Invisible mycosis fungoides: a diagnostic challenge.
We describe a 76-year-old woman who had 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 biopsy specimens 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 J(8)V(2)C(2) 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.
Pujol
Ramon M
RM
Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. rmpujol@usa.net
Gallardo
Fernando
F
Llistosella
Enric
E
Blanco
Aurora
A
Bernadó
Lluís
L
Bordes
Ramon
R
Nomdedeu
Josep F
JF
Servitje
Octavio
O
D000368:Aged; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D009182:Mycosis Fungoides / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology; D016133:Polymerase Chain Reaction; D011537:Pruritus / Q000209:etiology; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology