Clinics in Dermatology (2014) 32, 153–158

The red face revisited: Connective tissue disordersJana Kazandjieva, MD, PhDa,⁎, Nikolai Tsankov, MD, PhDb,Kyrill Pramatarov, MD, PhD c

aDepartment of Dermatology and Venereology, Medical University, Sofia, BulgariabDepartment of Dermatology and Venereology, Tokuda Hospital, Sofia, BulgariacDepartment of Dermatology, Medical University-Sofia, University Hospital Lozenez, Sofia, Bulgaria

Abstract Red face is not a rare finding in patients with connective tissue disorders. The malar eruption is themost frequent cutaneous manifestation of systemic lupus erythematosus (LE). This condition is moreapparent among fair-skinned individuals, and it usually appears after sun exposure. A very important clinicalsign is that nasolabial folds remain free of any erythematous or other changes. With subacute cutaneous LE,sun exposure can provoke a red face that resembles the malar eruption of systemic LE. The typical clinicalfindings of chronic cutaneous LE are the discoid lesions. There is a clinical form of chronic cutaneous LEcalled erythema perstans faciei. This form is purely erythematous, and it usually appears on the face. Otherrare “red face” forms of chronic cutaneous LE are LE tumidus and LE telangiectaticus.

Red face is not typical of systemic sclerosis, but facial telangiectasias are frequent, especially withCREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia)syndrome. The differential diagnoses of other red face manifestations are easy due to the additional findings.Telangiectasias are accompanied by calcinosis, sclerodactyly, digital ischemia, and Raynaud disease. Manystudies mention telangiectasias as markers of the severity of the systemic sclerosis, the disease duration, anypulmonary arterial hypertension, and any esophageal involvement.

Purple- or violet-colored upper eyelids are the hallmark and one of the first clinical signs that is helpful forthe diagnosis of dermatomyositis. This violaceous to dusky erythema can extend over the whole face and theupper aspects of the trunk. Erythematous changes on the face that are different from those of the heliotropesign which occurs with dermatomyositis may be observed in both sun-exposed skin and non–sun-exposedskin. Malar and facial erythema, linear extensor erythema, V-sign or shawl sign, and other photodistributederuptions can also appear.© 2014 Elsevier Inc. All rights reserved.

Red face with lupus erythematosus

The skin lesions of patients with lupus erythematosus(LE) can present as lupus-specific or lupus-nonspecificfindings. Lupus-specific skin changes occur with chronic

⁎ Corresponding author. Tel.: +359 2 9230511.E-mail address: janaderm@abv.bg (J. Kazandjieva).

0738-081X/$ – see front matter © 2014 Elsevier Inc. All rights reserved.http://dx.doi.org/10.1016/j.clindermatol.2013.05.037

cutaneous, subacute cutaneous, and acute cutaneous LE.1

Localized acute LE has commonly been referred to as theclassical “butterfly eruption.”2

Systemic lupus erythematosus

Systemic LE (SLE) involves cutaneous changes that arelisted in the criteria established by theAmericanRheumatologyAssociation.3 These include malar eruption, discoid lesions,photosensitivity, and oral mucosal lesions.

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The malar eruption is the most frequent cutaneousmanifestation of SLE. It is an erythematous and very slightlyedematous lesion that is located on both cheeks and acrossthe bridge of the nose. It is more obvious among affectedfair-skinned individuals, and it usually appears after sunexposure. These lesions will then disappear, and they leaveno scars or pigmentation. Malar eruption may be mistakenfor sunburn, the flush of rosacea, or photosensitivedermatitis. If the erythema persists for a few weeks or ifnew erythema appears elsewhere, the true diagnosis is themalar eruption of SLE. A very important clinical sign is thatthe nasolabial folds remain free of any skin changes; this isthe main clinical differentiation between malar eruption andseborrheic dermatitis. The differentiation of the malar eruptionsign of SLE from other connective tissue diseases may be verydifficult and sometimes impossible. Clinical differential diag-noses include—in addition other conditions—telangiectaticrosacea, superficial fungal infections, photosensitive dermatitis,erythema emotivum, rubeosis diabeticorum, and the eruptionof some viral infections. The histological findings depend onthe type of clinical representation. The main pathologicfindings are thinning of the epidermis; hydropic degenerationof the basal layer with disruption of the dermoepidermaljunction; edema of the dermis in upper portion; sparseinfiltration of lymphocytes in most of the dermis; and fibrinoiddegeneration of the connective tissue. With direct immunoflu-orescence, deposits of immunoglobulins G and M, as well as ofC3, are detected at the basal membrane zone; these deposits arefound both in lesions and in uninvolved skin. The histopath-ologic findings are compatible with both SLE and dermato-myositis, but direct immunofluorescence is negative in patientswith dermatomyositis.

