Clinics in Dermatology (2014) 32, 153–158
The red face revisited: Connective tissue disorders Jana Kazandjieva, MD, PhD a,⁎, Nikolai Tsankov, MD, PhD b , Kyrill Pramatarov, MD, PhD c a
Department of Dermatology and Venereology, Medical University, Sofia, Bulgaria Department of Dermatology and Venereology, Tokuda Hospital, Sofia, Bulgaria c Department of Dermatology, Medical University-Sofia, University Hospital Lozenez, Sofia, Bulgaria b
Abstract Red face is not a rare finding in patients with connective tissue disorders. The malar eruption is the most frequent cutaneous manifestation of systemic lupus erythematosus (LE). This condition is more apparent among fair-skinned individuals, and it usually appears after sun exposure. A very important clinical sign 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 clinical findings of chronic cutaneous LE are the discoid lesions. There is a clinical form of chronic cutaneous LE called erythema perstans faciei. This form is purely erythematous, and it usually appears on the face. Other rare “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 with CREST (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. Many studies mention telangiectasias as markers of the severity of the systemic sclerosis, the disease duration, any pulmonary 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 for the diagnosis of dermatomyositis. This violaceous to dusky erythema can extend over the whole face and the upper aspects of the trunk. Erythematous changes on the face that are different from those of the heliotrope sign which occurs with dermatomyositis may be observed in both sun-exposed skin and non–sun-exposed skin. Malar and facial erythema, linear extensor erythema, V-sign or shawl sign, and other photodistributed eruptions 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-nonspecific findings. Lupus-specific skin changes occur with chronic
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(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 the classical “butterfly eruption.”2
Systemic lupus erythematosus Systemic LE (SLE) involves cutaneous changes that are listed in the criteria established by the American Rheumatology Association.3 These include malar eruption, discoid lesions, photosensitivity, and oral mucosal lesions.
154 The malar eruption is the most frequent cutaneous manifestation of SLE. It is an erythematous and very slightly edematous lesion that is located on both cheeks and across the bridge of the nose. It is more obvious among affected fair-skinned individuals, and it usually appears after sun exposure. These lesions will then disappear, and they leave no scars or pigmentation. Malar eruption may be mistaken for sunburn, the flush of rosacea, or photosensitive dermatitis. If the erythema persists for a few weeks or if new erythema appears elsewhere, the true diagnosis is the malar eruption of SLE. A very important clinical sign is that the nasolabial folds remain free of any skin changes; this is the main clinical differentiation between malar eruption and seborrheic dermatitis. The differentiation of the malar eruption sign of SLE from other connective tissue diseases may be very difficult and sometimes impossible. Clinical differential diagnoses include—in addition other conditions—telangiectatic rosacea, superficial fungal infections, photosensitive dermatitis, erythema emotivum, rubeosis diabeticorum, and the eruption of some viral infections. The histological findings depend on the type of clinical representation. The main pathologic findings are thinning of the epidermis; hydropic degeneration of the basal layer with disruption of the dermoepidermal junction; edema of the dermis in upper portion; sparse infiltration of lymphocytes in most of the dermis; and fibrinoid degeneration of the connective tissue. With direct immunofluorescence, deposits of immunoglobulins G and M, as well as of C3, are detected at the basal membrane zone; these deposits are found both in lesions and in uninvolved skin. The histopathologic findings are compatible with both SLE and dermatomyositis, but direct immunofluorescence is negative in patients with dermatomyositis.
