Home            Past Issues            About IP            About IAP           Author Information            Subscription            Advertisement              Search  

   
correspondence

Indian Pediatr 2013;50: 615

Further Considerations on The So-Called Rowell Syndrome


V Bonciolini and M Caproni

Department of Surgery and Translational Medicine, Section of Dermatology, University of Florence,
Piazza Indipendenza, 11, 50129 Florence, Italy
Email: [email protected]
 


The recent article by Solanki, et al. [1] described a further case of Rowell Syndrome (RS), that is a long debated nosological entity, historically defined as a unique clinical association between cutaneous lupus erythematosus (CLE) with erythema multiforme (EM) like lesions and characteristic immunologic pattern. Last year we reviewed all the 71 cases reported as RS up to 2011, and questioned its framing as separate entity [2].

The 13-years old female child described by Solanki, et al. [1] apparently fulfilled the diagnostic criteria suggested first by Rowell, et al. [3] in 1963 and then classified as major and minor by Zeitouni, et al. [4] in 2000. However, as already pointed out in our review, in the majority of cases, different entities were reported as RS, misdiagnosing association between subacute CLE (SCLE) annular polycyclic type, described for the first time in 1977 by Gilliam and better defined by Sontheimer et al. in 1979, and other specific type of CLE as discoid LE (DLE), acute CLE or chilblain lupus variant.

In our opinion, the case reported by Solanki, et al. [1] should be considered as SCLE, since it shows annular-polycyclic lesions on the upper chest (different from symmetrical typical raised targetoid lesions of EM) with erosive lesions of the hard palate, frequently reported as non-specific lesions of SCLE, representing a clinical marker of active disease (American College of Rheumatology Criteria). Other features, including photosensitivity and malar rash, strongly support our hypothesis, despite the negativity of anti-Ro antibodies, that are absent in about one third of the patients with SCLE.

In conclusion, we reiterate our critical opinion about RS, stressing the concept that different entities have been wrongly reported under this name. In particular, annular-polycyclic type of SCLE is often misdiagnosed as EM-like rash. Moreover, the real association between LE and EM, as happens for other associations (i.e. CLE and lichen planus or psoriasis), should be considered a mere coincidence, that does not justify the framing of a separate syndrome as originally suggested by Rowell, et al. [3]

Acknowledgments: Professor P. Fabbri and Doctor E. Antiga.

Reference

1. Solanki LS, Dhingra M, Thami GP. Rowell Syndrome. Indian Pediatr. 2012;49:854-5.

2. Antiga E, Caproni M, Bonciani D, Bonciolini V, Fabbri P. The last world on the so-called ‘Rowell’s syndrome’?. Lupus. 2012;21:577-85.

3. 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.

4. Zeitouni NC, Funaro D, Cloutier RA, Gagné E, Claveau J. Redefining Rowell’ syndrome. Br J Dermatol. 2000;142:343-6.

5. Sontheimer RD, Thomas JR, Gilliam JN. Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset. Arch Dermatol. 1979;115:1409-15.

 

Copyright 1999-2012 Indian Pediatrics