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Clinical reasoning: Case report

Vol. 156 No. 1 (2026)

Pigmented purpuric dermatosis in two patients with common variable immunodeficiency

Cite this as:
Swiss Med Wkly. 2026;156:3993
Published
28.01.2026

Summary

Pigmented purpuric dermatosis is a benign type of chronic angiodermatitis prevalent in the general population, presenting with a relapsing-remitting course, with predominantly idiopathic aetiology and both humoral and cell-mediated immune pathogenesis.

We report to our knowledge the first two cases of clinical association between such dermal capillaritis and the most clinically heterogeneous form of primary immunodeficiency: common variable immunodeficiency. Furthermore, in the light of already suggested triggers and pathophysiological mechanisms correlating pigmented purpuric dermatosis with various forms of secondary immunodeficiency, we complete our case descriptions by the proposal of a new putative aetiopathogenic actor that would likely account for the occurrence of the clinical association described.

References

  1. 1. Tolaymat L, Hall MR. Pigmented Purpuric Dermatosis. 2023 Apr 17. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan.
  2. 2. Doucas C, Kapetanakis J. Eczematid-like purpura. Dermatologica. 1953;106(2):86–95. doi: https://doi.org/10.1159/000256830
  3. 3. Martínez Pallás I, Conejero Del Mazo R, Lezcano Biosca V. Pigmented Purpuric Dermatosis: A Review of the Literature. Actas Dermosifiliogr (Engl Ed). 2020 Apr;111(3):196–204. doi: https://doi.org/10.1016/j.ad.2019.02.013
  4. 4. Spigariolo CB, Giacalone S, Nazzaro G. Pigmented Purpuric Dermatoses: A Complete Narrative Review. J Clin Med. 2021 May;10(11):2283. doi: https://doi.org/10.3390/jcm10112283
  5. 5. Ilagan FM, Wu YH. A retrospective study on the direct immunofluorescence findings in pigmented purpuric dermatosis. J Cutan Pathol. 2024 Jan;51(1):63–9. doi: https://doi.org/10.1111/cup.14507
  6. 6. Gangemi S, Allegra A, Musolino C. Lymphoproliferative disease and cancer among patients with common variable immunodeficiency. Leuk Res. 2015 Apr;39(4):389–96. doi: https://doi.org/10.1016/j.leukres.2015.02.002
  7. 7. Amato G, Vita F, Quattrocchi P, Minciullo PL, Pioggia G, Gangemi S. Involvement of miR-142 and miR-155 in Non-Infectious Complications of CVID. Molecules. 2020 Oct;25(20):4760. doi: https://doi.org/10.3390/molecules25204760
  8. 8. Naik S. One Size Does Not Fit All: Diversifying Immune Function in the Skin. J Immunol. 2022 Jan;208(2):227–34. doi: https://doi.org/10.4049/jimmunol.2100758
  9. 9. Remiker A, Bolling K, Verbsky J. Common Variable Immunodeficiency. Med Clin North Am. 2024 Jan;108(1):107–21. doi: https://doi.org/10.1016/j.mcna.2023.06.012
  10. 10. Richards S, Aziz N, Bale S, Bick D, Das S, Gastier-Foster J, et al.; ACMG Laboratory Quality Assurance Committee. Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology. Genet Med. 2015 May;17(5):405–24. doi: https://doi.org/10.1038/gim.2015.30
  11. 11. Warnatz K, Denz A, Dräger R, Braun M, Groth C, Wolff-Vorbeck G, et al. Severe deficiency of switched memory B cells (CD27(+)IgM(-)IgD(-)) in subgroups of patients with common variable immunodeficiency: a new approach to classify a heterogeneous disease. Blood. 2002 Mar;99(5):1544–51. doi: https://doi.org/10.1182/blood.V99.5.1544
  12. 12. Wehr C, Kivioja T, Schmitt C, Ferry B, Witte T, Eren E, et al. The EUROclass trial: defining subgroups in common variable immunodeficiency. Blood. 2008 Jan;111(1):77–85. doi: https://doi.org/10.1182/blood-2007-06-091744
  13. 13. Naranjo CA, Busto U, Sellers EM, Sandor P, Ruiz I, Roberts EA, et al. A method for estimating the probability of adverse drug reactions. Clin Pharmacol Ther. 1981 Aug;30(2):239–45. doi: https://doi.org/10.1038/clpt.1981.154
  14. 14. Pantanowitz L, Dezube BJ, Pinkus GS, Tahan SR. Histological characterization of regression in acquired immunodeficiency syndrome-related Kaposi’s sarcoma. J Cutan Pathol. 2004 Jan;31(1):26–34. doi: https://doi.org/10.1046/j.0303-6987.2004.0132.x
  15. 15. Garrido PM, Espinosa-Lara P, Aguado-Lobo M, Soares-Almeida L, Borges-Costa J. Dermoscopy of Pigmented Purpuric Lichenoid Dermatitis of Gougerot and Blum in an HIV-infected Patient. Dermatol Pract Concept. 2020 Oct;10(4):e2020075. doi: https://doi.org/10.5826/dpc.1004a75
  16. 16. Bonnet U, Selle C, Isbruch K, Isbruch K. Recurrent purpura due to alcohol-related Schamberg’s disease and its association with serum immunoglobulins: a longitudinal observation of a heavy drinker. J Med Case Rep. 2016 Oct;10(1):301. doi: https://doi.org/10.1186/s13256-016-1065-6