ACQUIRED CUTIS LAXA ASSOCIATED WITH CUTANEOUS ANGIOCENTRIC T-CELL LYMPHOMA

A 64-year-old man presenting with adenomegaly was diagnosed with stage IV A angiocentric cutaneous T-cell lymphoma in 1988. Since he showed a poor response to psoralen plus UVA (PUVA) after 1 year of therapy, h e was switched to systemic chemotherapy (COP), and was considered to b e in remission after completion of 5 months of this regimen. In Decemb er 1989, the patient noted an asymptomatic lesion at the superior and internal aspect of his right thigh; on examination, there was a poorly circumscribed, noninflammatory, whitish, atrophic macula near the ing uinal fold and a palpable adenopathy; its surface was irregular, showi ng small wrinkling, and could be depressed on palpation. There were no preceding inflammatory lesions and no cutaneous lymphoma lesions at t his site. Slowly, the initial macule extended, and new similar lesions insidiously appeared symmetrically on the left thigh. In October 1990 , the patient received electron beam therapy for a relapse that presen ted as troncular nodules and responded well to this therapy. In the mo nths that followed, the skin of his whole body became progressively sa gging and pendulous, without recoil. This was not preceded by an infla mmatory phase or eruption. There was no family history of elastolytic disorder, and the patient was not taking any medication other than tha t described above. On questioning, there were no symptoms suggesting s ystemic involvement of this elastolytic disorder. On physical examinat ion, the patient's whole body skin seemed redundant, nonresilient, and wrinkled, this being most obvious in the axillae, inguinal region, an tecubital fossa, neck, and trunk, where loose and pendulous skin folds could be seen (Fig. 1). Also, the patient's face was excessively wrin kled and sagging and he had bilateral ectropion. At the superior and i nternal aspect of both thighs, large, poorly circumscribed, but distin ct, round patches of 1-5 cm in diameter were observed; they were atrop hic and whitish, with an uneven surface, and could be depressed on pal pation. Apart from 1 cm, mobile, but firm, axillary and inguinal adeno pathy and three subcutaneous nodules on the arm and trunk, the rest of the physical examination was unremarkable. Routine blood tests were n ormal, as was a chest X-ray. Sezary cells were absent on blood smear, and borrelia and syphilis serology were negative. Thyroid function tes ts, angiotensin converting enzyme, and immunoelectrophoresis were with in normal limits, and antinuclear antigen was negative. Two cutaneous biopsies were performed: one in an atrophic herniating lesion of the r ight thigh and the one on the abdomen. Both showed, on hematoxylin-phl oxin-safran staining, a mild dermal perivascular inflammatory infiltra te with a predominance of mononuclear cells. On Weigert staining, both were marked by a net reduction in elastic fibers throughout the dermi s.