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Lupus erythematosus tumidus: a series of 26 cases

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Objective  To study 26 cases of lupus erythematosus tumidus (LET), a subset of chronic cutaneous lupus erythematosus (CCLE), referred to in the literature as a rare entity.Patients and methods  A retrospective study was conducted of 26 patients
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   International Journal of Dermatology   2006, 45  , 512–517© 2004 The International Society of Dermatology   512  Abstract  Objective  To study 26 cases of lupus erythematosus tumidus (LET), a subset of chronic cutaneous lupus erythematosus (CCLE), referred to in the literature as a rare entity.  Patients and methods  A retrospective study was conducted of 26 patients diagnosed with LET between 1996 and 2002. The clinical characteristics, histopathologic and laboratory findings, response to treatment, association with other subsets of lupus, course, and diagnostic criteria were analyzed.  Results  The incidence by sex was similar. The mean age of presentation was 49.19 years. The clinical presentation usually involved erythematous, edematous plaques located on the face, chest, back, or extremities, related to sun exposure. A dermal lymphocytic infiltrate with a perivascular disposition and differing degrees of mucin deposition was observed in all cases. Minimal epidermal changes were present in 18 cases, and 11 of these also showed minimal dermal–epidermal changes. Only one case showed dermal–epidermal changes without any epidermal alteration. Direct immunofluorescence test was performed in 15 patients, and 11 were negative. All cases showed a benign course without systemic manifestations. The response to topical steroids or antimalarial treatment was excellent, but a seasonal recurrence was usually observed.  Discussion  No defined criteria for LET are universally accepted. The main controversies are the acceptance of LET as a separate subset of CCLE, and the histopathologic diagnostic features, mainly the presence or absence of epidermal and dermal–epidermal changes in these lesions.  Conclusions  No inflexible histologic criteria should be employed for the diagnosis of LET. This subset of lupus erythematosus is characterized by intense photosensitivity, definite clinical lesions, a benign course, the absence of systemic disease, good response to antimalarial treatment, and a tendency to recur. More studies should be performed in order to establish the true incidence of LET because this subset of CCLE is probably underestimated.  BlackwellPublishing,Ltd.Oxford,UKIJDInternationalJournalofDermatology1365-4632BlackwellPublishingLtd,200445  Report  LupuserythematosustumidusVieiraetal.Report  Lupus erythematosus tumidus: a series of 26 cases  Vanessa Vieira, MD  , Jesús Del Pozo, MD  , Maria Teresa Yebra-Pimentel, MD  , Walter Martínez, MD  , and Eduardo Fonseca, MD  From the Departments of Dermatology and Pathology, Hospital Juan Canalejo, La Coruña, Spain  Correspondence  Vanessa Vieira, MD  Servicio de DermatologíaHospital Juan CanalejoXubias de Arriba 8415006 La CoruñaSpainE-mail: der@canalejo.org  Introduction  Gilliam’s classification includes lupus tumidus as a specificlupus erythematosus skin disease a form of chronic cutaneous lupuserythematosus (CCLE) with urticarial plaque morphology.   1  Lupus erythematosus tumidus (LET) as a subset of CCLEwas described by Gougerot and Burnier in 1930.   2  A previousdescription was possibly given by Hoffman in 1909.   3  Some articles on this entity have been published recently.All refer to LET as a rare entity.   4–6  Although only a fewcases of LET have been described in the literature, there isno reason to consider this entity as a rare disease. Its trueprevalence and incidence are unknown and probably havebeen underestimated.The consideration of LET as a distinct subset of CCLE hasbeen neglected in the literature. Numerous cases of LET havebeen diagnosed as polymorphic light eruption, Jessner’slymphocytic infiltration of the skin, reticular erythematousmucinosis, pseudolymphoma, subacute cutaneous lupuserythematosus, and other variants of CCLE.   7  Kuhn et al   .   8  recently analyzed the expression of epidermal surfacemolecules in patients with primary and UV-induced lesions of LET, discoid lupus erythematosus (DLE), and systemic lupuserythematosus (SLE), and concluded that LET is a distinctsubset of CCLE.No definitive clinical and histopathologic diagnostic criteriafor LET have been accepted. Kuhn et al   .   