Subacute cutaneous lupus erythematosus Patients with subacute cutaneous LE have disseminated, nonscarring, photodistributed eruptions. There are two major subtypes of the disease: erythematous annular, which resembles erythema annulare centrifugum, and erythematous squamous, which resembles psoriasis or lichen planus. Subacute cutaneous LE belongs to the group of well-defined photodermatoses. Sun exposure can provoke a red face that resembles the malar eruption of SLE,4 and it may also provoke systemic involvement in some patients. This condition also involves a differential 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. The typical lesions of this condition are the discoid lesions. Because these lesions are the most common skin changes, sometimes the term chronic discoid LE is used as a synonym for chronic cutaneous LE. The discoid lesions begin with red papules or maculae that are covered with silvery scales. A closer view of
J. Kazandjieva et al. the scales shows follicular keratosis, which is a very important clinical finding for this disease. Later, atrophy and scarring appear after the active stage of the disease. The histology of chronic cutaneous LE includes the following findings: atrophic epidermis, compact hyperkeratosis with follicular plugging, and hydropic degeneration of the cells of the basal layer. In the upper portion of the dermis, there are edema and mucin; during the later stages of the disease, sclerosis, dilatation of the blood vessels, and dense lymphocytic infiltrate around the vessels of the upper and deep vessels and the adnexa are also present. With the use of direct immunofluorescence, deposits of immunoglobulins G and M 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 called erythema perstans faciei. This form is purely erythematous, and it usually appears on the face. The lesions are erythematous macules or plaques with little scaling and very little edema. There is a lack of severe inflammation, and the pathologic process is superficial. The lesions disappear with minimal atrophy. The acute stage of these lesions resembles the malar eruption of SLE, and it is very difficult to differentiate between these conditions.6 Some rare variants of chronic cutaneous LE present the clinical picture of a red face. LE edematosus has been described as erythematous and edematous, without well-demarcated plaques, follicular plugging, or scarring.7 The lesions are sometimes multiple, and they regress without residual changes. The diagnosis may be difficult because the histological and immunologic findings are not always compatible with chronic cutaneous 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-exposed areas.8 Follicular plugging and scarring are usually absent. The lesions can be widespread; they affect all photoexposed areas (ie, back, neck, arms, and shoulders), but they occupy the face predominantly. They sometimes disappear spontaneously, but they can also coalesce to form annular plaques that 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 the spectrum of LE, and Ro/SSA antibodies are detected in all patients. Due to the peculiarities of the clinical and histopathological findings, this variant needs to be differentiated from polymorphous light eruption and Jessner lymphocytic infiltration. Additional differentiation from reticular erythematous mucinosis is also required. LE telangiectaticus is another rare form of chronic cutaneous LE (Figure 2). The most important clinical feature of this variant is erythema. A closer view reveals that this erythema is caused by telangiectasias. The lesions involve the face, and they can also occur on the extremities and the back. The telangiectasias are usually reticulate, 9 and follicular plugging and scarring are usually absent. The most important clinical differential diagnosis of this subtype
Connective tissue disorders
Fig. 1 LE tumidus. From Pramatarov K. Chronic cutaneous lupus erythematosus—Clinical spectrum. Clin Dermatol. 2004;22:113–120.
is rosacea. Sometimes, prominent hemorrhagic changes are one of the most common clinical signs of the type of chronic cutaneous LE that presents with multiple petechiae (Figure 3). The initial skin changes of this variant, which is called LE hemorrhagicus, resemble an acute photosensitive reaction. It should be noted that similar telangiectasias have been observed
Fig. 2 LE telangiectaticus. From Pramatarov K. Chronic cutaneous lupus erythematosus—Clinical spectrum. Clin Dermatol. 2004;22:113–120.
Fig. 3 LE hemorrhagicus. From Pramatarov K. Chronic cutaneous lupus erythematosus—Clinical spectrum. Clin Dermatol. 2004;22:113–120.
in patients with other connective tissue diseases (eg, systemic sclerosis, rheumatoid arthritis, dermatomyositis, and undifferentiated connective tissue diseases). Chilblain lupus is another form of this disease, and it is most common in the northern countries. The typical lesions associated with this condition are acrocyanotic, and they resemble perniones (Figure 4). They are localized on the skin of the face and predominantly on the nose and the ears. Skin
Fig. 4 Chilblain lupus. From Pramatarov K. Chronic cutaneous lupus erythematosus—Clinical spectrum. Clin Dermatol. 2004;22: 113–120.
