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2015 ; 94
(46
): e2102
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Blisters and Loss of Epidermis in Patients With Lupus Erythematosus: A
Clinicopathological Study of 22 Patients
#MMPMID26579826
Merklen-Djafri C
; Bessis D
; Frances C
; Poulalhon N
; Debarbieux S
; Cordel N
; Lipsker D
Medicine (Baltimore)
2015[Nov]; 94
(46
): e2102
PMID26579826
show ga
The nosology of bullous lesions or equivalents (vesicles, erosions, and crusts)
in patients with lupus erythematosus (LE) is rarely addressed.The primary aim of
this study was to draw up a precise phenotypic inventory of such skin lesions;
the secondary objective was to assess a potential relationship between the
different types of loss of epidermis and extracutaneous lupus manifestations.We
conducted a retrospective multicenter study including 22 patients with definite
LE and bullous lesions or equivalents. All biopsies were reviewed. Patients were
recruited in the dermatology departments of 6 centers. Patients were included if
they met the diagnosis of systemic LE according to American College of
Rheumatology and/or Systemic Lupus International Collaborating Clinics criteria
or diagnosis of cutaneous LE based on classic clinical criteria and/or
histological ascertainment of LE. Patients were recruited through clinician's
memory and photographic collections.Three clinico-pathological patterns could be
individualized. First, toxic epidermal necrolysis (TEN)-like, sheet-like, skin
detachment; sun-exposure, mild mucosal involvement, and dermal mucin deposition
allow differential diagnosis with classical Lyell syndrome. Second,
vesiculo-bullae and/or crusting occurring on typical lesions of subacute
cutaneous lupus erythematosus or chronic cutaneous lupus erythematosus. Third,
tense vesicles and/or blisters with an underlying neutrophilic dermatosis and a
usual response to dapsone.A careful analysis of 22 LE patients with epidermal
detachment reveals 2 main pathomechanisms: a classic LE interface dermatitis,
which can be hyperacute and lead to TEN-like skin detachment; and a neutrophilic
dermatosis, with tense vesicles and/or blisters, including classic bullous LE.
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