Neutrophilic urticarial dermatosis of the extremities: Report of one case

Sara Kerroum1, Mariame Meziane1, Nadia Ismaili1, Laila Benzekri1, Kaoutar Znati2, Karima Senouci1

1Department of Dermatology, Mohammed V University in Rabat, Ibn Sina University Hospital, Rabat, Morocco, 2Department of Histopathology, Mohammed V University in Rabat, Ibn Sina University Hospital, Rabat, Morocco

Corresponding author: Sara Kerroum, MD, E-mail: kerroums1992@gmail.com

How to cite this article: Kerroum S, Meziane M, Ismaili N, Benzekri L, Znati K, Senouci K. Neutrophilic urticarial dermatosis of the extremities: Report of one case. Our Dermatol Online. 2022;13(4):461-462.
Submission: 06.11.2021; Acceptance: 15.02.2022
DOI: 10.7241/ourd.20224.28

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Sir,

Neutrophilic urticarial dermatosis (NUD) is a recently described entity part of the spectrum of neutrophilic dermatoses (ND). It is often associated with inflammatory diseases or neoplasms, such as hemopathies.

Herein, we report the case of a 67-year-old patient with a history of pulmonary tuberculosis, treated and declared cured, chronic obstructive pulmonary disease, operated prostatic adenoma, smoking at a rate of 50 pack-years, weaned four months ago, alcoholism, and cannabis use, weaned sixteen years ago. The patient presented with non-itchy, slightly infiltrated, maculopapular, erythematous-to-purplish plaques, which were present only in the extremities of the upper and lower limbs (Figs. 1a – 1c). The rash had persisted for almost ten years in relapses or remissions and progressed against a background of fever, polyarthralgia, and asthenia.

Figure 1a: Erythemato-purplish, maculo-papular plaques involving the back of hands.
Figure 1b: Erythemato-purplish, maculo-papular plaques involving the palms of the hands.
Figure 1c: Lesions on the feet.

There was a biological inflammatory syndrome, with increased ESR and CRP, ferritinemia, and an inflammatory profile on protein electrophoresis.

Histology revealed a perivascular neutrophilic infiltrate without associated vasculitis (Fig. 2). Thus, the diagnosis of urticarial neutrophilic dermatosis was established. An entire paraneoplastic assessment was requested and returned unremarkable. The patient was treated with indomethacin with a regression of the lesions.

Figure 2: Histological aspect of neutrophilic urticarial dermatosis.

Neutrophilic urticarial dermatosis (NUD) is a rare variant, falling within the spectrum of neutrophilic dermatoses.

The concept of NUD was recently proposed by D. Lipsker et al. [1] to designate a rash distinct from common urticaria or urticarial vasculitis by its slightly raised and non-itchy character as well as by a pink-red color. All these clinical characteristics were found in our patient. The localization of lesions reduced to the extremities of the upper and lower limbs was a peculiarity of our present case.

NUD is often associated with inflammatory diseases, such as systemic lupus, Still’s disease, and juvenile idiopathic arthritis, and neoplastic diseases, such as hemopathies (myeloids, lymphoids) and solid tumors (digestive, pulmonary, testicular, mammary) [2]. These diseases were absent in our case.

Histologically, NUD is characterized by a perivascular and interstitial neutrophilic infiltrate, known as the „single file” or „en file indienne” [2], without vasculitis or dermal edema; all of these histological elements consolidated the diagnosis of this entity in our patient.

The treatment adopted for this case based on indomethacin allowed the lesions to regress rapidly. It should be noted that colchicine [3], dapsone, and general corticosteroid therapy may also be relevant therapeutic options.

Herein, we reported a case of neutrophilic urticarial dermatosis (UND) with an acral localization. It is imperative to keep in mind the need for usual supervising to search for an associated neoplasia or connectivitis.

Consent

The examination of the patient was conducted according to the principles of the Declaration of Helsinki.

The authors certify that they have obtained all appropriate patient consent forms, in which the patients gave their consent for images and other clinical information to be included in the journal. The patients understand that their names and initials will not be published and due effort will be made to conceal their identity, but that anonymity cannot be guaranteed.

REFERENCES

1. Kieffer C, Cribier B, Lipsker D. Neutrophilic urticarial dermatosis:A variant of neutrophilic urticaria strongly associated with systemic disease. Report of 9 new cases and review of the literature. Medicine (Baltimore). 2009;88:23-31.

2. Ben Hamouda M, Soua Y, Njim L, Belhadjali H, Youssef M, Zili J. Dermatite urticarienne neutrophilique: propos d’un cas. Service de dermatologie et d’anatomopathologie, hôpital Fattouma Bourguiba, Monastir, Tunisie. 2018.

3. Ben Rajeb M, Korbi M, Ben Hamouda M, Ben Abdeljelil N, Soua Y, Njim L, et al. Deux cas de dermatose neutrophilique urticarienne. Ann Dermatol Vénérol. 2019;146:A223-4.

Notes

Source of Support: Nil,

Conflict of Interest: The authors have no conflict of interest to declare.

 

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