Reumatología Clínica (English Edition) Reumatología Clínica (English Edition)
Reumatol Clin 2018;14:53-5 - Vol. 14 Num.1 DOI: 10.1016/j.reumae.2016.09.003
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Purpuric Component Features that Differentiate Urticarial Vasculitis and Urticaria Without Vasculitis
Características del componente purpúrico de la urticaria con vasculitis y de la urticaria sin vasculitis
Belén Lozano Masdemonta,, , Celia Horcajada Realesa, Laura Gómez-Recuero Muñoza, Verónica Parra Blancob
a Servicio de Dermatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain
b Servicio de Anatomía Patológica, Hospital General Universitario Gregorio Marañón, Madrid, Spain
Received 08 May 2016, Accepted 22 September 2016
Case Reports

We studied a series of 8 patients (5 men and 3 women) who presented with urticaria-like wheals and purpura that had developed more than 24h earlier.

Diagnosis and Disease Course

Four patients were diagnosed with urticarial vasculitis (UV), 2 of them with hypocomplementemia, and another 4 patients with urticaria without vasculitis. None of them had extracutaneous involvement. Their protein profiles and immunoglobulin levels were normal and serological tests for human immunodeficiency virus and hepatitis B and C viruses were negative (other parameters are shown in Table 1).

Table 1.

Summary of Clinical and Analytical Data.

  Case 1  Case 2  Case 3  Case 4  Case 5  Case 6  Case 7  Case 8 
  (Fig. 1a)  (Fig. 1b)  (Fig. 1c)  (Fig. 1d)  (Fig. 2a)  (Fig. 2b)  (Fig. 2c)  (Fig. 2d) 
Sex/age  M/37 years  F/81 years  F/68 years  F/60 years  M/51 years  M/42 years  M/60 years  M/50 years 
Comorbidities  —  Nonallergic bronchial asthma  —  Invasive ductal breast adenocarcinoma  —  —  —  — 
Site  Thighs and abdomen  Chest, abdomen and LL  Abdomen  Chest, arms and thighs  Arms, thighs and abdomen  UL, trunk, LL  UL, trunk, LL  UL, trunk, LL 
Time since onset  7 days  3 days  12 days  3 days  12 days  3 days  5 days  7 days 
Local symptoms  Pruritus  Pruritus  Pain  Burning sensation  Pruritus  Pruritus  Pruritus  Pruritus 
  Dolor  —  —  Pain  —  —  —  — 
General symptoms  No  Fever  No  No  No  No  No  No 
Histology  Deep and superficial perivascular neutrophilic inflammatory infiltrate. Moderate number of eosinophils. Focal fibrinoid necrosis.  Fibrinoid necrosis, dermal edema, blood extravasation.  Fibrinoid necrosis, dermal edema, blood extravasation.  Superficial perivascular neutrophilic inflammatory infiltrate. Moderate number of eosinophils. Focal fibrinoid necrosis. Nuclear fragments.  Superficial perivascular lymphocytic inflammatory infiltrate with eosinophils, blood extravasation.  Superficial perivascular lymphocytic inflammatory infiltrate, blood extravasation.  Superficial perivascular lymphocytic inflammatory infiltrate with eosinophils, blood extravasation.  Superficial perivascular lymphocytic inflammatory infiltrate, blood extravasation. 
  DIF: fibrinogen surrounding the vessels  DIF: negative  DIF: negative  Dermal edema and blood extravasation.  DIF: negative  DIF: negative  DIF: negative  DIF: negative 
        DIF: fibrinogen surrounding the vessels         
Analytical study  ANA by IIF/ELISA: negative  ANA by IIF/ELISA: negative  ANA by IIF/ELISA: negative  ANA by IIF: 1:320 homogeneous pattern  ANA by IIF/ELISA: negative  ANA by IIF/ELISA: negative  ANA by IIF/ELISA: negative  ANA by IIF/ELISA: negative 
  ANCA: negative  ANCA: negative  ANCA: negative  anti-ENA ab: negative  ANCA: negative  ANCA: negative  ANCA: negative  ANCA: negative 
  C3: 50.9mg/dL (91–190)  C3: normal  C3: normal  ANCA: negative  C3: normal  C3: normal  C3: normal  C3: normal 
  C4: 7.1mg/dL (18–56)  C4: normal  C4: normal  C3: 79.8mg/dL  C4: normal  C4: normal  C4: normal  C4: normal 
  C1q: not done      C4: 15.2mg/dL         
        C1q: 235mg/L (100–255)         
Treatment  PRED 0.5mg/kg/day in tapering regimen over 1 month  PRED 0.5mg/kg/day in tapering regimen over 1 month  PRED 0.5mg/kg/day in tapering regimen over 1 month  PRED 0.5mg/kg/day in tapering regimen over 1 month  H2-antihistamines  H2-antihistamines  H2-antihistamines  H2-antihistamines 
Outcome  Frequent relapses over a year of follow-up  Two previous episodes. No relapses over a 1-year period  No relapses over 1 year of follow-up  Lost to follow-up  No relapses over 1 year of follow-up  No relapses over 1 year of follow-up  No relapses over 1 year of follow-up  No relapses over 1 year of follow-up 

