Disseminated recurrent papular B‐cell pseudolymphoma

Abstract

A 41‐year‐old man in apparently good health presented in June 1989 with a moderately pruritic disseminated papular eruption of 2 weeks duration. The lesions were smooth brown‐red papules, 3–8 mm in size and most prominent on the arms, chest, and legs. Past history was remarkable for perennial allergic rhinitis and bronchospasm with seasonal exacerbation in May‐July. Allergy evaluation revealed sensitivity to house dust, mites, and grass pollens. Desensitization shots were started after the eruption had appeared. Medications included terbutaline inhaler, cromolyn ophthalmic solution, beclomethasone nasal spray, clemastine fumarate, and terfenadlne. A biopsy from a papule on the right arm was interpreted by two outside pathologists as atypical lympho‐reticular infiltrate.Left axillary adenopathy was detected but lymph node biopsy revealed only slight reactive changes. Computed tomography of the chest, abdomen, and pelvis showed no significant adenopathy. Complete blood count, serum protein electrophoresis, VDRL, and chemistry panel were within normal limits or negative except for a slightly elevated alanine aminotransferase of 52 U/L (normal, 0–45). The erythrocyte sedimentation rate (ESR) was elevated at 58 mm/h (normal, 0–10). The patient recalled tick bites a few months before the eruption but had no signs or symptoms of Lyme disease. Titers of Borrelia burgdorferi antibodies were within normal limits.Four months later he presented with about 10 papules on the trunk and extremities. The lesions were smooth without crusting (Figs 1, 2). An excisional biopsy from a papule on the chest revealed a mid‐dermal nodular infiltrate in which there were lymphoid follicles with a typically heterogeneous composition surrounded by mantle zones of smaller lymphocytes. The infiltrate between follicles consisted of lymphocytes, histiocytes, enlarged mononuclear cells, eosinophlls, and occasional plasma ceils (Fig. 3). Cells intermediate between plasma cells and small lymphocytes, or plasma cells and immunoblasts, as occur in immunocytoma, were not seen. Features typical of lymphomatoid papulosis, such as large atypical lymphocytes, fibrin in vessel walls, edema, and infiltration of the epidermis by small lymphocytes, were also not seen. Several venules had swollen endothelial cells.Avidin‐biotin immunoperoxidase studies were carried out. BER‐H2 (CD30) showed only scattered positivity of dermal mononuclear cells. The UCHL1 (CD45RO) stain, which recognizes memory T cells, showed heavy staining of much of the infiltrate in‐between, follicles with scattered staining of the follicular structures with moderate staining of the surrounding mononuclear cells. Frozen‐section immunoperoxidase studies performed at Stanford University demonstrated a diffuse T‐cell infiltrate without pan T‐cell antigen loss, and scattered B cells with an admixture of kappa and lambda expressing cells. T‐cell receptor probes used in a Southern blot analysis of extracted DNA from frozen tissue did not show diagnostic rearranged bands. T‐cell receptor genes, rather than immunoglobulln genes, were analyzed because of the clinical suspicion of lymphomatoid papulosis. On the basis of the histology and special studies a final diagnosis of cutaneous lymphoid hyperplasia was made.While under observation the patient identified various papules which, he positively stated, waxed and waned at the same lesion sites. Thus a 5‐mm papule on the right elbow (Fig. 2) wqs said to have increased and decreased in height at the same location 6–8 times during that period. Owing to the fact that, because of distance, the patient was seen only about every 3 months, this could not be verified; however, his account of this behavior was spontaneous.To rule out a drug‐induced eruption his allergy medications were not taken from fall to spring of 1989–90 and 1990–91, but there was no improvement while he was off drugs. Medium and high potency steroid creams only relieved pruritus. Tetracycline 250–1000 mg daily was without significant benefit. Repeat chest X‐rays, blood counts, chemistries, and ESR showed no significant abnormalities.The patient continued to have recurrent papules on the trunk and extremities, but not on the head or neck, for the next 3 years, although with decreasing intensity until January 1992 when he was free of lesions. He has had no recurrence for almost 4 years.