Severe subacute cutaneous lupus erythematosus

presenting with generalized erythroderma

and bullae
Diya F. Mutasim, MD
Cincinnati, Ohio

A 31-year-old woman presented with progressive generalized erythroderma and bullae. Histologic evalu-
ation revealed dyskeratosis and interface dermatitis with a paucity of infiltrate. Serologic evaluation revealed
markedly elevated titer of Ro/SS-A and La/SS-B antibodies. Further workup revealed leukopenia. The
generalized eruption cleared with prednisone. The patient later had the classic discrete lesions of subacute
cutaneous lupus erythematosus develop. The erythrodermic and bullous presentation of subacute cutane-
ous lupus erythematosus is rare and requires a high index of suspicion. (J Am Acad Dermatol 2003;48:
947-9.)

I n 1979 Sontheimer et al1 characterized subacute

cutaneous lupus erythematosus (SCLE) as a sub-
set of lupus erythematosus (LE) that differed
from chronic cutaneous or discoid LE by several
factors.2,3 The clinical features include more exten-
eruption resolved with oral corticosteroids and
slowly evolved to the classic scaly patches of SCLE.

sive distribution, tendency to spare the face, minimal
or no scarring (mild telangiectasis and hypopigmen-
tation), and a characteristic morphology, namely an-
nular and polycyclic or papulosquamous and psori-
asiform.4,5 Approximately 50% of patients with SCLE
have 4 or more American Rheumatologic Associa-
tion criteria for systemic LE but have very mild dis-
ease. Histologic features of SCLE include paucity of
infiltrate compared with discoid LE, lack of follicular
plugging ,and lack of severe epidermal atrophy. The
majority of patients have circulating anti-Ro/SS-A
(and/or less frequently, anti-La/SS-B) antibodies.1,3,6
Unusual presentations of SCLE have been re-
ported. These include 1 case of toxic epidermal
necrolysis-like lesions,7 1 case with erythroderma,8
and 1 case of generalized poikiloderma.9 A patient is
reported herein who presented with erythroderma
and acral bullae. Histologic and serologic evaluation
confirmed the diagnosis of SCLE. The generalized
From the Department of Dermatology, University of Cincinnati Col-
lege of Medicine.
Funding sources: None.
Conflict of interest: None identified.
Reprint requests: Diya F. Mutasim, MD, Department of Dermatology,
University of Cincinnati College of Medicine, 231 Albert Sabin
Way, PO Box 670592, Cincinnati, OH 45267-0592. E-mail:
diya.mutasim@uc.edu.
Copyright © 2003 by the American Academy of Dermatology, Inc.
0190-9622/2003/$30.00 ⫹ 0
doi:10.1067/mjd.2003.244
CASE REPORT
A 31-year-old white woman presented with a
1-year history of progressive, pruritic patches and
blisters with recent onset of chills. The lesions did
not respond to a 10-day course of prednisone and
topical steroids. She was taking propranolol daily
and chlordiazepoxide HCl occasionally. Examina-
tion revealed an eruption all over the skin surface,
including the face, that consisted of confluent,
deeply erythematous patches with brownish des-
quamation (Fig 1, A). There were large bullae on the
sides and plantar aspects of both feet (Fig 1, B).
There were no oral or nail findings. Histologic eval-
uation of 3 biopsy specimens revealed focal to dif-
fuse parakeratosis, mild to severe dyskeratosis of the
epidermal keratinocytes resulting in focally full-
thickness epidermal necrosis (Fig 2), mild exocytosis
of lymphocytes, and a mild to minimal lymphocytic
infiltrate in the papillary dermis and focally along the
dermoepidermal interface. Direct immunofluores-
cence revealed deposition of IgG, IgA, IgM, C3, and
fibrinogen within numerous epidermal cells. There
was no fluorescence along the dermoepidermal
junction. A complete blood count revealed a white
blood cell count of 2.3 (normal ⱖ 3.5). The diagno-
sis of drug eruption presenting as interface derma-
titis was suspected. Chlordiazepoxide HCl was dis-
continued.
The patient was treated with prednisone 1 mg/
kg/d for 2 weeks, then tapered slowly. The lesions
were completely clear in 4 to 5 weeks. Prednisone
was discontinued 7 weeks after initiation of therapy.

