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Inherited Immunodeficiency Inherited Immunodeficiency: Brigitte Bader-Meunier, MD, a, b Frédéric Rieux-Laucat, PhD, b Fabien Touzot, MD, PhD, c Marie-Louise Frémond, MD, a, b AIsabelle New André-Schmutz, Association PhD, b Sylvie Fraitag, MD, d Christine With Bodemer, MD, PhDEarly-b, e, f Onset Childhood Panniculitis abstract We report on 4 children who presented with asepticTRNT1 panniculitisNF- κb2 associated with inherited immunodeficiency.‍ Three patients had a B-cell immunodeficiency resulting from mutations in the and genes aDépartements d'Immunologie, Hématologie, et LCK Rhumatologie Pédiatrique, dd'Anatomie Pathologique, (no mutation was found in the third patient), and 1 had a T-cell deficiency and ede Dermatologie Pédiatrique, Hôpital Necker-Enfants (mutation in the gene).‍ Panniculitis occurred before the age of 2 years Malades, Assistance Publique-Hôpitaux de Paris, Paris, b f in the 4 patients and preceded the onset of recurrent infections because France; Institut Imagine, INSERM U1163; Université Paris Descartes, Sorbonne Cité, Paris, France; and cDépartement of immunodeficiency in 2.‍ It presented either as nodules, which resolved d’Immunologie et Rhumatologie Pédiatrique, Centre spontaneously within 1 to 2 weeks (3 patients), or chronic ulcerative lesions Hospitalier Universitaire Sainte-Justine, Université de Montréal, Quebec, Canada (1 patient) associated with unexplained fever and elevated acute phase Drs Bodemer, Fraitag, and Bader-Meunier reactants, without evidence of infection or high-titer autoantibodies.‍ Febrile conceptualized and designed the study, supervised nodules relapsed in 2 patients, and recurrent attacks of unexplained fever the data collection, and drafted the initial (without relapse of panniculitis) occurred in the third.‍ Skin biopsy revealed manuscript; Drs Touzot and Frémond conducted predominantly lympho-histiocytic or septal neutrophilic panniculitis in the initial analyses and reviewed and revised the manuscript; Dr Fraitag was involved in the 1 and 3 patients, respectively.‍ Panniculitis was associated with dermal pathologic analysis; Drs André-Schmutz and involvement in the 4 patients.‍ Patients with B-cell deficiency received Rieux-Laucat were involved in the genetic studies; monthly intravenous immunoglobulin replacement.‍ Two patients who and all authors approved the final manuscript as submitted. underwent bone marrow transplant died of bone marrow transplant- DOI: https:// doi. org/ 10. 1542/ peds. 2017- 0213 related complications.‍ The 2 remaining patients had persistent, mild autoinflammatory disease, which did not require specific treatment.‍ In Accepted for publication Aug 28, 2017 these cases, the need for careful immunologic evaluation of patients who Address correspondence to Brigitte Bader-Meunier, present with unexplained panniculitis, especially early-onset panniculitis MD, Hémato-Immunologie Pédiatrique, Hôpital Necker APHP, 149 rue de Sèvres, 75015 Paris, before the age of 2 years, is highlighted.‍ France. E-mail: [email protected] PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Pediatric panniculitis occurs in a group disorders.‍ Some types of panniculitis Copyright © 2018 by the American Academy of Pediatrics of rare disorders characterized by 1, 2 are specific to children, including inflammation of the subcutaneous fat.‍ those only seen in neonates such FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships Histopathological analysis is required as subcutaneous fat necrosis of relevant to this article to disclose. to confirm the diagnosis because the newborn, or occur in a specific FUNDING: No external funding. the lesions may present differently, context such as postcorticosteroid1, 2 often consisting of erythematous panniculitis and cold panniculitis.‍ POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of nodules or large infiltrated plaques.‍ Other recognized etiologies described interest to disclose. Panniculitis cases are classified as in adults may be observed in children mostly lobular, mostly septal, or but are less frequent or rare in this – mixed, depending on the distribution population, including: infectious, To cite: Bader-Meunier B, Rieux-Laucat F, Touzot F, – et al. Inherited Immunodeficiency: A New Association of the dominant subcutaneous3 connective tissue disease related, With Early-Onset Childhood Panniculitis. Pediatrics. inflammatory infiltrates.‍ Panniculitis subcutaneous lymphoma like, 2018;141(s5):e20170213 can be a primary disease but is metabolic (alpha1-antitrypsin most often a secondary process deficiency, pancreatitis), and drug- related to many different underlying or trauma-induced panniculitis.