Erosive pustular dermatosis of the scalp and Kindler syndrome: a new association

Erosive pustular dermatosis of the scalp and Kindler syndrome: a new association Dear Editor,Erosive pustular dermatosis of the scalp (EPDS) is a rare inflammatory disease that predominantly affects elderly people, with a chronic course and long‐term management. Sterile pustules, superficial erosions and crusted lesions on atrophic skin, resulting in cicatricial alopecia, characterize the typical clinical presentation. Triggering factors include several drugs, actinic damage, local trauma, surgery and physical or chemical procedures for actinic keratosis. To the best of our knowledge, no previous association with Kindler syndrome was found in the literature.A 71‐year‐old Caucasian man reported a two‐year history of scalp lesions, treated with topical antibiotics and corticosteroids without improvements. The patient was otherwise affected by Kindler syndrome, with a omozygous c.862C>T (p.Arg288*) mutation in FERM1 gene (Institute of Research, IRCCS‐IDI, Rome). Typical hallmarks of the disease were present including skin fragility, trauma‐induced blistering, photosensitivity, generalized poikiloderma (Fig. a), haemorrhagic mucositis, periodontal disease and ectropion of the lower eyelids (Fig. b). Pseudosyndactyly, nail dystrophy (Fig. c), palmar hyperkeratosis, dermatoglyphics loss were also prominent findings (Fig. d).Kindler syndrome signs: scattered trauma‐induced blistering covered with serohematic crusts, diffuse poikiloderma (inset a), ectropion and haemorrhagic mucositis (inset b), pseudosyndactyly with onychodystrophy (inset c) and palmar hyperkeratosis, dermatoglyphics loss (d).Physical examination of scalp revealed multiple erythematous large, erosive and crusted http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of the European Academy of Dermatology & Venereology Wiley

Erosive pustular dermatosis of the scalp and Kindler syndrome: a new association

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Publisher
Wiley Subscription Services, Inc., A Wiley Company
Copyright
Copyright © 2018 European Academy of Dermatology and Venereology
ISSN
0926-9959
eISSN
1468-3083
D.O.I.
10.1111/jdv.14577
Publisher site
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Abstract

Dear Editor,Erosive pustular dermatosis of the scalp (EPDS) is a rare inflammatory disease that predominantly affects elderly people, with a chronic course and long‐term management. Sterile pustules, superficial erosions and crusted lesions on atrophic skin, resulting in cicatricial alopecia, characterize the typical clinical presentation. Triggering factors include several drugs, actinic damage, local trauma, surgery and physical or chemical procedures for actinic keratosis. To the best of our knowledge, no previous association with Kindler syndrome was found in the literature.A 71‐year‐old Caucasian man reported a two‐year history of scalp lesions, treated with topical antibiotics and corticosteroids without improvements. The patient was otherwise affected by Kindler syndrome, with a omozygous c.862C>T (p.Arg288*) mutation in FERM1 gene (Institute of Research, IRCCS‐IDI, Rome). Typical hallmarks of the disease were present including skin fragility, trauma‐induced blistering, photosensitivity, generalized poikiloderma (Fig. a), haemorrhagic mucositis, periodontal disease and ectropion of the lower eyelids (Fig. b). Pseudosyndactyly, nail dystrophy (Fig. c), palmar hyperkeratosis, dermatoglyphics loss were also prominent findings (Fig. d).Kindler syndrome signs: scattered trauma‐induced blistering covered with serohematic crusts, diffuse poikiloderma (inset a), ectropion and haemorrhagic mucositis (inset b), pseudosyndactyly with onychodystrophy (inset c) and palmar hyperkeratosis, dermatoglyphics loss (d).Physical examination of scalp revealed multiple erythematous large, erosive and crusted

Journal

Journal of the European Academy of Dermatology & VenereologyWiley

Published: Jan 1, 2018

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