947
948 Mutasim
Fig 1. A, Clinical examination revealed generalized erup-
tion consisting of confluent erythematous patches with
brownish peripheral desquamation. B, Clinical examina-
tion of plantar skin revealed diffuse erythema and exten-
sive bulla formation.
Six weeks later photorelated new lesions developed
all over the skin surface. The diagnosis of photosen-
sitive LE was suspected. Histologic evaluation re-
vealed findings similar to the previous biopsy spec-
imens. Direct immunofluorescence revealed
moderate granular deposition of IgG, IgM, C3, and
faint deposition of IgA within the epidermal basal
layer and, to a lesser degree, in the suprabasal ker-
atinocytes characteristic of SCLE.10 There was diffuse
deposition of fibrinogen along the dermoepidermal
junction. Serologic evaluation revealed positive an-
tinuclear antibody on Hep-2 cells (1:160, speckled),
markedly elevated titer of Ro/SS-A antibodies by
enzyme-linked immunosorbent assay (3285; normal
1-16), markedly elevated titer of La/SS-B antibodies
(1848; normal 1-16). Anticardiolipin antibodies
(both IgG and IgM) and antinative DNA antibodies
(by immunofluorescence) were negative. The diag-
nosis of SCLE was made. She was treated with hy-
droxychloroquine (200 mg twice daily), and UVA
J AM ACAD DERMATOL
JUNE 2003
Fig 2. Histologic examination revealed scattered dysker-
atotic epidermal cells, basal vacuolization, and a sparse
lymphocytic infiltrate. (Hematoxylin-eosin stain; original
magnification ⫻100.)
and UVB sunscreens. The eruption cleared com-
pletely in 6 weeks. There were mild flare-ups of few
annular and scaly patches in the following 2 years.
DISCUSSION
The diagnosis of SCLE in this case is on the basis
of the combination of clinical findings (especially
the later annular and scaly patches), histopathology
(interface dermatitis), immunofluorescence (granu-
lar fluorescence in keratinocyte cytoplasm), and
markedly increased titer of Ro/SS-A and La/SS-B
antibodies. The initial delay in the diagnosis of SCLE
was a result of an unusual clinical presentation and
nonspecific initial histopathology. Similar findings
may be seen in drug eruptions, Stevens-Johnson
syndrome, toxic epidermal necrolysis, and graft-ver-
sus-host disease. The clinical presentation of this
case is unique. First, the skin lesions were extensive,
involving the entire skin surface. Second, the patient
displayed severe desquamation and necrolysis re-
sulting in bullae mimicking Stevens-Johnson syn-
J AM ACAD DERMATOL
VOLUME 48, NUMBER 6
drome and toxic epidermal necrolysis. The role of
chlordiazepoxide as a possible trigger for SCLE in
this case is unlikely, because the eruption continued
for several months after discontinuation of the drug.
The prevalence of erythrodermic SCLE with bul-
lae is not known. One report7 describes a woman
with SCLE and systemic LE in whom bullae and
erosions developed at the active margins of polycy-
clic lesions of SCLE. Histologic examination revealed
keratinocyte necrosis, exocytosis of lymphocytes
and neutrophils, and clefts at the dermoepidermal
junction. She had antibodies to Ro/SS-A. The pres-
ence or absence of La/SS-B antibodies was not re-
ported. Another report8 describes a woman with
SCLE presenting with exfoliative erythroderma,
likely precipitated by excessive sun exposure. She
had similar findings to the current case, namely,
positive antinuclear antibody, positive Ro/SS-A an-
tibodies, positive La/SS-B antibodies, and leukope-
nia. The diagnosis of SCLE should be suspected in
some cases with generalized erythroderma, espe-
cially if there is no apparent cause, if the history
suggests a relation to UV light exposure, or if the
histologic findings reveal interface dermatitis. Sero-
logic evaluation for antinuclear antibodies and, spe-
cifically, Ro/SS-A and La/SS-B antibodies, would
help confirm the diagnosis.
In summary, a high index of suspicion is required
to confirm the diagnosis of SCLE that presents with
erythroderma and bullae. Histologic and immuno-
Mutasim 949
fluorescence evaluation is helpful. Serologic evalu-
ation usually confirms the diagnosis.
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