6,9  excluded the dia-gnosis of LET in cases of lupus erythematosus with dermal–epidermal junction or epidermal changes. Nevertheless, Dekle  et al   .   5  considered the diagnosis of LET in cases with minimalchanges in the epidermis or the dermal–epidermal junction.We report the clinical and histopathologic characteristicsof 26 cases of LET collected and analyzed retrospectively inour hospital.   © 2004 The International Society of DermatologyInternational Journal of Dermatology   2006, 45  , 512–517  513  Vieira   et al.Lupus erythematosus tumidus  Report  Materials and Methods  A retrospective study of 26 cases of LET diagnosed between 1996 and 2002 was conducted at the Dermatology Department of the Juan Canalejo Hospital. The diagnosis of LET was made according to clinical, histologic, and treatment response criteria (Table 1).Thirteen females and 13 males between 18 and 74 years of age (mean, 42.29 years) were included in the study. The main clinical characteristics of our cases are summarized in Table 2. A histopathologic study of lesional skin was performed in all cases (hematoxylin and eosin and Alcian blue stains), and a direct immunofluorescence test in 15.Routine laboratory tests were performed in all cases, antinuclear antibody (ANA) test in 25 cases, and complement levels in 16 cases. The recommended therapy and treatment response were evaluated.  Results  Erythematous and edematous plaques without scaling werethe most frequent presentation. Two cases showed superficialfine scaling, five a lilac color, three an annular configuration,and two a papular malar eruption (Figs 1–3).Twenty patients had facial lesions, five lesions on the chest,four on the back, seven on the upper extremities, and one onthe lower limbs. Nine patients presented with lesions inseveral locations, the face and upper extremities being themost frequent. Table 2 Clinical characteristics of lupus erythematosus tumidus patients   SexAgeLocationClinical featuresApproximate evolution until diagnosisRelationship with sun exposure 1F71FaceErythematous, edematous plaques with superficial scale8 yearsNo2F29FaceErythematous, edematous plaques5 yearsYes3M57BackErythematous, edematous plaques20 monthsNo4M38Face, chestErythematous, edematous plaques2 yearsNo5F31ChestErythematous, violaceous, edematous plaquesSeveral monthsYes6M48BackViolaceous plaques with edematous border and annular configuration1 yearNo7M49ChestErythematous, edematous plaques15 daysYes8M47Face, chestErythematous, violaceous, edematous plaques2 monthsYes9M47FaceViolaceous, edematous plaques3 yearsNo10M74FaceErythematous, edematous plaques1 yearYes11M30Face, upper extremitiesErythematous, violaceous plaques10 yearsYes12M44Face, upper extremitiesErythematous, edematous plaques13 yearsYes13F42Face, upper extremitiesErythematous, edematous plaques4 yearsNo14F48FaceErythematous, edematous plaques3 yearsNo15F25FaceErythematous, edematous plaquesYearsYes16F18FaceMalar eruption20 daysYes17M45FaceErythematous, edematous lesion with annular configuration1 monthNo18F24FaceMalar eruptionYearsNo19F26FaceErythematous plaquesYearsYes20F32Face, upper extremitiesErythematous papules10 yearsYes21M36Face, back, upper extremitiesErythematous, edematous plaquesYearsYes22F25FaceErythematous, edematous plaques with superficial scaleYearsYes23M68Face, chest, upper extremitiesErythematous plaques8 yearsYes24F46FaceErythematous edematous plaquesYearsYes25M45Upper and lower extremitiesErythematous plaques3 daysYes26F52ChestErythematous plaques with papulated margins2 yearsNo Table 1 Diagnostic criteria of lupus erythematosus tumidus in our patients   ClinicalErythematous, succulent, urticaria-like, nonscarring papules and plaques with a smooth surface, related to sun exposureHistologicPerivascular and periadnexal lymphocytic infiltrateInterstitial mucin depositionAbsence of or minimal epidermal changesEpidermal atrophySlight vacuolar degeneration of basal cellsSlight to moderate hyperkeratosisFollicular pluggingThickening and tortuosity of basal membrane with periodic acid–Schiff (PAS) stainNegative direct immunofluorescence (usually)AnalyticalNegative serologic tests, e.g. antinuclear antibody (ANA) (usually)   International Journal of Dermatology   2006, 45  , 512–517© 2004 The International Society of Dermatology   514Report  Lupus erythematosus tumidus  Vieira   et al.  