A Case of Ketron-Goodman Disease

Abstract

Pagetoid reticulosis (PR) is a rare form of cutaneous T-cell lymphoma [Mod Pathol 2000;13:502–510]. Two variants of the disease are described: the localized type Woringer-Kolopp disease (WKD) and the disseminated type Ketron-Goodman disease (KGD). KGD may have disseminated lesions, high rate of recurrence and a guarded prognosis [Mod Pathol 2000;13:502–510]. In patients with KGD, therefore, long-term observation is necessary. Disappearance of cutaneous lesions does not mean resolution of the disease [J Am Acad Dermatol 2002;47:183–186]. Herein we report the case of an 84-year-old man with erythematous patches of the trunk and the upper and lower extremities in whom the diagnosis of KGD was made. We describe this case for the rarity of this pathology and for the good response to therapy (IFN).

Case for diagnosis

“…1 In contrast, the few published cases of KGD report fatal outcomes, systemic involvement and interferon therapy, with an 8-month followup without relapse. 5,6 In our case, a follow-up of 2 years without recurrences or systemic involvement favors an indolent form of cutaneous T-lymphoma. Long-term observation is however necessary, considering that even if the cutaneous lesions clear up this may not mean resolution of the disease, since the disease may recur as long as 10 years after therapy.…”

Rapidly progressive hyperpigmented keratotic plaques with epidermotropism

“…However, skin-limited disease has been reported rarely, similar to our case. 5 Histological features include dense dermal lymphocytic infiltrates, with marked pagetoid epidermotropism composed of pleomorphic small-to medium-sized T-cells. The neoplastic T-cells have a CD8+/CD4-cytotoxic T-cell profile with granzyme B, perforin and TIA-1 marker expression.…”

Pagetoid Reticulosis: Report of Two Cases and Review of the Literature

“…Recently documented cases of PR [ 12 , 13 , 14 , 15 , 16 , 17 ] have included patients of all ages, including children. Localized perioral lesions have also been reported [ 18 ] as well as a generalized form of the disorder known as Ketron-Goodman disease [ 19 ]. On dermoscopy, PR has been said to show features that are similar to those of Bowen's disease.…”