156 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 the disease appears during the winter and resolves during the summer months. The chilblain lesions occur after the development of the discoid lesions; when the latter disappear after treatment, however, the chilblain lesions persist. In half of the patients with chilblain lupus, the perniotic lesions are the only clinical sign of the disease; it is quite possible that these patients develop systemic involvement later.5,10 The main differential diagnoses of chilblain lupus are lupus pernio (a form of sarcoidosis) and acrocyanosis. LE profundus is a subtype of LE. It can be a clinical manifestation of chronic cutaneous LE and also of SLE. LE profundus was probably first described by Kaposi in 1883. In 1940, Irgang reviewed the cases reported by Kaposi and named this clinical entity. There is still some controversy about the use of the term LE profundus. This condition is sometimes referred to as LE panniculitis (as a synonym for LE profundus), whereas other times this name is used when LE profundus is a clinical sign of SLE. LE profundus is characterized by deep inflammatory nodules that resemble those of panniculitis. The nodules are located in the deep dermis and in the hypodermis. LE profundus most commonly affects the cheeks, but it can also affect the rest of the face (especially the eyelids), the arms, the hands, the breasts, the buttocks, the trunk, and the legs. The size of the lesions varies from 1 cm to several centimeters in diameter. The overlying skin is normal or red, or it may show the changes of discoid LE. Local trauma often plays a role as a provoking factor. Longstanding lesions are atrophic, and clinically they form typical depressions of the skin. A case of LE profundus with periorbital swelling has been reported.11 The periorbital swelling may be the first clinical sign of LE profundus, and it may persist during the course of the 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. The acronym CREST stands for calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia; it is considered a rare form of systemic sclerosis. CREST syndrome was first reported by dermatologist George Thibierge and rheumatologist Raymond Weissenbach in 1910.12 They described the occurrence of subcutaneous calcification in patients with scleroderma and emphasized that this was not a coincidental finding but rather a causal link.13 The diagnosis of CRST (calcinosis, Raynaud phenomenon, sclerodactyly, and facial and truncal telangiectasiae) was coined in 1964 by Winterbauer.14,15 That author was the first to mention telangiectasias as a part of the syndrome, which he described in a series of eight patients. In
J. Kazandjieva et al.
Facial teleangiectasias in systemic sclerosis.
1979, CRST was lengthened to CREST by Shulman and colleagues, who added esophageal involvement to the cardinal manifestations.16 Antinuclear antibodies, recognized as chromosomal centromere proteins, were later reported as a characteristic sign that was present in more than 50% of cases. Other manifestations include arthralgias, pulmonary hypertension (late finding), and primary biliary cirrhosis. Many authors have used the term limited cutaneous systemic sclerosis rather than CREST syndrome, when referring to possible systemic involvement.17 A histologic examination of telangiectasias in the setting of limited scleroderma was made.18 The investigation revealed dilated postcapillary venules located in the papillary and superficial reticular dermis. The vessel walls consisted of nonfenestrated endothelial cells surrounded by a variable number of pericytes and smooth muscle cells. According to the study, the immunohistological and ultrastructural features of the lining endothelium of established telangiectasias in patients with longstanding limited scleroderma appeared to be benign. Another study looked at endoglin levels in the serum of patients with limited cutaneous SSc.19 The investigators’ data showed that patients with elevated serum endoglin levels had telangiectasia more frequently as compared with those patients with normal levels. According to the study, serum endoglin levels were significantly elevated in these patients as compared with the levels found in those with diffuse cutaneous SSc and SLE. A proposed classification system separated the scleroderma spectrum disorders into six different categories. 20 According to this categorization, CREST syndrome is type
Connective tissue disorders VI. An exact diagnosis requires telangiectasia with one or more other CREST base criteria or anticentromere antibodies with any two or more criteria. Contrary to the commonly accepted opinion, telangiectasias are often present as part of the clinical picture of SSc. In a study of 120 patients with progressive systemic sclerosis, the incidence of telangiectasias was calculated to be 75% to 100%.21 According to the investigators, the ramosus and telangiectatic mats types of telangiectasias are typical for SSc. The ramosus type mainly appears on the face, neck, and chest, whereas the telangiectatic mats type usually involves the upper extremities and is the only type that appears at the lips. Cuticular telangiectasia is a third type; this is as important as the others but less frequent. Many studies mention telangiectasias as a marker for the severity of SSc. The incidence of telangiectasias is believed to be related to disease duration,12 and a significant association between pulmonary arterial hypertension in patients with scleroderma and their number of telangiectases has been observed.22 A Japanese study concluded that the presence of telangiectasia may be a marker of esophageal involvement, decreased carbon monoxide diffusion capacity, and heart involvement.23 Nine criteria with the abbreviation ABCDCREST have been proposed: (1) autoantibodies to centromere proteins; (2) bibasilar pulmonary fibrosis; (3) contractures of the digital joints; (4) dermal thickening proximal to the wrists; (5) calcinosis cutis; (6) Raynaud phenomenon; (7) esophageal distal hypomotility; (8) sclerodactyly; and (9) telangiectasias.24 The presence of three or more of these criteria indicates definite systemic scleroderma. The differential diagnosis with other red face manifestations is easy because of the additional findings. Telangiectasias are accompanied by calcinosis (confirmed with plain radiographs), sclerodactyly, digital ischemia, infarction secondary to Raynaud disease, or some combination of these. A biopsy is required to make the diagnosis of Barrett esophagitis or adenocarcinoma. Pulsed-dye laser treatment has been shown to be effective for the treatment of facial telangiectasia.25 This treatment modality has been studied in patients with SSc but not in those with CREST syndrome.26 Many methods have been successfully used to treat symptomatic gastrointestinal telangiectasia (eg, medical treatment with estrogen and progesterone or desmopressin; laser ablation; sclerotherapy). Bowel resection for uncontrollable gastrointestinal bleeding as a result of telangiectasia is rarely necessary.