The interval between the onset of the disease and theconfirmation of the diagnosis ranged between 3 and 13 years.This period was usually of several years, and no differencesbetween males and females were observed with regard to theonset of the disease.A clear relationship with solar exposure was found in 16 of the 25 patients. In the remaining patients, this finding was notnoted in the history. The interval between sun exposure anderuption development was less than 2 weeks.The histopathologic features are summarized in Table 3.Minimal epidermal changes were found in 18 patients, includ-ing epidermal atrophy, slight vacuolar degeneration of basalcells, slight to moderate hyperkeratosis, and follicular plugging(Fig. 4). An enhanced and irregular basal membrane at thedermal–epidermal junction was observed in 11 patients (50%),accompanying epidermal changes. Only one patient had noepidermal changes (Fig. 5). All patients showed a predomin-antly lymphocytic infiltrate with a perivascular and peri-adnexal disposition. A variable degree of mucin depositionin the dermis and around the adnexae was demonstrated in allpatients (Fig. 6). A direct immunofluorescence test was per-formed in 15 patients, with 11 being negative. Two patientsshowed slight immunoglobulin G (IgG) deposition at the basalmembrane, one had moderate deposition of IgG and slightdeposition of C   3  , and one had slight deposition of IgM, C   3  ,and C   1q  .Twenty-three patients had normal hemograms. In three,slight anemia, with hemoglobin values around 10 g/dL, wasdetected. The erythrocyte sedimentation rate was measuredin 22 cases: 20 were normal and two showed slight elevation.Twenty-four patients had a negative ANA test. One patienthad a positive ANA test at a dilution of 1 : 160 with a homo-geneous pattern and negative anti-DNA and anti-ENA (extract-able Nuclear Antigen) test. Complement values were deter-mined in 16 patients, with normal values in 11. C   4  was decreasedin three cases, and C   3  and C   4  were decreased in two cases.Solar protection was recommended in all patients. Theinitial pharmacologic treatment with moderate potency topicalsteroids controlled the symptomatology and cleared the Figure 1 Erythematous and edematous plaque of the face Figure 2 Multiple confluent lesions on the back Figure 3 Papular and plaque-like lesions on the face   © 2004 The International Society of DermatologyInternational Journal of Dermatology   2006, 45  , 512–517  515  Vieira   et al.Lupus erythematosus tumidus  Report  cutaneous lesions in 21 patients. Only five patients requiredoral antimalarial treatment for control of their cutaneouslesions.The majority of patients had mild recurrences, usuallyduring spring or summer, related to the first sun exposure.These recurrences were successfully controlled with the sametreatments. More than 50% of patients remained asympto-matic only with adequate solar protection.None of the patients developed SLE or other type of CCLEduring the course of their disease.  Discussion  The main difficulty in this study was this variant of CCLE isto define LET. No criteria for this entity have been universallyaccepted, although several proposals have been made.   6,10  Notall authors accept LET as a variant of CCLE. It is possible that Table 3 Histopathologic features 1234567891011121314151617181920212223242526 Epidermal atrophy+–+–––+–+–+––+–––+–––––+–– Slight vacuolar degeneration of basal cells –––––++–++–++––++++––+––+– Slight to moderate hyperkeratosis ––+––+–––+–+––++–+–––+–––– Follicular plugging+++–––––––++–––––––––––––––– Enhanced and irregular basal membrane+––+––+–+–++–+–+–++–––––+– Lymphocytic infiltrate++++++++++++++++++++++++++Mucin deposition++++++++++++++++++++++++++IgG (DIF)++––––No–NoNoNoNo+–No–No–+No–––No–NoNoIgM (DIF)–––––No–NoNoNoNo––No–No––No+––No–NoNoC 3  (DIF)+––––No–NoNoNoNo––No–No––No+–No–NoNoC 1q  (DIF)–––––No–NoNoNoNo––No–No––No+––No–NoNo DIF, direct immunofluorescence; Ig, immunoglobulin; No, not performed. Figure 4 Minimal dermal–epidermal changes. Slight perivascular and interstitial dermal lymphocytic infiltrate (hematoxylin and eosin stain, × 40) Figure 5 Periodic acid–Schiff (PAS) stain showing slight thickening of the basal membrane ( × 40)   International Journal of Dermatology   2006, 45  , 512–517© 2004 The International Society of Dermatology   516Report  Lupus erythematosus tumidus  Vieira   et al.  some cases of LET have previously been included as otherforms of CCLE, that other variants of CCLE have beendiagnosed as LET, and that patients with initial findingscompatible with LET have evolved into other variants of CCLE. In addition, some cases of LET may not have beencategorized as CCLE and instead diagnosed as other entities,such as reticular erythematous mucinosis or lymphocyticinfiltrate of Jessner.In our series, no gender predominance was observed (13males and 13 females), contrary to Kuhn et al   .   