Acquired cutis laxa associated with cutaneous angiocentric T‐cell lymphoma

Abstract

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, he was switched to systemic chemotherapy (COP), and was considered to be in remission after completion of 5 months of this regimen. In December 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 inguinal fold and a palpable adenopathy; its surface was irregular, showing small wrinkling, and could be depressed on palpation. There were no preceding inflammatory lesions and no cutaneous lymphoma lesions at this 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 presented as troncular nodules and responded well to this therapy. In the months that followed, the skin of his whole body became progressively sagging and pendulous, without recoil. This was not preceded by an inflammatory phase or eruption. There was no family history of elastolytic disorder, and the patient was not taking any medication other than that described above. On questioning, there were no symptoms suggesting systemic involvement of this elastolytic disorder. On physical examination, the patient's whole body skin seemed redundant, nonresllient, and wrinkled, this being most obvious in the axillae, inguinal region, antecubital fossa, neck, and trunk, where loose and pendulous skin folds could be seen (Fig. 1). Also, the patient's face was excessively wrinkled and sagging and he had bilateral ectropion. At the superior and internal aspect of both thighs, large, poorly circumsoribed, but distinct, round patches of 1‐5 cm in diameter were observed; they were atrophic and whitish, with an uneven surface, and could be depressed on palpation. Apart from 1 cm, mobile, but firm, axillary and inguinal adenopathy and three subcutaneous nodules on the arm and trunk, the rest of the physical examination was unremarkable.Routine blood tests were normal, as was a chest X‐ray. Sezary cells were absent on blood smear, and borrelia and syphilis serology were negative. Thyroid function tests, angiotensin converting enzyme, and immunoelectrophoresis were within normal limits, and antinuclear antigen was negative. Two cutaneous biopsies were performed: one in an atrophic herniating lesion of the right thigh and the one on the abdomen. Both showed, on hematoxylin‐phloxin‐safran staining, a mild dermal perivascular inflammatory infiltrate with a predominance of mononuclear cells. On Weigert staining, both were marked by a net reduction in elastic fibers throughout the dermis.