Test your diagnostic skills with the following dermatology quiz. Answers are on page 2.
A 31-year-old man presented in May 2007 with generalized painful, ulcerated, and necrotic papules and plaques worsening for the last few months. He had been diagnosed with mycosis fungoides in October 2005. Treatments included topical corticosteroids and psoralen with ultraviolet A light therapy (PUVA), with the latter being discontinued because of the development of blisters. For the last 6 months, he had been treated with oral bexarotene (Targretin), with initial improvement of his skin lesions.
On physical examination, there were painful, erythematous hyperkeratotic, crusted, annular and circinar plaques and papules, many with central ulceration and drainage of serosanguinous fluid, involving scalp, face (including mouth), neck, trunk, and extremities (see Figures 1A and 1B). He was afebrile, and the vital signs were normal. There was cervical and axillary lymphadenopathy but no hepatosplenomegaly noted. The remainder of the physical examination was unremarkable.
Laboratory evaluation showed a normal blood cell count except for elevated platelet count 4.3 105/L. Comprehensive chemistry panel was normal except for mildly elevated LDH 224 U/L. Sézary cell count was negative. A punch biopsy was performed (see Figures 2A and 2B).
1) The photographs reveal findings noted at the time of a diagnostic and therapeutic procedure. What is your diagnosis?
a) Bullous erythema multiforme
b) Mycosis fungoides CD8+ cytotoxic T-cell lymphoma
c) Disseminated squamous cell
d) Cutaneous epidermotropic
e) Necrotizing vasculitis carcinomas
1. Lu D, Patel KA, Duvic M, et al: Clinical and pathological spectrum of CD8-positive cutaneous T-cell lymphomas. J Cutan Pathol 29:465-472, 2002.
2. Willemze R, Jaffe ES, Burg G, et al: WHO-EORTC classification for cutaneous lymphomas. Blood 105:3768-3785, 2005.
3. Berti E, Tomasini D, Vermeer MH, et al: Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas: A distinct clinicopathological entity with an aggressive clinical behavior. Am J Pathol 55:483-492, 1999.
4. Vermeer MH, van Doorn R, Dukers D, et al: CD8+ T cells in cutaneous T-cell lymphoma: Expression of cytotoxic proteins, Fas ligand, and killing inhibitory receptors and their relationship with clinical behavior. J Clin Oncol 19:4322-4329, 2001.