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Our findings indicated that SBP and severity of BP profiles were significantly and independently associated with DCAN, respectively. These suggested BP profile may influence the progression of DCAN and also provided insight into biological functions. ClinicalTrials.gov Identifier NCT02461472.

References 1 Berron-Ruiz A, Berron-Perez R, Ruiz-Maldonado R. Cutaneous markers of primary immunodeficiency diseases in children. Pediatr Dermatol 2000; 17: 91-96. 2 Van Dongen JJ, Langerak AW, Bruggemann M et al. Design and standardization of PCR primers and protocols for detection of clonal immunoglobulin and T-cell receptor gene recombinations in suspect lymphoproliferations: report of the BIOMED-2 Concerted Action BMH4-CT98-3936. Leukemia 2003; 17: 2257-2317. 3 Bentur L, Shear N, Roifman CM. Cutaneous lymphohistiocytic infiltrates in patients with hypogammaglobulinemia. J Pediatr 1990; 116: 68-72. 4 Fleming MG, Gewurz AT, Pearson RW. Caseating cutaneous granulomas in a patient with X-linked infantile hypogammaglobulinemia. J Am Acad Dermatol 1991; 24: 629-633. 5 Park JY, Kim YS, Shin DH et al. Primary cutaneous peripheral T-cell lymphoma in a patient with X-linked agammaglobulinaemia. Br J Dermatol 2011; 164: 677-679. 6 Petrella T, Maubec E, Cornillet-Lefebvre P et al. Indolent CD8-positive lymphoid proliferation of the ear: a distinct primary cutaneous T-cell lymphoma? Am JEADV 2016, 30, 446-556 464 Letters to the EditorThis report provides additional evidence that LMNA mutations are associated with HGPS and a broad spectrum of associated clinical features. This finding will contribute to the understanding of the pathogenesis of HGPS and allow relevant clinical phenotypic traits to be predicted and treated.

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