CVID-associated Granulomatous Dermatosis Resembling Sarcoidosis
Common variable immunodeficiency (CVID) is a disorder of the adaptive immune system predominantly characterized by hypogammaglobulinemia and variable T-cell abnormalities. Patients classically present with recurrent sinopulmonary infections, but interstitial lung disease, autoimmunity, and lymphoproliferative disorders are also common. Approximately 10% of CVID patients are reported to have noncaseating granulomatous disease that is indistinguishable from sarcoidosis on pathology; it most commonly affects the lungs, lymph nodes, and liver.
We present the case of a 75-year-old male with known CVID who presented with granulomatous dermatosis on the trunk and extremities in the setting of progressive cognitive impairment, new-onset cough with ground glass opacities on chest computed tomography in the setting of stable mediastinal lymphadenopathy, and the presence of granulomas on prior bone marrow biopsy, initially suggesting the diagnosis of sarcoidosis. Though clinically and histologically similar, it is important to make the distinction between CVID-associated granulomatous disease and sarcoidosis in order to select appropriate treatment.
Ardeniz O, Cunningham-rundles C. Granulomatous disease in common variable immunodeficiency. Clin Immunol. 2009;133(2):198-207.
Abdelhakim S, Cafone J, Basak RB. Cutaneous manifestations of primary immunodeficiency. Indian J Paediatr Dermatol 2017;18:155-9
Salzer U, Warnatz K, Peter HH. Common variable immunodeficiency: an update. Arthritis Res Ther. 2012;14(5):223.
Awada H, Abi-Karam G, Fayad F. Musculoskeletal and other extrapulmonary disorders in sarcoidosis. Best Pract Res Clin Rheumatol. 2003;17:971–987
Yanardag H, Tetikkurt C, Bilir M, Demirci S, Iscimen A. Diagnosis of cutaneous sarcoidosis; clinical and the prognostic significance of skin lesions. Multidiscip Respir Med. 2013;8(1):26.
Sargın G, Yavaşoğlu I, Kadıköylü G, Bolaman Z. Bone and bone marrow involvement in sarcoidosis. Turk J Haematol. 2014;31(2):192-3.
Zhou Y, Lower EE, Li H, Farhey Y, Baughman RP. Clinical characteristics of patients with bone sarcoidosis. Semin Arthritis Rheum. 2017;47(1):143-148.
Malhotra K, Ramanathan S, Agrawal D, Rana S, Scott T. Neurology Apr 2014, 82 (10 Supplement) P5.171
Arnold DF, Wiggins J, Cunningham-rundles C, Misbah SA, Chapel HM. Granulomatous disease: distinguishing primary antibody disease from sarcoidosis. Clin Immunol. 2008;128(1):18-22.
Roelandt PR, Blockmans D. Common variable immunodeficiency (CVID): case report and review of the literature. Acta Clin Belg. 2009; 64:355-60
Quinti I, Soresina A, Spadaro G, et al. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol. 2007;27(3):308-16.
Smith KJ, Skelton H: Common variable immunodeficiency treated with a recombinant human IgG, tumour necrosis factor-alpha receptor.
Abstract - 176 Full Article PDF - 57
- There are currently no refbacks.