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Case Reports
. 2021 Jan;7(1):21-24.
doi: 10.1159/000509042. Epub 2020 Oct 26.

KID Syndrome and Hidradenitis Suppurativa: A Rare Association Responding to Surgical Treatment

Vincenzo Bettoli  1 , Riccardo Forconi  1 , Ilaria Pezzini  2 , Ruby Martinello  3 , Valeria Scuderi  1 , Piera Zedde  1 , Natale Schettini  1 , Lucrezia Pacetti  1 , Monica Corazza  1
Affiliations
Case Reports

KID Syndrome and Hidradenitis Suppurativa: A Rare Association Responding to Surgical Treatment

Vincenzo Bettoli et al. Skin Appendage Disord. 2021 Jan.

Abstract

Background: Keratitis-ichthyosis-deafness (KID) syndrome is a rare genodermatosis characterized by keratitis, neurosensorial auditory impairment and ichthyosiform skin involvement. Frequent complications of the syndrome are chronic, opportunistic cutaneous infections, and the development of skin cancers. Several cases of association between KID syndrome and other conditions, including hidradenitis suppurativa (HS), are described in the literature. This correlation could be explained by the hyperproliferative state of the epidermis, which occurs in KID syndrome and may favor follicular plugging.

Objectives: The aim of this study was to describe a very rare case of association between KID syndrome and HS and its complex therapeutic management.

Results: The failure of the drugs commonly used in HS and the excellent results of surgery, although difficult to achieve, were experienced.

Conclusion: Despite the technical difficulties related to surgery, namely, cutaneous superinfections, frequent dehisce of the suture, and closure by secondary intention, the authors strongly recommend the surgical approach in these patients.

Keywords: Genodermatosis; Hidradenitis suppurativa; Keratitis-ichthyosis-deafness syndrome; Rare; Surgical treatment.

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Conflict of interest statement

None of the authors have any declaration of any conflict of interest to report.

Figures

Fig. 1
Fig. 1
Presurgical HS localizations: right inguinal and crural region, right thigh root, right hemivulva, and perianal region. HS, hidradenitis suppurativa.
Fig. 2
Fig. 2
Surgical strategy: the arrows start from the skin areas used for sliding the flaps and end in the portions to be repaired after excision.
Fig. 3
Fig. 3
Postsurgical outcome.
See this image and copyright information in PMC

References

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