Vol 10, No 3 (2024)
Case report
Published online: 2024-08-14

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Undiagnosed Darier’s disease comorbid with ambiguous viral infection

Konrad Bagiński1, Małgorzata Duzinkiewicz1, Anna Baran1, Patrycja Lemiesz1, Iwona Flisiak1
Forum Dermatologicum 2024;10(3):93-96.

Abstract

Introduction: Darier’s disease (DD) is a rare autosomal dominant genodermatosis characterized by hyperkeratotic papules, primarily located on seborrheic areas as well as with palmoplantar, nail or oral mucosa involvement. The course of the disease is chronic with the possible occurrence of coinfection, most commonly with the herpes simplex virus. Each new infection can significantly intensify the symptoms.

Case description: A 42-year-old patient presented with a prolonged history of undiagnosed brownish-grey papules on the limbs and trunk, worsening in the summer was admitted to the dermatology department, to diagnose and treat the one-week history of skin lesions on his arms, chest, and neck, and partially face. Lesions were more intensified on the left side, initially displaying as intense erythema, then covered with multiple papules and grouped vesicles, accompanied by a burning sensation and fever. Laboratory tests showed elevated inflammatory markers. Ultrasonography revealed oedema of the subcutaneous tissue and enlarged cervical lymph nodes on the left side. In the blood culture methicillin-resistant Staphylococcus Aureus (MRSA) was detected. Systemic intravenous treatment with antibiotics and acyclovir resulted in slow clinical improvement with normalization in laboratory tests. Histopathological examination in correlation with the clinical data suggested a Poxviridae infection along with a typical Darier’s disease picture.

Conclusions: Darier’s disease, particularly when complicated by cutaneous viral infections, may be misleading, as seen in this case. Eczema herpeticum, eczema vaccinatum, or other pox-zoonoses may explain the severe course observed. The histopathological examination is crucial for accurate diagnosis.

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References

  1. Takagi A, Kamijo M, Ikeda S. Darier disease. J Dermatol. 2016; 43(3): 275–279.
  2. Tavadia S, Mortimer E, Munro CS. Genetic epidemiology of Darier's disease: a population study in the west of Scotland. Br J Dermatol. 2002; 146(1): 107–109.
  3. Kositkuljorn C, Suchonwanit P. Darier's disease: report of a case with facial involvement. Case Rep Dermatol. 2019; 11(3): 327–333.
  4. Rogner DF, Lammer J, Zink A, et al. Darier and Hailey-Hailey disease: update 2021. J Dtsch Dermatol Ges. 2021; 19(10): 1478–1501.
  5. Kurzeja M, Rakowska A, Jasinska M, et al. Dermoscopy as a noninvasive diagnostic tool for Hailey-Hailey disease and darier disease. Dermatol Ther (Heidelb). 2023; 13(10): 2345–2355.
  6. Yamamoto T, Aoyama Y. Role of pro-inflammatory cytokines in the pathophysiology of herpes simplex virus superinfection in Darier's disease. J Dermatol. 2021; 48(10): 1607–1611.
  7. Dykes R, Ergen E, Martin T, et al. Exacerbation of Darier's disease with COVID-19. JAAD Case Rep. 2022; 27: 64–66.
  8. Haase O, Moser A, Rose C, et al. Generalized cowpox infection in a patient with Darier disease. Br J Dermatol. 2011; 164(5): 1116–1118.
  9. Claudy AL, Gaudin OG, Granouillet R. Pox virus infection in Darier's disease. Clin Exp Dermatol. 1982; 7(3): 261–265.
  10. Nitsch A, Pauli G (2007) Infections with cowpox virus in Germany — an overview: Cats are currently important as carriers. RKI Bulletin. 9.3.2007, No.10.
  11. Dicken CH, Bauer EA, Hazen PG, et al. Isotretinoin treatment of Darier's disease. J Am Acad Dermatol. 1982; 6(4 Pt 2 Suppl): 721–726.
  12. Palacios-Álvarez I, Andrés-Ramos I, Silva MY, et al. Treatment of Darier's disease with diclofenac sodium 3% gel. Dermatol Ther. 2017; 30(3).
  13. Campos MO, Figueiredo GA, Evangelista AC, et al. Darier's disease: treatment with topical sodium diclofenac 3% gel. An Bras Dermatol. 2023; 98(6): 882–884.
  14. Sondhi M, Vashist S, Mahajan VK. Darier's disease - response to oral vitamin A: report of a case and brief review. Indian Dermatol Online J. 2020; 11(1): 72–78.
  15. Guenin S, Lebwohl M. Bexarotene use in refractory Darier's disease. Int J Dermatol. 2023; 62(11): e587–e589.