10.5826/dpc.1401a35
text/xml
2160-9381
3652
en
Vincenzo Maione
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
apremilast
Successful Treatment of Recalcitrant Subcorneal Pustular Dermatosis (Sneddon-Wilkinson Diseases) With Apremilast
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2024-01-31
2023-07-01
2024-01-31
2024-02-21
e2024035
Dermatol Pract Concept
Vincenzo Maione
2024/01/31
10.5826/dpc.1401a35
e2024035
Dermatology Department, University of Brescia, ASST Spedali Civili di Brescia, Brescia, Italy
2160-9381
apremilast
en
e2024035
Schett G, Sloan VS, Stevens RM, Schafer P. Apremilast: a novel PDE4 inhibitor in the treatment of autoimmune and inflammatory diseases. Ther Adv Musculoskelet Dis. 2010;2(5):271-278. DOI:10.1177/1759720X10381432. PMID: 22870453. PMCID: PMC3383510.
Successful Treatment of Recalcitrant Subcorneal Pustular Dermatosis (Sneddon-Wilkinson Diseases) With Apremilast
https://dpcj.org/index.php/dpc/article/download/3652/2535
  Successful Treatment of Recalcitrant Subcorneal
 Pustular Dermatosis (Sneddon-Wilkinson Diseases)
                  With Apremilast
      Luca Bettolini1, Stefano Bighetti1, Paolo Incardona2, Piergiacomo Calzavara-Pinton1,
                                        Vincenzo Maione1


1 Dermatology Department, University of Brescia, ASST Spedali Civili di Brescia, Brescia, Italy
2 Departement of Pathology, ASST Spedali Civili di Brescia, University of Brescia, Brescia, Italy



Key words: Sneddon-Wilkinson disease, subcorneal pustural dermatosis, neutrophilic dermatosis, apremilast
Citation: Bettolini L, Bighetti S, Incardona P, Calzavara-Pinton P, Maione V. Successful Treatment of Recalcitrant Subcorneal Pustular
Dermatosis (Sneddon-Wilkinson Diseases) With Apremilast. Dermatol Pract Concept. 2024;14(1):e2024035. DOI: https://doi.org/10.5826/
dpc.1401a35
Accepted: July 30, 2023; Published: January 2024
Copyright: ©2024 Bettolini et al. This is an open-access article distributed under the terms of the Creative Commons Attribution-
NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use,
distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing Interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding Author: Dr Luca Bettolini, Department of Dermatology, Spedali Civili, University of Brescia, Brescia, Italy.
Tel +39 030 3995301 Fax +39 0303995015 E-mail: l.bettolini@unibs.it




Introduction                                                               in the formation of fine scale and crusted lesions in the axil-
                                                                           lary regions (Figure 1A). The histopathological examination
Subcorneal pustular dermatosis (SPD), also referred to as                  revealed the presence of subcorneal vesicles or pustules pri-
Sneddon-Wilkinson disease, is a chronic, rare condition of                 marily composed of neutrophils, occasionally accompanied
unknown origin characterized by the recurrent formation                    by eosinophils. Additionally, focal acantholysis was evident,
of sterile pustules in the superficial layers of the skin. These           along with vascular ectasia and congestion in the papillary
pustules typically appear in annular or serpiginous patterns               dermis (Figure 2, A-C). Direct immunofluorescence and fungal
and are commonly found on flexural surfaces and intertrigi-                stain were negative. No psoriasiform pattern of hyperplasia
nous areas. In this case report, we present a patient with SPD             were observed as well. He had undergone several treatments,
successfully treated with apremilast.                                      including dapsone 100 mg/daily, acitretin 25 mg/daily and
                                                                           colchicine 1 mg/daily with mild and transient improvement.

Case Presentation                                                              Considering the contraindication of immunosuppressants
                                                                           due to unfavorable prognostic factors related to the pri-
A 61-year-old man with a medical history of prostatic ade-                 mary tumor, we decided to initiate oral apremilast treatment
nocarcinoma underwent radical prostatectomy two years                      at a dosage of 30 mg twice a day. Remarkably, complete
prior, presented an 11-year history of SPD. During the phys-               ­remission of the disease was achieved within two months
ical examination, the patient exhibited the presence of ves-               (Figure 1B). No side effects or relapses occurred during six-
icles, pustules, as well as superficial erosion, which resulted            month follow-up.




Research Letter | Dermatol Pract Concept. 2024;14(1):e2024035                                                                           1
                Figure 1. Sneddon-Wilkinson disease. (A) Vesicles, pustules, fine scale and crusted lesions in the axillary
                regions. (B) Resolution of the lesions after 8 weeks therapy with apremilast.




Figure 2. (A,B) Histopathology displaying a subcorneal pustule with thin roof leaning on the malpighian layer slightly sunken and an absent
granular layer (H&E, 4x and H&E, 10x, respectively). (C) The superficial epidermis shows an acantholytic appearance below the pustule
together with a granulocytic exocytosis (H&E, 40x).




2                                                                           Research Letter | Dermatol Pract Concept. 2024;14(1):e2024035
   In the management of SPD, oral dapsone is commonly               favorable safety profile and can be used in patients with spe-
considered as the initial treatment of choice. Several thera-       cific comorbidities, such as a personal history of cancer.
peutic alternatives are available, including sulphapyridines,
nicotinamide, oral corticosteroids, retinoids, and photo-
therapy [1]. In some cases, off-label use of tumor necrosis         Conclusions
factor-alfa blocking agents, interleukin-23 inhibitor (gusel-
                                                                    In conclusion, this case report highlights the remarkable
kumab) has also been reported as a potential treatment op-
                                                                    therapeutic efficacy of apremilast in the management of SPD,
tion [2]. Furthermore, a case demonstrating improvement of
                                                                    particularly in cases where conventional and off-label treat-
recalcitrant SPD with the use of apremilast has been docu-
                                                                    ments have shown limited response or are contraindicated.
mented [3].
   Apremilast is an oral, small molecule inhibitor of phos-
phodiesterase 4 (PDE4) that has gained approval for treat-          References
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Research Letter | Dermatol Pract Concept. 2024;14(1):e2024035                                                                          3
