Case Discussion
SLE is a chronic, recurrent, potentially fatal multisystem autoimmune
and inflammatory connective tissue disorder whose diagnosis can be
difficult due to the broad range of clinical manifestations and the lack
of pathognomic features or specific laboratory tests (6–8). Before
puberty, the female-to-male ratio of SLE occurance is 3:1; after
puberty, the ratio increases to 9:1. SLE is generally classified into
chronic cutaneous LE (CCLE), subacute cutaneous LE (SCLE), and acute
cutaneous LE (ACLE) (6,7).
The kidneys, brain, lungs, heart, skin, and joints are the major organs
affected by SLE, with commonly presenting symptoms including fatigue,
fever, arthralgias, myalgias, weight loss, rash, oral ulcers,
thrombocytopenia, and leucopenia. The mainstay of laboratory testing for
the diagnosis of SLE is the assessment of ANA. While a positive ANA test
result is useful in diagnosis, it is not specific for SLE. In contrast,
anti-ds DNA is relatively specific for SLE (8). Up to 30% of patients
will present with cutaneous symptoms, including butterfly-shaped facial
rash, red macules, papules, and plaques, alopecia, and mucosal ulcers.
(5)
In the case of the patient, the likely diagnosis of SLE was made based
on the clinical presentations, including fever, oral ulcers, and a
positive anti-ds DNA.
EM, on the other hand, is an acute, immune‑mediated mucocutaneous
condition characterized by the presence of multiple symmetric, typical,
or atypical target lesions with or without crusting at the center of the
lesion and concentric color variation mainly on extremities (hands,
feet, and the extensor aspects of limbs), with or without itching and
prodromal symptoms (7).
Classical or true EM is precipitated by trigger factors such as
infective agents like herpes simplex virus, mycoplasma pneumoniae, drugs
like anticonvulsants, antibiotics, and non-steroid anti-inflammatory
drugs, any underlying malignancy, or connective tissue disorders, and is
not associated with any specific serological abnormalities commonly seen
in autoimmune disease or with chilblain (6,7,9). In the case of the
patient, there was no identifiable precipitating cause of erythema
multiforme.
True EM is never associated with any specific autoimmune or serological
abnormalities. In our patient, the skin manifestations and
histopathologic findings are suggestive of EM, but the absence of any
triggering factors does not favor the diagnosis of true EM.
Cases of EM associated with LE lesions where an EM trigger factor is
missing, are considered a diagnostic criterion for RS (10). Rowell
syndrome was originally described in 1963 by Rowell et al., who
identified four females with discoid LE, EM-like lesions, and the
presence of one of the following serology: speckled ANA, anti-Ro/La
antibody, or rheumatoid factor (RF) (1). RS is characterized by the
combination of EM, LE, and typical serological abnormalities (11).
RS is an uncommon presentation of lupus erythematosus with erythema
multiforme-like lesions associated with specific serological changes,
including positive rheumatoid factor (RF), speckled ANA, positive
rheumatoid factor, or anti-La antibodies in the serum (7,11,12). The
speckled pattern of ANA is the most consistent diagnostic feature of
Rowell’s syndrome. Anti-La antibodies and rheumatoid factor seem to be
less consistent features (9). In our case, the patient had a positive RF
and a positive ANA with a speckled pattern.
There is a question as to whether the EM-like lesions of RS represent a
subset of SCLE since vesicobullous lesions that resemble EM may occur
rarely in SCLE. However, the vesicobullous lesions of SCLE do not result
in clinical necrosis or scarring, and the histopathological features do
not include necrosis of keratinocytes (11). Skin biopsy of the patient
reveals areas of ulceration, follicular plugging, mixed inflammatory
cells exocytosis, edematous, and hemorrhagic dermis with thrombi
formation suggestive of inflammatory dermatoses as depicted in figure 4.
In patients with SCLE, a positive ANA is seen in 75%; the pattern is
usually homogenous. Positive anti-Ro is found in 60% and a positive
rheumatoid factor in 30–40%. Immunofluorescence of lesional skin
reveals linear IgA, IgM, and C3 at the dermo-epidermal junction in 60%
of patients. The immunofluorescence from an EM-like lesion in our
patient was negative (11).
Before considering a diagnosis of RS, it is important to rule out common
triggering agents and other differentials of EM. In this case, no
precipitating factor for EM was identified. Also, a diagnosis of RS is
based on the presence of major and minor criteria, as seen in Table 1
(1). All three major and at least one minor criteria are required to
confirm RS (9).
In our patient, as all the major criteria, along with one minor
criterion i.e., positive RF, were present, we consider our case to be a
classic RS.
Table 1: Criteria for RS Diagnosis by Zeitouni et al. (1).