ab, antibody; ANA, antinuclear antibodies; ANCA, antineutrophil cytoplasmic antibodies; ELISA, enzyme-linked immunosorbent assay; ENA, extractable nuclear antigens; DIF, direct immunofluorescence; F, female; IIF, direct immunofluorescence LL, lower limbs; m, male; PRED, prednisone; UA, upper airway; UL, upper limbs;

Discussion

Urticarial vasculitis is characterized in clinical terms by urticaria-like wheals and histologically by leukocytoclastic vasculitis (LV). The rash lasts more than 24h and leaves residual purpura.1 In clinical practice, it is not uncommon to see urticaria-like conditions that persist over 24h that are accompanied by a purpuric component. Biopsy only reveals the presence of a superficial perivascular lymphocytic infiltrate. The cause of purpura in the absence of LV is controversial: some authors maintain that the origin is lymphocytic vasculitis2 and others that it is due to scratching.3 Lee et al. combine the 2 types of lesions as characteristics of prolonged urticaria with purpura, granting greater importance to the clinical similarities than to the histological resemblance. In our experience, recent UV has a homogeneous erythematous or purpuric color, and leaves purpura in the entire region of the rash on resolution (Fig. 1). The wheals with the lymphocytic infiltrate have areas of an ecchymotic yellowish color within the acute lesions or extending beyond their bounds (Fig. 2). We consider that, when urticarial wheals persist more than 24h, the residual purpura surrounding the entire area of the lesions supports the diagnosis of UV, whereas a yellowish or ecchymotic discoloration on the periphery or within the wheal corroborates the diagnosis of what Lee et al. classify as prolonged urticaria with purpura in the absence of LV. This could enable the avoidance of biopsy and other studies in single and self-limiting episodes.

Fig. 1.
(0.17MB).

Urticarial vasculitis. The 4 patients had undergone a biopsy that confirmed the diagnosis of leukocytoclastic vasculitis. Note the presence of acute urticaria-like wheals and lesions in resolution with purpura (a–d).

Fig. 2.
(0.2MB).

Prolonged urticaria without leukocytoclastic vasculitis. The 4 patients had undergone at least one biopsy that revealed the presence of a superficial perivascular lymphocytic inflammatory infiltrate, with or without eosinophils, in the absence of vasculitis. Note the presence of an ecchymotic component within the acute lesions (a and b) and extending beyond the bounds of the wheals (c and d).

Ethical DisclosuresProtection of human and animal subjects

The authors declare that no experiments were performed on humans or animals for this study.

Confidentiality of data

The authors declare that they have followed the protocols of their work center on the publication of patient data.

Right to privacy and informed consent

The authors have obtained the written informed consent of the patients or subjects mentioned in the article. The corresponding author is in possession of this document.

Conflicts of Interest

The authors declare they have no conflicts of interest.

References
1
F. Moreno-Suárez,Á. Pulpillo-Ruiz,T. Zulueta Dorado,J. Conejo-Mir Sánchez
Urticarial vasculitis: a retrospective study of 15 cases
Actas Dermosifiliogr, 104 (2013), pp. 579-585 http://dx.doi.org/10.1016/j.adengl.2012.12.005
2
J.S. Lee,T.H. Loh,S.C. Seow,S.H. Tan
Prolonged urticaria with purpura: the spectrum of clinical and histopathologic features in a prospective series of 22 patients exhibiting the clinical features of urticarial vasculitis
J Am Acad Dermatol, 56 (2007), pp. 994-1005 http://dx.doi.org/10.1016/j.jaad.2006.10.962
3
J. Guitart
“Lymphocytic vasculitis” is not urticarial vasculitis
J Am Acad Dermatol, 59 (2008), pp. 353 http://dx.doi.org/10.1016/j.jaad.2008.02.037

Please cite this article as: Lozano Masdemont B, Horcajada Reales C, Gómez-Recuero Muñoz L, Parra Blanco V. Características del componente purpúrico de la urticaria con vasculitis y de la urticaria sin vasculitis. Reumatol Clin. 2018;14:53–55.

Corresponding author. (Belén Lozano Masdemont belenmasdemont@gmail.com)
Copyright © 2016. Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología
Reumatol Clin 2018;14:53-5 - Vol. 14 Num.1 DOI: 10.1016/j.reumae.2016.09.003