distinguishes it from other variants of mycosis fungoides.^1-4 Treatment options depend on involvement of the disease. Studies have shown patients being treated successfully with wide excision with radiation therapy, while some patients respond to chemotherapy, infusions, topical corticosteroids, phototherapy with UVB or UVA. Even with those treatments described, recurrence can occur.^1,2

Woringer-Kolopp disease can resemble non-malignant skin conditions such as tinea pedis or corporis, impetigo, as in this case, eczema, psoriasis, an infected ulcer, and other plaque-forming conditions.² Because of this, and confusing nomenclature, there is commonly a delay in diagnosis and underreporting of cases, respectively. This case has reminded me of the importance of obtaining a history beyond the patient, and not forgetting my clinical acumen and training in biopsy techniques. I hope that this case helps you consider performing wide excisions of wounds versus debridement alone and sending your specimen to a pathology lab that has the expertise and technologies to determine the correct diagnosis that may improve or save your patients’ lives.

Dr. Saysoukha is a foot and ankle surgeon at Premier Foot & Ankle Centers of Tennessee, an AdvancedHEALTH practice. She is a Fellow of the American Society of Podiatric Surgeons, a Fellow of the American College of Podiatric Medicine, and an Associate of the American College of Foot and Ankle Surgeons. She is board certified by the American Board of Podiatric Medicine and board qualified by the American Board of Foot & Ankle Surgery in Foot and Reconstructive Rearfoot and Ankle Surgery.

References

1. James WD, Berger TG, Neuhaus IM, Elston DM. Cutaneous lymphoid hyperplasia, cutaneous T-cell lymphoma, other malignant lymphomas, and allied diseases. In: Andrew's disease of The Skin: Clinical Dermatology (12^th ed) Elsevier;2016:727-733. 

2. Kim EJ, Hess S, Richardson SK, et al. (2005). Immunopathogenesis and therapy of cutaneous T cell lymphoma. J Clin Invest. 2005;115(4):798-812. https:doi.org10.1172JCI24826.

3.  Haghighi B, Smoller B, LeBoit P, et al. Pagetoid reticulosis (Woringer-Kolopp disease): An immunophenotypic, molecular, and clinicopathologic study 2. Mod Pathol 2000;13:502–510. https:doi.org10.1038modpathol.3880088.

4.  Larson K, Wick MR. Pagetoid reticulosis: Report of two cases and review of literature. Dermatopathol. 2016;3:8-12. https:doi.org10.1159000444660.