Asymptomatic Macules and An Ulcerated Shoulder in an Elderly Woman

Antonio Giovanni Richetta, MD, PhD; Simona Giancristoforo, MD; Carlo Mattozzi, MD; Sara D'Epiro, MD; Stefano Calvieri, MD


December 09, 2016


The diagnosis of primary cutaneous aggressive epidermotropic CD8+ cytotoxic T- cell lymphoma was established according to the World Health Organization (WHO) and European Organization for Research and Treatment of Cancer (EORTC) classification. The WHO-EORTC classification for primary cutaneous lymphoma includes mycosis fungoides, Sézary syndrome, the group of the primary cutaneous CD30 lymphoproliferations, and subcutaneous panniculitis-like T-cell lymphomas.

Each diagnosis above is recognized as a distinct, well-defined entity, which together constitute approximately 85% of all primary cutaneous T-cell lymphomas (CTCL). In the WHO-EORTC classification, most cases of primary CTCL that do not belong to one of above entities are included in the group of unspecified primary cutaneous peripheral T-cell lymphomas (PTLs), and rare cases such as extranodal NK/T-cell lymphoma, nasal type.[1] In the unspecified group, primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, cutaneous gamma/delta T-cell lymphoma, and primary cutaneous small-medium CD4+ T-cell lymphoma are separate provisional entities. For the remaining diseases that do not fit into either of these provisional entities, the designation unspecified primary cutaneous PTL is used.

Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma is a rare subtype of CTCL. It is a distinct and clinically aggressive form of CTCL that accounts for less than 1% of all cases of cutaneous lymphoma and carries a very poor prognosis.[2,3,4] The clinical course is extremely rapidly progressive, with an average 5-year survival estimated at 18% compared with the typical 88% in the more indolent mycosis fungoides. The EORTC Cutaneous Lymphoma Task Force's data reported that all 4 patients died from their disease within 3 years of diagnosis, with a median survival of only 22.5 months.[4] Patients affected by primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma typically have rapid progression with extra-cutaneous dissemination that affects quality-of-life and the overall prognosis. The pathogenesis or genetic characteristics of this disease are not known as this type of lymphoma is extremely rare and only a limited number of cases have been reported.

Clinically, the disease usually presents with localized or disseminated eruptive papules, nodules, and tumors showing central ulceration and necrosis, or with superficial, hyperkeratotic patches and plaques.[2,3] Unlike classic mycosis fungoides, patients do not generally follow the typical progression through patch, plaque, and finally tumor-stage disease, but rather present from the onset with widespread plaque- and tumor-stage disease.[4] However, mycosis fungoides, pagetoid reticulosis, and Sézary syndrome are included in the differential diagnosis. The clinical features of this disease are in fact very similar to those observed in patients with a cutaneous gamma/delta T-cell lymphoma and cases described as generalized pagetoid reticulosis (Ketron-Goodman type) in the past.[1]


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