Subacute cutaneous lupus erythematosus

Patients with subacute cutaneous LE have disseminated,nonscarring, photodistributed eruptions. There are twomajor subtypes of the disease: erythematous annular,which resembles erythema annulare centrifugum, anderythematous squamous, which resembles psoriasis orlichen planus. Subacute cutaneous LE belongs to thegroup of well-defined photodermatoses.

Sun exposure can provoke a red face that resembles themalar eruption of SLE,4 and it may also provoke systemicinvolvement in some patients. This condition also involves adifferential diagnosis that includes rosacea, photodermatitis,rubeosis, erythema emotivum, dermatomyositis and so on.

Chronic cutaneous lupus erythematosus

Chronic cutaneous LE is limited to the skin only. Thetypical lesions of this condition are the discoid lesions.Becausethese lesions are the most common skin changes, sometimesthe term chronic discoid LE is used as a synonym for chroniccutaneous LE. The discoid lesions begin with red papules ormaculae that are covered with silvery scales. A closer view of

the scales shows follicular keratosis, which is a very importantclinical finding for this disease. Later, atrophy and scarringappear after the active stage of the disease.

The histology of chronic cutaneous LE includes thefollowing findings: atrophic epidermis, compact hyperkera-tosis with follicular plugging, and hydropic degeneration ofthe cells of the basal layer. In the upper portion of the dermis,there are edema and mucin; during the later stages of thedisease, sclerosis, dilatation of the blood vessels, and denselymphocytic infiltrate around the vessels of the upper anddeep vessels and the adnexa are also present. With the use ofdirect immunofluorescence, deposits of immunoglobulins GandM as well as C3 are detected at the basal membrane zone,but these are found in the skin only.5

There is a clinical form of the chronic cutaneous LE callederythema perstans faciei. This form is purely erythematous,and it usually appears on the face. The lesions areerythematous macules or plaques with little scaling andvery little edema. There is a lack of severe inflammation, andthe pathologic process is superficial. The lesions disappearwith minimal atrophy. The acute stage of these lesionsresembles the malar eruption of SLE, and it is very difficultto differentiate between these conditions.6

Some rare variants of chronic cutaneous LE present theclinical picture of a red face. LE edematosus has been describedas erythematous and edematous, without well-demarcatedplaques, follicular plugging, or scarring.7 The lesions aresometimes multiple, and they regress without residual changes.The diagnosis may be difficult because the histological andimmunologic findings are not always compatible with chroniccutaneous LE.

Another rare form of chronic cutaneous LE is LE tumidus(Figure 1). This rare form is characterized by erythematous,succulent, edematous, nonscarring plaques in sun-exposedareas.8 Follicular plugging and scarring are usually absent.The lesions can be widespread; they affect all photoexposedareas (ie, back, neck, arms, and shoulders), but they occupythe face predominantly. They sometimes disappear sponta-neously, but they can also coalesce to form annular plaquesthat resemble the annular type of subacute cutaneous LE.Patients with LE tumidus never show systemic involvement.This is one of the most photosensitive subtypes within thespectrum of LE, and Ro/SSA antibodies are detected in allpatients. Due to the peculiarities of the clinical andhistopathological findings, this variant needs to be differen-tiated from polymorphous light eruption and Jessner lympho-cytic infiltration. Additional differentiation from reticularerythematous mucinosis is also required.

