Ann Dermatol.  2019 Aug;31(4):446-449. 10.5021/ad.2019.31.4.446.

Treatment of Hidradenitis Suppurativa Patient with Klinefelter Syndrome by Adalimumab

Affiliations
  • 1Department of Dermatology, Soon Chun Hyang University Bucheon Hospital, Bucheon, Korea.
  • 2Department of Dermatology, Soon Chun Hyang University Cheonan Hospital, Cheonan, Korea. freesia0210@naver.com

Abstract

Hidradenitis suppurativa (HS) is a chronic, inflammatory and painful skin disease with recurrent nodules and tracts involving the intertriginous regions. It is known that the patient with HS shows an increased risk of metabolic disorders such as diabetes, metabolic syndrome and autoimmune diseases. Klinefelter syndrome (KS) is a sex chromosomal disorder occurring in males due to an abnormality of sexual differentiation, characterized by 47, XXY karyotype. Also, KS is related with somatic comorbidities such as metabolic syndrome, autoimmune and rheumatologic disorders as HS is. We report a HS patient with KS who shows a big improvement while on tumor necrosis factor-alpha inhibitor treatment.

Keyword

Hidradenitis suppurativa; Klinefelter syndrome

MeSH Terms

Adalimumab*
Autoimmune Diseases
Chromosome Disorders
Comorbidity
Hidradenitis Suppurativa*
Hidradenitis*
Humans
Karyotype
Klinefelter Syndrome*
Male
Sex Differentiation
Skin Diseases
Tumor Necrosis Factor-alpha
Adalimumab
Tumor Necrosis Factor-alpha

Figure

  • Fig. 1 (A) Deep-seated, inflamed and painful nodules with odorous discharge from perianal lesions and scar. (B) Postoperative scar on the axilla after surgical resection.

  • Fig. 2 Chromosome analysis of the patient demonstrating the karyotype of KS, 47XXY.

  • Fig. 3 (A, B) Histological examination revealed extensive fibrosis and inflammatory cell infiltration in the dermis to subcutis (H&E: A, ×100; B, ×400).

  • Fig. 4 Improved and decreased the painful and purulent nodules around the perianal lesions after 2nd adalimumab injection.


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