‍ In Downloaded from www.aappublications.org/news by guest on September 23, 2021 CASE REPORT PEDIATRICS Volume 141, number s5, April 2018:e20170213 Patient 1 + many pediatric patients, no classic, low levels of CD27 B cells and specific cause can be identified.‍ Such switch memory B cells.‍ IVIg was A 2-month-old girl was referred cases are designated as idiopathic started (400 mg/kg per day).‍ He because of fever and an erythematous panniculitis.‍ Here, we describe the developed further chronic fever, nodule on 1 toe lasting 2 weeks (Fig 1).‍ cases of 4 patients who presented diarrhea, arthritis, failure to thrive, C-reactive protein (CRP) was with early-onset panniculitis and multiple ulcerative plaques (Fig 1) 42 mg/L.‍ A skin biopsy revealed associated with inherited B- or T-cell that did not undergo biopsy.‍ At predominantly septal lympho- immunodeficiency.‍ We propose that onset, he responded to treatment histiocytic panniculitis (Fig 1).‍ The inherited immunodeficiency is a new with high doses of prednisone (2 symptoms resolved spontaneously β cause of panniculitis, underlying mg/kg per day) and treatment with within 4 weeks.‍ During the course of – the need for careful immunologic interleukin-1 inhibitors (anakinra, the disease, the patient presented with evaluation for cases of unexplained canakinumab), anti tumor necrosis relapsing noninfectious panniculitis pediatric panniculitis, particularly factor inhibitors (infliximab), and coinciding with febrile attacks with onset before the age of 2 years.‍ cyclophosphamide were not steroid- without any other manifestations sparing.‍ He underwent a bone marrow CASE REPORTS and elevated acute-phase reactants transplant but died of treatment- without any other manifestations.‍ related complications within the first Two to 4 episodes per year occurred NF-κB2 days of the procedure.‍ WES identified We identified 4 patients and resolved spontaneously within 1 who displayed inherited a mutation in the gene.‍ to 2 weeks.‍ Other findings included Patient 3 immunodeficiency associated with recurrent otitis and ataxia, and we panniculitis among 18 cases of performed immunologic tests that pediatric idiopathic panniculitis revealed hypogammaglobulinemia+ (onset before the age of 16 years) and CD27 memory B-cell deficiency A 12-month-old girl was referred recorded in the database of the (Table 1).‍ Replacement intravenous because of recurrent attacks of Department of Dermatological immunoglobulin (IVIg) therapy was fever from the age of 2 months, Pathology of the Necker-Enfants started (400 mg/kg per day).‍ At 8 with or without documented Malades hospital from January 2004 years of age, she was normal-sized infections, and failure to thrive.‍ to December 2014.‍ Specific infectious for her age, and no more infections CRP levels were elevated during causes for the panniculitis lesions – had occurred.‍ Relapsing panniculitis each episode.‍ Infections consisted were excluded by specific stains and a mild ataxia persisted.‍ WES of recurrent septicemias.‍ Aseptic (periodic acid Schiff, Grocott, Ziehl, was performed but did not allow febrile manifestations were vulvitis, and Gram) in cases of neutrophilic identification of the underlying parotitis, adenitis, and the occurrence and/or macrophagic infiltrates, Patientgenetic defect2 .‍ of 1 noninfectious erythematous and Epstein-Barr encoding region nodule of the limb (at 10 months).‍ staining was used for those A skin biopsy of the nodule revealed composed of lymphocytes.‍ Blood A 20-month-old boy was referred lobular and septal neutrophilic cultures and serological tests because of undocumented lower panniculitis.‍ Other features included for Epstein-Barr virus, human Salmonella respiratory tract infections, chronic microcytic anemia (from the herpesvirus 6, and cytomegalovirus infections, and failure to age of 2 months), low levels of serum were performed if an infection was thrive.‍ From the age of 10 months, IgG, IgA, and IgM, and low levels of suspected.‍ Specific gene sequencing + he had a history of noninfectious, CD27 B cells and memory B cells.‍ or whole exome sequencing (WES) relapsing episodes of fever associated IVIg was started (400 mg/kg per was performed on DNA of the 4 ’ with multiple erythematous nodules day).‍ At 6 years of age, her height patients after obtaining informed (hands, feet, bottom) (Fig 1) and was normal, and no infections had consent from the patients parents.‍ undocumented diarrhea.‍ CRP levels occurred.‍ She continued to have 3 The 4 patients were referred to the rose substantially during these to 4 febrile attacks (lasting for 1 to Department of Pediatric Immunology attacks (Table 1).‍ A skin biopsy of 1 2 days) each month, associated with and Rheumatology of the Necker- nodule revealed septal and lobular elevated CRP levels.‍ There were no Enfants
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