LE telangiectaticus is another rare form of chroniccutaneous LE (Figure 2). The most important clinical featureof this variant is erythema. A closer view reveals that thiserythema is caused by telangiectasias. The lesions involvethe face, and they can also occur on the extremities and theback. The telangiectasias are usually reticulate,9 andfollicular plugging and scarring are usually absent. Themost important clinical differential diagnosis of this subtype

Fig. 1 LE tumidus. From Pramatarov K. Chronic cutaneous lupuserythematosus—Clinical spectrum.ClinDermatol. 2004;22:113–120.

Fig. 3 LE hemorrhagicus. From Pramatarov K. Chroniccutaneous lupus erythematosus—Clinical spectrum. Clin Dermatol.2004;22:113–120.

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is rosacea. Sometimes, prominent hemorrhagic changes areone of the most common clinical signs of the type of chroniccutaneous LE that presents with multiple petechiae (Figure 3).The initial skin changes of this variant, which is called LEhemorrhagicus, resemble an acute photosensitive reaction. Itshould be noted that similar telangiectasias have been observed

Fig. 2 LE telangiectaticus. From Pramatarov K. Chroniccutaneous lupus erythematosus—Clinical spectrum. Clin Dermatol.2004;22:113–120.

in patients with other connective tissue diseases (eg, systemicsclerosis, rheumatoid arthritis, dermatomyositis, and undiffer-entiated connective tissue diseases).

Chilblain lupus is another form of this disease, and it ismost common in the northern countries. The typical lesionsassociated with this condition are acrocyanotic, and theyresemble perniones (Figure 4). They are localized on the skinof the face and predominantly on the nose and the ears. Skin

Fig. 4 Chilblain lupus. From Pramatarov K. Chronic cutaneouslupus erythematosus—Clinical spectrum. Clin Dermatol. 2004;22:113–120.

Fig. 5 Facial teleangiectasias in systemic sclerosis.

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lesions can occur also on the dorsal surfaces of the hands,feet, toes, fingers, knuckles, knees, and elbows. Sometimes,follicular plugging and scarring are present. Usually thedisease appears during the winter and resolves duringthe summer months. The chilblain lesions occur after thedevelopment of the discoid lesions; when the latter disappearafter treatment, however, the chilblain lesions persist. In halfof the patients with chilblain lupus, the perniotic lesions arethe only clinical sign of the disease; it is quite possible thatthese patients develop systemic involvement later.5,10 Themain differential diagnoses of chilblain lupus are lupuspernio (a form of sarcoidosis) and acrocyanosis.

LE profundus is a subtype of LE. It can be a clinicalmanifestation of chronic cutaneous LE and also of SLE. LEprofundus was probably first described by Kaposi in 1883. In1940, Irgang reviewed the cases reported by Kaposi andnamed this clinical entity. There is still some controversyabout the use of the term LE profundus. This condition issometimes referred to as LE panniculitis (as a synonym forLE profundus), whereas other times this name is usedwhen LE profundus is a clinical sign of SLE.

LE profundus is characterized by deep inflammatorynodules that resemble those of panniculitis. The nodules arelocated in the deep dermis and in the hypodermis. LEprofundus most commonly affects the cheeks, but it can alsoaffect the rest of the face (especially the eyelids), the arms, thehands, the breasts, the buttocks, the trunk, and the legs. Thesize of the lesions varies from 1 cm to several centimeters indiameter. The overlying skin is normal or red, or it may showthe changes of discoid LE. Local trauma often plays a role as aprovoking factor. Longstanding lesions are atrophic, andclinically they form typical depressions of the skin.

A case of LE profundus with periorbital swelling has beenreported.11 The periorbital swelling may be the first clinicalsign of LE profundus, and it may persist during the course ofthe disease if the changes are localized to the face.

