Literature DB >> 33614714

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

Vincenzo Bettoli1, Riccardo Forconi1, Ilaria Pezzini2, Ruby Martinello3, Valeria Scuderi1, Piera Zedde1, Natale Schettini1, Lucrezia Pacetti1, Monica Corazza1.   

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.
Copyright © 2020 by S. Karger AG, Basel.

Entities:  

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

Year:  2020        PMID: 33614714      PMCID: PMC7879297          DOI: 10.1159/000509042

Source DB:  PubMed          Journal:  Skin Appendage Disord        ISSN: 2296-9160


  11 in total

1.  Allele-Specific Small Interfering RNA Corrects Aberrant Cellular Phenotype in Keratitis-Ichthyosis-Deafness Syndrome Keratinocytes.

Authors:  Ming Yang Lee; Hong-Zhan Wang; Thomas W White; Tony Brooks; Alan Pittman; Heerni Halai; Anastasia Petrova; Diane Xu; Stephen L Hart; Veronica A Kinsler; Wei-Li Di
Journal:  J Invest Dermatol       Date:  2019-11-06       Impact factor: 8.551

2.  Successful treatment with alitretinoin of dissecting cellulitis of the scalp in keratitis-ichthyosis-deafness syndrome.

Authors:  Sumangali Chandra Prasad; Anette Bygum
Journal:  Acta Derm Venereol       Date:  2013-07-06       Impact factor: 4.437

3.  Successful treatment of pityriasis lichenoides chronica with narrow-band ultraviolet B therapy in a patient with Keratitis-Ichthyosis-Deafness syndrome: a case report.

Authors:  Andac Salman; Dilek Seckin Gencosmanoglu; Ayse Deniz Yucelten; Nursel Elcioglu; Gabriele Richard; Cuyan Demirkesen
Journal:  Dermatol Online J       Date:  2016-05-15

Review 4.  Recognizing syndromic hidradenitis suppurativa: a review of the literature.

Authors:  J Gasparic; P Theut Riis; G B Jemec
Journal:  J Eur Acad Dermatol Venereol       Date:  2017-09-07       Impact factor: 6.166

5.  Keratitis-ichthyosis-deafness syndrome in association with follicular occlusion triad.

Authors:  Laura Maintz; Regina C Betz; Jean-Pierre Allam; Jörg Wenzel; Axel Jaksche; Nicolaus Friedrichs; Thomas Bieber; Natalija Novak
Journal:  Eur J Dermatol       Date:  2005 Sep-Oct       Impact factor: 3.328

Review 6.  Keratitis-ichthyosis-deafness syndrome: disease expression and spectrum of connexin 26 (GJB2) mutations in 14 patients.

Authors:  J Mazereeuw-Hautier; E Bitoun; J Chevrant-Breton; S Y K Man; C Bodemer; C Prins; C Antille; J-H Saurat; D Atherton; J I Harper; D P Kelsell; A Hovnanian
Journal:  Br J Dermatol       Date:  2007-03-23       Impact factor: 9.302

7.  Malignant proliferating pilar tumors arising in KID syndrome: a report of two patients.

Authors:  Gurston G Nyquist; Christina Mumm; Renee Grau; A Neil Crowson; Daniel L Shurman; Paul Benedetto; Pamela Allen; Kelli Lovelace; David W Smith; Ilona Frieden; C Patrick Hybarger; Gabriele Richard
Journal:  Am J Med Genet A       Date:  2007-04-01       Impact factor: 2.802

8.  A novel connexin 26 gene mutation associated with features of the keratitis-ichthyosis-deafness syndrome and the follicular occlusion triad.

Authors:  Jay R Montgomery; Thomas W White; Bryan L Martin; Maria L Turner; Steven M Holland
Journal:  J Am Acad Dermatol       Date:  2004-09       Impact factor: 11.527

9.  More than keratitis, ichthyosis, and deafness: Multisystem effects of lethal GJB2 mutations.

Authors:  Evelyn Lilly; Christopher G Bunick; Alexander M Maley; Shali Zhang; Mary K Spraker; Amy J Theos; Karina L Vivar; Lucia Seminario-Vidal; Adam E Bennett; Robert Sidbury; Yasushi Ogawa; Masashi Akiyama; Barbara Binder; Smail Hadj-Rabia; Raffaella A Morotti; Earl J Glusac; Keith A Choate; Gabriele Richard; Leonard M Milstone
Journal:  J Am Acad Dermatol       Date:  2018-10-02       Impact factor: 11.527

10.  Keratitis-Ichthyosis-Deafness Syndrome, Atypical Connexin GJB2 Gene Mutation, and Peripheral T-Cell Lymphoma: More Than a Random Association?

Authors:  Claudio Fozza; Fausto Poddie; Salvatore Contini; Antonio Galleu; Francesca Cottoni; Maurizio Longinotti; Francesco Cucca
Journal:  Case Rep Hematol       Date:  2011-08-10
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