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Rowell syndrome
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Rowell syndrome — extra information
Synonyms:
Erythema multiforme-like lupus erythematosus, Lupus erythematosus and erythema multiforme-like syndrome
Categories:
Systemic diseases
ICD-10:
L93.2
ICD-11:
EB51
SNOMED CT:
403491004, 238926009

Systemic diseases

Rowell syndrome


Authors: Riyad NH Seervai, MD/PhD Student, Baylor College of Medicine, Houston, Texas, USA; Claire Jordan Wiggins, Medical Student, Baylor College of Medicine, Houston, Texas, USA. DermNet Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. June 2020.

Introduction Demographics Causes Clinical features Complications Diagnosis Differential diagnoses Treatment Outcome

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What is Rowell syndrome?

Rowell syndrome is a rare entity consisting of erythema multiforme-like lesions coexisting with lupus erythematosus (LE) along with characteristic immunological findings. The acceptance of Rowell syndrome as a distinct entity is controversial and not widely acknowledged.

The syndrome was first described in 1963 by Rowell, Beck, and Anderson who reported four out of 120 patients with chronic discoid LE with erythema multiforme-like lesions on the arms, legs, face, and chest, coinciding with a speckled pattern of antinuclear antibody (ANA), positive rheumatoid factor, and a precipitating antibody to saline extract from human tissue (anti-Sj-T) [1].

Rowell syndrome is currently classified as a subtype of chronic cutaneous lupus erythematosus [2].

Who gets Rowell syndrome?

Rowell syndrome has been reported in patients with systemic and discoid lupus erythematosus. Of the 96 reported cases, 18 are from India [3].

  • The majority of cases are women aged 20–70 years [1].
  • The ratio of affected women to men is 8:1 [3].
  • Indian patients have a median age 23 compared to age 32 worldwide and are less likely to have discoid lupus (5.6% versus 21.1% worldwide) [3].
  • Rowell syndrome is often preceded by a history of LE, but it has been reported to be its first sign [4–6].

What causes Rowell syndrome?

The pathogenesis of LE involves genetics and environmental factors [7].

  • The higher incidence in females has been attributed to oestrogen signalling, the composition of the microbiome, and increased levels of toll-like receptor TLR7 in immune cells [8,9].
  • Rowell syndrome been induced by drugs including terbinafine [10–12], omeprazole [12–14], norfloxacin [15], and sodium valproate [16].
  • It has also been provoked by sun exposure [3,17,18].

What are the clinical features of Rowell syndrome?

Rowell syndrome is characterised by erythema multiforme-like lesions in a patient with LE.

  • The erythema multiforme-like lesions are painful, pruritic, annular or targetoid, erythematous, red-to-violet plaques or blisters.
  • They are found on the chest and back, arms and legs, hands and feet, and face.
  • Other features of LE include:

What are the complications of Rowell syndrome?

Complications are mostly due to systemic LE, such as haematological and renal involvement, or other conditions [3,19]. Stevens Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) has been reported [20].

Rowell syndrome has also been associated with:

How is Rowell syndrome diagnosed?

The specific features of Rowell syndrome have been controversial [21,25-35].

It is currently considered to be a distinct type of chronic cutaneous LE with the following criteria [2].

1. Major criteria (all required)

a. Presence of chronic cutaneous LE

b. Erythema multiforme-like lesions (typical or atypical target lesions)

c. ≥1 positive speckled ANA (present in >90% [27,28]), anti-Ro/SSA, or anti-La/SSB antibodies

d. Negative direct immunofluorescence.

2. Minor criteria (one required)

a. Absence of infectious or pharmacological triggers

b. Lesions are not in acral or mucosal areas (the typical sites of erythema multiforme)

c. Presence of at least one additional criterion for systemic LE.

Histology shows a periadnexal lymphocytic infiltrate and periadnexal CD123+ plasmacytoid dendritic cells [32].

What is the differential diagnosis for Rowell syndrome?

The differential diagnosis for Rowell syndrome includes [2,21,31,32,34]:

What is the treatment for Rowell syndrome?

The treatment of Rowell syndrome includes the range of medicines used to treat LE, including:

What is the outcome for Rowell syndrome?

Most patients with Rowell syndrome respond well to therapy.