Red face with systemic sclerosis

Red face is not typical for patients with systemic sclerosis(SSc), but facial telangiectasias are a common finding(Figure 5), especially in those with CREST syndrome. Theacronym CREST stands for calcinosis, Raynaud phenome-non, esophageal dysmotility, sclerodactyly, and telangiecta-sia; it is considered a rare form of systemic sclerosis.

CREST syndrome was first reported by dermatologistGeorge Thibierge and rheumatologist Raymond Weissen-bach in 1910.12 They described the occurrence of subcuta-neous calcification in patients with scleroderma andemphasized that this was not a coincidental finding but rathera causal link.13 The diagnosis of CRST (calcinosis, Raynaudphenomenon, sclerodactyly, and facial and truncal telangiec-tasiae) was coined in 1964 by Winterbauer.14,15 That authorwas the first to mention telangiectasias as a part of thesyndrome, which he described in a series of eight patients. In

1979, CRST was lengthened to CREST by Shulman andcolleagues, who added esophageal involvement to thecardinal manifestations.16 Antinuclear antibodies, recognizedas chromosomal centromere proteins, were later reported as acharacteristic sign that was present inmore than 50% of cases.Other manifestations include arthralgias, pulmonary hyper-tension (late finding), and primary biliary cirrhosis. Manyauthors have used the term limited cutaneous systemicsclerosis rather than CREST syndrome, when referring topossible systemic involvement.17

A histologic examination of telangiectasias in the settingof limited scleroderma was made.18 The investigationrevealed dilated postcapillary venules located in the papillaryand superficial reticular dermis. The vessel walls consisted ofnonfenestrated endothelial cells surrounded by a variablenumber of pericytes and smooth muscle cells. According tothe study, the immunohistological and ultrastructuralfeatures of the lining endothelium of established telangiec-tasias in patients with longstanding limited sclerodermaappeared to be benign.

Another study looked at endoglin levels in the serum ofpatients with limited cutaneous SSc.19 The investigators’data showed that patients with elevated serum endoglinlevels had telangiectasia more frequently as compared withthose patients with normal levels. According to the study,serum endoglin levels were significantly elevated in thesepatients as compared with the levels found in those withdiffuse cutaneous SSc and SLE.

A proposed classification system separated the scleroder-ma spectrum disorders into six different categories.20

According to this categorization, CREST syndrome is type

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VI. An exact diagnosis requires telangiectasia with one ormore other CREST base criteria or anticentromere antibodieswith any two or more criteria.

Contrary to the commonly accepted opinion, telangiecta-sias are often present as part of the clinical picture of SSc. Ina study of 120 patients with progressive systemic sclerosis,the incidence of telangiectasias was calculated to be 75% to100%.21 According to the investigators, the ramosus andtelangiectatic mats types of telangiectasias are typical forSSc. The ramosus type mainly appears on the face, neck, andchest, whereas the telangiectatic mats type usually involvesthe upper extremities and is the only type that appears at thelips. Cuticular telangiectasia is a third type; this is asimportant as the others but less frequent.

Many studies mention telangiectasias as a marker for theseverity of SSc. The incidence of telangiectasias is believedto be related to disease duration,12 and a significantassociation between pulmonary arterial hypertension inpatients with scleroderma and their number of telangiectaseshas been observed.22 A Japanese study concluded that thepresence of telangiectasia may be a marker of esophagealinvolvement, decreased carbon monoxide diffusion capac-ity, and heart involvement.23

Nine criteria with the abbreviation ABCDCREST havebeen proposed: (1) autoantibodies to centromere proteins; (2)bibasilar pulmonary fibrosis; (3) contractures of the digitaljoints; (4) dermal thickening proximal to the wrists; (5)calcinosis cutis; (6) Raynaud phenomenon; (7) esophagealdistal hypomotility; (8) sclerodactyly; and (9) telangiecta-sias.24 The presence of three or more of these criteriaindicates definite systemic scleroderma.

