A 51-year-old man slowly developed a 15 cm × 8 cm erythematous, notably indurated, and variably scaly plaque located on the medial and dorsal aspect of the right foot. By the time he sought medical attention, this minimally pruritic lesion had been present about 2.5 years. The rest of the cutaneous examination was unremarkable. Ipsilateral inguinal adenopathy was not detected. Several skin biopsies showed a similar pattern, consisting of hyperkeratosis and acanthosis accompanied by an intraepidermal clonal infiltrate of CD8+ atypical lymphocytes with pleomorphic nuclei, and a dense upper dermal infiltrate composed of more benign-appearing lymphocytes.
C. Woringer-Kolopp disease
This picture is quite classic for the disease entity “localized pagetoid reticulosis,” also known as Woringer-Kolopp disease (WKD). Ketron-Goodman disease is similar histologically, but is typically characterized by diffuse cutaneous involvement, a progressive course, and a poor outcome. The World Health Organization (WHO)-European Organisation for Research and Treatment of Cancer (EORTC) classification considers WKD to be a cutaneous T-cell lymphoma, namely an indolent variant of mycosis fungoides.[3,4] However, there are some histologic differences that may allow differentiation. In contrast to WKD, classic mycosis fungoides is typically CD4+, atypical lymphocytes are located both in the dermis and epidermis, eosinophils are admixed among the atypical lymphocytes, and neither hyperkeratosis nor acanthosis is necessarily present. Moreover, from a clinical standpoint, WKD runs a relatively long and slow course, resulting in neither disease-related significant morbidity nor mortality.[1,4,6,7]
The incidence and gender distributionof WKD are both difficult to define, and the pathogenesis remains unknown. There is usually considerable delay in diagnosis due to indolent and asymptomatic growth; hence, patients often experience a long history of incorrect diagnosis and inappropriate therapy.[7,8] The diagnosis of WKD should always be entertained when a slowly expanding, psoriasiform or hyperkeratotic plaque is detected on a distal extremity, particularly the foot. Contact dermatitis, atopic eczema, blastomycosis, chromomycosis, Bowen disease, extramammary Paget disease, and psoriasis vulgaris may also be in the clinical differential diagnosis. Although usually asymptomatic, WKD may occasionally be associated with lesional pruritus or pain. WKD is a true malignant lymphoma of T-cell lineage. Very rare instances of progression to disseminated lymphoma have been reported, but this remains the exception rather than the rule.[7,10]
A great many therapeutic options have been utilized for WKD. Traditionally, complete surgical removal resulted in adequate treatment for many, if not most, patients.[10,11] However, larger lesions (such as the one depicted here) may prove to be surgically challenging, and this approach may result in undesirable functional or cosmetic outcomes. Thus, localized orthovoltage or electron beam radiation therapy, which is quite effective, has largely displaced surgery as the treatment of choice for WKD.[5,8,12]
1. Cribier B: History: Frederic Woringer (1903-1964) and Woringer-Kolopp disease. Am J Dermatopathol. 2005;27:534–545.
2. Nakada T, Sueki H, Iijima M. Disseminated pagetoid reticulosis (Ketron-Goodman disease): Six-year follow-up. J Am Acad Dermatol. 2002;47:183–86.
3. Matsuzaki Y, Kimura K, Nakano, H, et al. Localized pagetoid retidulosis (Woringer-Kolopp Disease) in early childhood. J Am Acad Dermatol. 2009;61:120–23.
4. Burns MK, Chan LS, Cooper KD. Woringer-Kolopp Disease (localized pagetoid reticulosis) or unilesional mycosis fungoides? An analysis of eight cases with benign disease. Arch Dermatol. 1995;131:325–29.
5. Lee AD, Cohen PR. What is Woringer-Kolopp Disease? SKINmed. 2013;11:17–20.
6. Haghighi B, Smoller BR, LeBoit PE, et al. Pagetoid reticulosis (Woringer-Kolopp Disease): an immunophenotypic, molecular and clinicopathologic study. Mod Pathol. 2000;13:502–10.
7. Cho-Vega JH, Tschen J, Duvic M, et al. Early-stage mycosis fungoides variants: Case-based review. Ann Diagn Pathol. 2010;14:369–85.
8. Lee J, Viakhireva N, Cesca C, et al. Clinicopathologic features and treatment outcomes in Woringer-Kolopp disease. J Am Academy of Dermatol. 2008;59:706–12.
9. Palmer RA, Keefe M, Slater D, et al. Case 4: pagetoid reticulosis (Woringer-Kolopp type) or unilesional mycosis fungoides (MF). Clin Exp Dermatol. 2002;27:345–46.
10. Zackheim HS. Is “localized epidermotropic reticulosis” (Woringer-Kolopp disease) benign? J Am Acad Dermatol. 1984;11:276–83.
11. Martin SJ, Cohen PR, Cho-Vega JH, et al. CD8+ Pagetoid reticulosis presenting as a solitary foot plaque in a young woman. J Clin Aesthet Dermatol. 2010;3:46–49.
12. Muhlhoff C, Krenkel B, Rubben A, et al. Pagetoid reticulosis in a patient with mycosis fungoides: Successful therapy with localized electron beam irradiation. Hautarzt. 2010;61:378–82.