References

  1. Rowell NR, Beck JS, Anderson JR. Lupus Erythematosus and Erythema Multiforme-Like Lesions. A Syndrome with Characteristic Immunological Abnormalities. Arch Dermatol 1963; 88: 176–80. DOI: 10.1001/archderm.1963.01590200064012. PubMed Central
  2. Torchia D, Romanelli P, Kerdel FA. Erythema Multiforme and Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Associated with Lupus Erythematosus. J Am Acad Dermatol 2012; 67: 417–21. DOI: 10.1016/j.jaad.2011.10.012. PubMed
  3. Sharma YK, Chauhan S. Overlap Syndrome with Rowell's Syndrome, Antiphospholipid Syndrome, Primary Sterility, and Sensorineural Hearing Loss: A Case Report, Brief Review, and Analysis of Cases of Rowell's Syndrome Reported from India and Abroad. Indian J Dermatol 2018; 63: 418–23. DOI: 10.4103/ijd.IJD_437_17. PubMed
  4. Basu A, Ray Y, Bhowmik P, Rahman M, Goswami RP. Sle in a Male Patient Presented Initially as Rowell's Syndrome. J Assoc Physicians India 2018; 66: 98–9. PubMed
  5. Akintayo RO, Olarinoye GM, Akintayo FC, Ilesanmi ON. Rowell Syndrome in Nigeria: Systemic Lupus Erythematosus Presenting as Recurrent Erythema Multiforme in a Young Woman. Acta Dermatovenerol Croat 2019; 27: 200–1. PubMed
  6. Gallo L, Megna M, Festa B, Stellato P, et al. Rowell Syndrome: A Diagnostic Challenge. J Clin Aesthet Dermatol 2020; 13: 40–2. doi: 10.3892/etm.2017.5557. PubMed Central
  7. Li Q, Wu H, Zhou S, Zhao M, Lu Q. An Update on the Pathogenesis of Skin Damage in Lupus. Curr Rheumatol Rep 2020; 22: 16. DOI: 10.1007/s11926-020-00893-9. PubMed
  8. Guéry JC. Why Is Systemic Lupus Erythematosus More Common in Women? Joint Bone Spine 2019; 86: 297–9. DOI: 10.1016/j.jbspin.2018.12.004. PubMed
  9. Laffont S, Guéry JC. Deconstructing the Sex Bias in Allergy and Autoimmunity: From Sex Hormones and Beyond. Adv Immunol 2019; 142: 35–64. DOI: 10.1016/bs.ai.2019.04.001. PubMed
  10. Champagne C, Ratnavel R, Wong T. Severe Rowell Syndrome Associated with Oral Terbinafine. Clin Exp Dermatol 2012; 37: 822–3. DOI: 10.1111/j.1365-2230.2012.04363.x. PubMed
  11. Murad A, Shudell E, Mulligan N. Rowell's Syndrome Induced by Terbinafine. BMJ Case Rep 2015; 2015: bcr2015210360. DOI: 10.1136/bcr-2015-210360. PubMed Central
  12. Brănișteanu DE, Ianoşi SL, Dimitriu A, Stoleriu G, et al. Drug-Induced Rowell Syndrome, a Rare and Difficult to Manage Disease: A Case Report. Exp Ther Med 2018; 15: 785–8. DOI: 10.3892/etm.2017.5557. PubMed
  13. Pozharashka J, Dourmishev L, Balabanova M, Vassileva S, Miteva L. Rowell's Syndrome Triggered by Omeprazole. Acta Dermatovenerol Croat 2019; 27: 124–6. PubMed
  14. Schissler C, Banea S, Tortel MC, Mahé A. [A New Case of Rowell's Syndrome]. Ann Dermatol Venereol 2017; 144: 263–7. DOI: 10.1016/j.annder.2017.02.005. PubMed
  15. Baroni A, Piccolo V, Russo T, Cozzolino D, et al. Norfloxacin-induced Subacute Cutaneous Lupus with Erythema Multiforme-Like Lesions: The Enigma of the Rowell Syndrome. J Dtsch Dermatol Ges 2014; 12: 1039–42. DOI: 10.1111/ddg.12392. PubMed
  16. Kacalak-Rzepka A, Kiedrowicz M, Bielecka-Grzela S, Ratajczak-Stefanska V, et al. Rowell's Syndrome in the Course of Treatment with Sodium Valproate: A Case Report and Review of the Literature Data. Clin Exp Dermatol 2009; 34: 702–4. DOI: 10.1111/j.1365-2230.2008.02972.x. PubMed
  17. Ahluwalia J, Marsch A. Photosensitivity and Photoprotection in Patients with Lupus Erythematosus. Lupus 2019; 28: 697–702. DOI: 10.1177/0961203319839486. PubMed