The differential diagnosis with other red face manifestationsis easy because of the additional findings. Telangiectasias areaccompanied by calcinosis (confirmed with plain radiographs),sclerodactyly, digital ischemia, infarction secondary to Raynauddisease, or some combination of these. A biopsy is required tomake the diagnosis of Barrett esophagitis or adenocarcinoma.

Pulsed-dye laser treatment has been shown to be effectivefor the treatment of facial telangiectasia.25 This treatmentmodality has been studied in patients with SSc but not inthose with CREST syndrome.26 Many methods have beensuccessfully used to treat symptomatic gastrointestinaltelangiectasia (eg, medical treatment with estrogen andprogesterone or desmopressin; laser ablation; sclerotherapy).Bowel resection for uncontrollable gastrointestinal bleedingas a result of telangiectasia is rarely necessary.

Fig. 6 Periorbital edema and telangiectasias in dermatomyositis.

Red face with dermatomyositis

Periorbital erythema is included in the set of the five criterianecessary for the diagnosis of myositis (together withprogressive proximal symmetrical weakness, elevated levelsof muscle enzymes, an abnormal finding on electromyogra-phy, and an abnormal finding on muscle biopsy).27 Purple orviolet-colored upper eyelids are the hallmark of and one of the

first clinical signs to be helpful for the diagnosis ofdermatomyositis. The term heliotrope sign refers to the flowerHeliotropium peruvianum and its specific purplish petals.28

This violaceous to dusky erythema can extend over the wholeface and the upper portion of the trunk. Among patients withdarker skin types (ie, types III through VI), erythema can besubtle and overlooked. The heliotrope eruption is oftenassociated with periorbital edema and telangiectasias on theupper eyelids (Figure 6). In those with chronic dermatomy-ositis, hypopigmentation or hyperpigmentation may appear atthe site of the erythema. Poikiloderma in a photosensitivedistribution is a possible finding in some cases; sun exposureusually aggravates the eruption. According to some studies inthose with juvenile dermatomyositis, the heliotrope eruptionand Gottron papules appeared less frequently.29

Erythematous changes of the face that differ from those ofthe heliotrope sign among patients with dermatomyositismay be observed in both sun-exposed skin and non–sun-exposed skin. Malar and facial erythema, linear extensorerythema, V-sign or shawl sign, and other photodistributederuptions can appear. A confluent erythema in the malardistribution that involves the cheeks and that extends overthe nasal bridge may be seen. Nasolabial folds are oftenspared with dermatomyositis, which differs from what isseen with SLE.30 Widespread erythema that affects theperioral area, the forehead, and the lateral face and ears canbe noticed. Erythema often involves the cartilaginous portionof the helix of the ear, with sparing of the earlobe. Theeruption may spread over the neck, the anterior chest (V-sign), or the back and shoulders (shawl sign). Oftenerythematous lesions are accompanied by secondarychanges, such as scaling, crusting, or erosion.

The skin changes are treated by avoiding sun exposureand by using sunscreens, topical corticosteroids, antimalarialagents, methotrexate, or mycophenolate mofetil. Intravenousimmunoglobulin not only benefits the muscle weakness butalso clears the skin lesions. Rituximab has been used for thetreatment of the skin changes, but results have been mixed.

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Red face with mixed connective tissue disease

Mixed connective tissue disease was first described in1972 as a disease syndrome with features that overlap thoseof systemic sclerosis, SLE, and polymyositis associated withantibodies to RNA-sensitive extractable nuclear antigen.31

The clinical manifestations of the separate diseases usuallydo not appear all at once and, therefore, red face is not oftenseen with mixed connective tissue disease. Commoncutaneous manifestations include erythematous plaques thatare similar to those of cutaneous LE (48%), swollen hands orsclerodactyly (50%), periungual telangiectasia (46%), alo-pecia (46%), dyspigmentation (28%), photosensitivity(28%), and vasculitis (22%).32 Facial erythema and facialtelangiectasias have also been reported.33 The therapeuticmeasures are similar to those used for the treatment of SLE.

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