157 first clinical signs to be helpful for the diagnosis of dermatomyositis. The term heliotrope sign refers to the flower Heliotropium peruvianum and its specific purplish petals.28 This violaceous to dusky erythema can extend over the whole face and the upper portion of the trunk. Among patients with darker skin types (ie, types III through VI), erythema can be subtle and overlooked. The heliotrope eruption is often associated with periorbital edema and telangiectasias on the upper eyelids (Figure 6). In those with chronic dermatomyositis, hypopigmentation or hyperpigmentation may appear at the site of the erythema. Poikiloderma in a photosensitive distribution is a possible finding in some cases; sun exposure usually aggravates the eruption. According to some studies in those with juvenile dermatomyositis, the heliotrope eruption and Gottron papules appeared less frequently.29 Erythematous changes of the face that differ from those of the heliotrope sign among patients with dermatomyositis may be observed in both sun-exposed skin and non–sunexposed skin. Malar and facial erythema, linear extensor erythema, V-sign or shawl sign, and other photodistributed eruptions can appear. A confluent erythema in the malar distribution that involves the cheeks and that extends over the nasal bridge may be seen. Nasolabial folds are often spared with dermatomyositis, which differs from what is seen with SLE.30 Widespread erythema that affects the perioral area, the forehead, and the lateral face and ears can be noticed. Erythema often involves the cartilaginous portion of the helix of the ear, with sparing of the earlobe. The eruption may spread over the neck, the anterior chest (Vsign), or the back and shoulders (shawl sign). Often erythematous lesions are accompanied by secondary changes, such as scaling, crusting, or erosion. The skin changes are treated by avoiding sun exposure and by using sunscreens, topical corticosteroids, antimalarial agents, methotrexate, or mycophenolate mofetil. Intravenous immunoglobulin not only benefits the muscle weakness but also clears the skin lesions. Rituximab has been used for the treatment of the skin changes, but results have been mixed.
Red face with dermatomyositis Periorbital erythema is included in the set of the five criteria necessary for the diagnosis of myositis (together with progressive proximal symmetrical weakness, elevated levels of muscle enzymes, an abnormal finding on electromyography, and an abnormal finding on muscle biopsy).27 Purple or violet-colored upper eyelids are the hallmark of and one of the
Periorbital edema and telangiectasias in dermatomyositis.
Red face with mixed connective tissue disease Mixed connective tissue disease was first described in 1972 as a disease syndrome with features that overlap those of systemic sclerosis, SLE, and polymyositis associated with antibodies to RNA-sensitive extractable nuclear antigen.31 The clinical manifestations of the separate diseases usually do not appear all at once and, therefore, red face is not often seen with mixed connective tissue disease. Common cutaneous manifestations include erythematous plaques that are similar to those of cutaneous LE (48%), swollen hands or sclerodactyly (50%), periungual telangiectasia (46%), alopecia (46%), dyspigmentation (28%), photosensitivity (28%), and vasculitis (22%).32 Facial erythema and facial telangiectasias have also been reported.33 The therapeutic measures are similar to those used for the treatment of SLE.
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