  18. Alkul S, Behrens E, Stetson C. Rowell Syndrome with Recurrence from Photoexacerbation: A Case Report. SAGE Open Med Case Rep 2019; 7: 2050313x19847337. DOI: 10.1177/2050313x19847337. PubMed
  19. Shahid S, Khan M, Qadar LT, Akmal M, Jamal A. The First Case of Rowell Syndrome with Lupus Nephritis and Lobar Pneumonia in a Male Child Reported in Pakistan. Cureus 2019; 11: e4604. DOI: 10.7759/cureus.4604. PubMed
  20. Mandelcorn R, Shear NH. Lupus-Associated Toxic Epidermal Necrolysis: A Novel Manifestation of Lupus? J Am Acad Dermatol 2003; 48: 525–9. DOI: 10.1067/mjd.2003.107. PubMed
  21. Aydogan K, Karadogan S, Balaban Adim S, Tunali S. Lupus Erythematosus Associated with Erythema Multiforme: Report of Two Cases and Review of the Literature. J Eur Acad Dermatol Venereol 2005; 19: 621–7. DOI: 10.1111/j.1468-3083.2005.01233.x. PubMed
  22. Shadid NH, Thissen CA, van Marion AM, Poblete-Gutiérrez P, Frank J. Lupus Erythematosus Associated with Erythema Multiforme: Rowell's Syndrome. Int J Dermatol 2007; 46 Suppl 3: 30–2. DOI: 10.1111/j.1365-4632.2007.03508.x. PubMed
  23. Chua SH, Giam YC, Sim CS. Systemic Lupus Erythematosus with Erythema Multiforme-Like Lesions and Histiocytic Necrotizing Lymphadenitis--a Case Report. Ann Acad Med Singapore 1996; 25: 599–601. PubMed
  24. Aguirre-Martinez I, Vélez-Tirado N, García-Romero MT, Rodríguez-Lozano AL, et al. Rowell Syndrome Complicated with Macrophage Activation Syndrome in a Child. Lupus 2019; 28: 1716–21. DOI: 10.1177/0961203319886030. PubMed
  25. Schlotz M. Lupus Erythematosus Acutus Disseminatus Haemorrhagicus. Arch Dermatol Syphiligr 1922; 6: 466–75. doi:10.1001/archderm.1922.02360040068008. Journal
  26. Ormsby OM, Montgomery H. Diseases of the Skin. 6 ed. London: Henry Kimpton; 1943. p. 895.
  27. Bhat RY, Varma C, Bhatt S, Balachandran C. Rowell Syndrome. Indian Dermatol Online J 2014; 5: S33–5. DOI: 10.4103/2229-5178.144526. PubMed
  28. López Caleya JF, Martín Rodrigo L, Gonzalvo Rodriguez P, Hidalgo García Y. Rowell's Syndrome: The Two Sides of the Truth. Reumatol Clin 2016; 12: 354–5. DOI: 10.1016/j.reuma.2015.11.007. PubMed
  29. Lee S, Schloss E, Kowichi J. Rowell's Syndrome: A Case Report with Subacute Cutaneous Lupus Erythematosus and Erythema Multiforme. Can J Dermatol 1995; 7: 807–10.
  30. Antiga E, Caproni M, Bonciani D, Bonciolini V, Fabbri P. The Last Word on the So-Called 'Rowell's Syndrome'? Lupus 2012; 21: 577–85. DOI: 10.1177/0961203311430513. PubMed
  31. Zeitouni NC, Funaro D, Cloutier RA, Gagné E, Claveau J. Redefining Rowell's Syndrome. Br J Dermatol 2000; 142: 343–6. DOI: 10.1046/j.1365-2133.2000.03306.x. PubMed
  32. Herold M, Nielson CB, Braswell D, Merkel K, et al. Clinicopathologic Comparison of Rowell Syndrome, Erythema Multiforme, and Subacute Cutaneous Lupus Erythematosus. J Am Acad Dermatol 2019; 81: 1435–8. DOI: 10.1016/j.jaad.2019.06.008. PubMed
  33. Bonciolini V, Antiga E, Caproni M, Fabbri P. Rowell Syndrome: Does It Exist? Clin Exp Dermatol 2014; 39: 58. DOI: 10.1111/ced.12183. PubMed
  34. Modi GM, Shen A, Mazloom A, Mawad J, et al. Lupus Erythematosus Masquerading as Erythema Multiforme: Does Rowell Syndrome Really Exist? Dermatol Online J 2009; 15: 5. PubMed
  35. Mendonca R. Lupus Erythematosus and Erythema Multiforme-Like Lesions. Rowell's Syndrome. Dermatol Online J 1997; 3: 4. PubMed

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