6  Nevertheless,the sample is too small to make firm conclusions aboutgender prevalence.Clinically tumid, erythematous and edematous lesionswere the predominant presentation. In our patients, similar toKuhn’s series,   6  these lesions did not occur in the context of DLE or SLE, as has been described by other authors.   4,10  The relationship with solar exposure was not documentedin all patients. This is possibly because the latency period insome cases may last for several weeks after sun exposure. Aphototest study performed in 60 patients with LET revealedcharacteristic skin lesions in 72% of cases.   11  Around 50% of cases reacted to UV-A, 50% reacted to UV-B, and 63%reacted to combined UV-A and UV-B. In some cases, specificskin lesions in these phototests appeared up to 4 weeksafter the last irradiation. Similar results have been describedby Sanders et al   .   12  in a study of 100 patients with lupuserythematosus.The location of the lesions, relation to solar exposure,common absence of systemic manifestations, response totopical steroids or systemic antimalarial treatment, andtendency to recur were similar in our patients and the casesdescribed in the literature.A key controversial question in LET is the histologicpicture. A lymphocytic perivascular and periadnexal infiltrate,dermal mucin deposition, and often negative direct immuno-fluorescence test are accepted by the majority of authors.Nevertheless, the possible existence of minimal epidermaland dermal–epidermal junction changes is accepted by somein LET,   5,13  and considered as an exclusion criterion byothers.   4,6,9,14  In our opinion, the existence of minimal epidermal ordermal–epidermal changes accompanying typical dermalfindings can be accepted in LET for the following reasons:  •  The clinical and histopathologic heterogeneity of all subsetsof cutaneous lupus erythematosus is evident.  •  The transition and overlap between several forms of CCLEin the same patient have been reported.   4  LET has beendescribed as coexisting or evolving to DLE   15  and associatedwith SLE.   16  •  Polymorphic light eruption, reticular erythematous mucino-sis, and lymphocytic infiltration of the skin   17  are consideredby some authors as variants of CCLE. The histologic find-ings of polymorphic light eruption may include minimalepidermal or dermal–epidermal changes.   18  •  Lehmann et al   .   19  experimentally reproduced skin lesions of lupus erythematosus with UV-A and UV-B irradiation; theyconcluded that UV-B possibly has more pronounced effectson the epidermis, because the epidermis absorbs up to 95%of UV-B, whereas UV-A is less damaging to keratinocytesbecause it penetrates deep into the dermis. Photosensitivitystudies in LET have revealed that 50% of cases are induced byUV-B radiation.   11,12  •  We described a case with initial findings of reticularerythematous mucinosis, without epidermal changes, thatdeveloped criteria for SLE. A repeated biopsy performedin the same area revealed the epidermal changes of lupuserythematosus.   20  It is possible that the appearance of epidermaland dermal–epidermal changes in patients with LET is alteredaccording to the evolution of the disease.  •  Sontheimer   7  includes LET in a polar extreme of the spec-trum of cutaneous histopathologic changes that can be seenin lupus erythematosus-specific skin disease. In this polarextreme, only dermal changes or minimal epidermal changesmay be included.  •  Kuhn’s criteria for LET exclude cases with epidermal ordermal–epidermal alteration. Dermal mucin deposition and alymphocytic infiltrate are the sole histopathologic changesaccepted by this author in LET, but they are not specific dia-gnostic criteria for lupus.With a less restrictive interpretation, the presence of epidermaland dermal–epidermal alterations, overlying the dermal inflam-mation and mucin deposition in the majority of patients,supports the classification of LET as a subset of CCLE.In our series, minimal epidermal changes were found in18 patients and dermal–epidermal changes in 12 patients. Theclinical picture of tumid lesions was confirmed, and a benigncourse was observed. Adequate response to topical steroidsand antimalarial treatment was observed. These findingssuggest that strict histologic criteria alone cannot be currentlyemployed in the diagnosis of LET. Figure 6 Alcian blue stain showing mucin deposition intermingled between dermal collagen bundles ( × 100)
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