Invisible mycosis fungoides: A diagnostic challenge

Pujol, Ramon M. MD
Gallardo, Fernando MD
Llistosella, Enric MD
Blanco, Aurora MD
Bernadó, Lluís
Bordes, Ramon MD
Nomdedeu, Josep F. MD
Servitje, Octavio MD

Barcelona and Girona, Spain
From the Hospital Princeps d'Espanya,^a Barcelona; Hospital Josep Trueta,^b Girona; Institut de Reçerca Oncologica,^c Barcelona; and Departments of Dermatology, Pathology,^d and Hematology,^e Hospital de la Santa Creu i Sant Pau, Barcelona, Spain

This supplement is made possible through an educational grant from Ortho Dermatological to the American Academy of Dermatology.

Reprint requests: Ramon M. Pujol, MD, Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Avda. Sant Antoni M. Claret 167, 08025-Barcelona. Spain. E-mail: [email protected]

0190-9622/2000/$12.00 + 0 16/4/98276

Journal of the American Academy of Dermatology 42():p 324-328, February 2000. | DOI: 10.1016/S0190-9622(00)90103-8

We describe a 76-year-old woman who presented persistent generalized pruritus as the only cutaneous manifestation of a cutaneous T-cell lymphoma (mycosis fungoides). No cutaneous lesions were observed throughout the patient's course. Skin biopsies obtained from normal-looking pruritic skin revealed a discrete perivascular lymphocytic infiltrate in the upper dermis and focal intraepidermal clusters of atypical lymphoid cells (Pautrier's microabscesses). PCR analysis of TCR-gamma gene disclosed a monoclonal T-cell rearrangement. Sequencing of the PCR monoclonal product identified the J₈V₂C₂ TCR gene rearrangement. This observation illustrates the existence of a peculiar and exceedingly rare form of mycosis fungoides characterized only by persistent pruritus unresponsive to several therapeutic approaches. The diagnostic difficulties of this rare variant are stressed. (J Am Acad Dermatol 2000;42:324-8.)