Dermatology: Practical and Conceptual


Image Letter  |  Dermatol Pract Concept 2020;10(1):e2020008 1

Dermatology Practical & Conceptual

Case Presentation

A 54-year-old-man diagnosed with a deficiency of interleu-

kin-36 receptor antagonist (DITRA) under anti-interleukin-1 

therapy with anakinra (described elsewhere [1]) presented 

with an erythrodermic pustular psoriasis 5 months after 

suspension of his biological treatment (Figure 1A). Anakinra 

and topical corticoids were then initiated. Three days later, 

a halo of nonreddened skin circumscribing an erythematous 

pustular plaque was observed on his right side (Figure 1B). 

The patient showed a complete clinical response in the fol-

lowing weeks.

Teaching Point

DITRA is an autoinflammatory disorder caused by muta-

tions in the ILRN36 gene, characterized by abrupt-onset 

episodes of generalized pustular psoriasis, fever, and systemic 

Woronoff Ring in Deficiency of Interleukin-36 
Receptor Antagonist (DITRA)

Daniel Morgado-Carrasco,1 Sebastian Podlipnik,1 José M. Mascaró, Jr1

1 Department of Dermatology, Hospital Clínic de Barcelona, Universitat de Barcelona, Spain

Keywords: Woronoff ring, psoriasis, DITRA, deficiency of interleukin-36 receptor antagonist, anakinra

Citation: Morgado-Carrasco D, Podlipnik S, Mascaró JM Jr. Woronoff ring in deficiency of interleukin-36 receptor antagonist (DITRA). 
Dermatol Pract Concept. 2020;10(1):e2020008. DOI: https://doi.org/10.5826/dpc.1001a08

Accepted: September 5, 2020; Published: December 31, 2019

Copyright: ©2019 Morgado-Carrasco et al. This is an open-access article distributed under the terms of the Creative Commons Attribution 
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are 
credited.

Funding: None.

Competing interests: The authors have no conflicts of interest to disclose.

Authorship: All authors have contributed significantly to this publication.

Corresponding author: José M. Mascaró, Jr, MD, Department of Dermatology, Hospital Clínic de Barcelona, Barcelona, Spain. Email: 
josemanuel.mascaro@gmail.com

Figure 1. Woronoff ring in de-

ficiency of interleukin-36 re-

ceptor antagonist (DITRA). (A) 

Widespread erythematous and 

pustular rash on the trunk. (B) 

Halo of nonreddened skin cir-

cumscribing an erythematous 

pustular plaque on the right side 

(3 days after initiating anakinra 

and topical corticosteroids).

mailto:josemanuel.mascaro@gmail.com


2 Image Letter  |  Dermatol Pract Concept 2020;10(1):e2020008

References

1. Podlipnik S, Morgado-Carrasco D, Fustà-Novell X, et al. Dynamics 

of plasma cytokines in a patient with deficiency of interleukin-36 

receptor antagonist successfully treated with anakinra. Br J Der-

matol. 2018;178(4):e258-e260.

2. Park KK, Swan JW, Eilers D, Tung R, Koo J. Woronoff ring associat-

ed with adalimumab therapy for psoriasis. Cutis. 2014;93(2):e1-e2.

involvement. The Woronoff ring is an underrecognized sign 

of psoriasis, a halo of nonerythematous skin surrounding 

psoriatic plaques. Its pathogenesis is not fully understood; a 

relative deficiency of prostaglandins and endoglin has been 

reported. The Woronoff ring can occur after topical corticoid 

or tar treatment, fumaric acid esters, phototherapy, biological 

therapy [2], or even in untreated psoriasis.