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Indian Pediatr 2013;50: 615 |
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Further Considerations on The So-Called Rowell
Syndrome
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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]
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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.
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