SKIN May 2023 Volume 7 Issue 3 (c) 2023 THE AUTHORS. Published by the National Society for Cutaneous Medicine. 834 BRIEF ARTICLE Bazex Syndrome Associated with Angioimmunoblastic T-cell Lymphoma Gregory Ugoh, BS1, Anthony Linfante, MD1, Allison Good, MD1, Janice Wilson, MD1, Kathleen Kroger, MD1 1The University of Texas Medical Branch at Galveston, Galveston, TX Bazex syndrome was first described by Bazex et al. in 1965 in association with malignancy of the upper aerodigestive tract.1 The main features are the presence of symmetrical psoriasiform eruptions, nail dystrophy, and xerotic scaling usually accentuated on the acral surfaces, ears, and nose.2 Several mechanisms for development of Bazex syndrome have been proposed. One theory suggests that antibodies against the tumor cross react with the keratinocytes or basement membrane leading to damage of the basal layer of the skin. Alternatively, an immune reaction directed against tumor-like antigens in the epidermis could be responsible for the cutaneous eruptions. Association with cutaneous squamous cell carcinoma, Hodgkin’s disease, peripheral T- cell lymphoma, and follicular lymphoma in- situ have been reported.3 We present a unique case of Bazex syndrome associated with AITL. To our knowledge, this is the first reported case of Bazex syndrome seen in association with AITL. A 60-year-old male presented with a three- month history of fever, diarrhea, xerosis, and a 20-lb weight loss. The patient reported a several-week history of a non-pruritic rash on his face and extremities. On physical exam, xerotic scaly plaques were noted on the ears, nose, and scalp, with ill-defined xerosis and adherent scale of the lower legs. Desquamating keratoderma was seen on the palms, soles, and dorsal hands, along with Beau’s lines of the fingernails ABSTRACT Bazex syndrome, also known as Acrokeratosis paraneoplastica, is a paraneoplastic disorder characterized by erythematous psoriasiform plaques involving the nose, ears, and acral sites. Although classically associated with squamous cell carcinomas of the upper aerodigestive tract, it has also been reported in association with adenocarcinoma, genitourinary tumors, multiple myeloma, and rarely, peripheral T-cell lymphoma and follicular lymphoma in-situ. Herein, we present a patient with Bazex syndrome associated with angioimmunoblastic T-cell lymphoma (AITL), a rare association not previously reported in the literature. INTRODUCTION CASE REPORT SKIN May 2023 Volume 7 Issue 3 (c) 2023 THE AUTHORS. Published by the National Society for Cutaneous Medicine. 835 Figure 1. (A) Psoriasiform eruption of ear (B) Beau’s lines on fingernails and scaling of hands (C) Xerotic scaly plaques on plantar feet Figure 2. H&E1 (20x): Psoriasiform acanthosis with broad and blunt rete ridges, H&E 2 (200x): Confluent parakeratosis and underlying hypogranulosis bilaterally (Figure 1). Diffuse lymphadenopathy was appreciated. CT scans revealed extensive lytic lesions throughout the spine, ribs, and scapula. Lymphoma was suspected and a lymph node biopsy revealed AITL. A tangential biopsy and punch biopsy were performed, revealing psoriasiform acanthosis with broad and blunt rete ridges, along with hypogranulosis, confluent parakeratosis, and minimal inflammatory infiltrate (Figure 2). The histological findings and clinical picture were consistent with Bazex syndrome due to underlying AITL. The patient was then started SKIN May 2023 Volume 7 Issue 3 (c) 2023 THE AUTHORS. Published by the National Society for Cutaneous Medicine. 836 on topical steroids and urea with some improvement. However, the patient expired from angioimmunoblastic T-cell lymphoma shortly thereafter. Bazex syndrome is a paraneoplastic disorder of the skin characterized by erythematous psoriasiform plaques in an acral distribution that classically also involve the nose and ears. Histopathologic features of Bazex syndrome are variable, but usually include acanthosis, hyperkeratosis, and parakeratosis. Inconstant features include variable spongiosis and dyskeratosis.4-5 While the histologic features are not always diagnostic, the absence of diagnostic features of other conditions and clinicopathologic correlation are key to rule out other entities on the differential diagnosis. In our patient, the presence of confluent parakeratosis excluded the possibility of malignancy- associated ichthyosis. Furthermore, the absence of other diagnostic features of psoriasis (i.e., neutrophilic microabscesses), in conjunction with the patient’s clinical presentation, excluded this possibility as well. The favored therapy for this paraneoplastic dermatosis is effective treatment of the underlying malignancy. For recalcitrant or palliative care, systemic treatments include corticosteroids, fluconazole, zinc, and cephalexin. Topical treatments include retinoids, zinc ointment, PUVA, and emollients. Although Bazex syndrome is most commonly associated with squamous cell carcinoma of the upper aerodigestive tract, it has also been reported in association with adenocarcinoma, genitourinary tumors, and rarely, peripheral T-cell lymphoma and follicular lymphoma. Herein, we present the first case of Bazex syndrome seen in association with AITL. Conflict of Interest Disclosures: None Funding: None Corresponding Author: Gregory Ugoh Ugoh The University of Texas Medical Branch at Galveston Email: gaugoh@utmb.edu References: 1. Humphrey SR, Hussain AS, Chandran R, Wilson B, George B. Acute Onset of Acrokeratosis Paraneoplastica (Bazex Syndrome). JAMA Dermatol. 2015;151(6):677–678. doi:10.1001/jamadermatol.2014.5622 2. Bazex A, Salvador R, Dupré A, et al. Syndrome paranéoplasique à type d'hyperkératose des extrémités: guérison après le traitement de l'épithélioma laryngé. Bull Soc Fr Dermatol Syphiligr. 1965;72:182. 3. Santos-Silva AR, Correa MB, Vargas PA, Almeida OP, Lopes MA. Bazex syndrome (acrokeratosis paraneoplastica) diagnosed in a patient with oral persistent ulcerations. Head Neck Pathol. 2010;4(4):312-317. doi:10.1007/s12105-010-0203-5 4. Eckstein J et al. A series of typical and atypical cases of Bazex syndrome: Identifying the red herring to avoid delaying cancer treatment. Clin Case Rep. 2020;8(11):2259-2264. 5. Publickal JK, Kaliyadan F. Acrokeratosis Paraneoplastica. [Updated 2020 May 24]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK459391/ DISCUSSION