Drug-induced SCLE is more common than in other subtypes, with terbinafine, tumour necrosis factor- inhibitors, antiepileptics, and proton pump inhibitors the most frequently reported culprits found in a 2012 population-based match case control study [26]. patients with CLE who progress to SLE typically meet the mildest of SLE criteria [5]. In 2012, the Systemic Lupus International Collaborating Clinics Classification Criteria (SLICC) was proposed as an updated method for diagnosing SLE, including the revised dermatologic criteria of ACLE, CCLE, oral ulcers, and nonscarring alopecia. This validated SLICC criteria is undergoing further comparative testing with the ACR SLE criteria in various populations [6]. In addition, there TC-DAPK6 is ongoing controversy over the classification of the cutaneous manifestations of LE from a dermatologic vantage point. Gilliam proposed a classification system that separated LE-specific lesions from LE-nonspecific lesions, based on histopathology. The various morphologies of CLE fall under the umbrella of LE-specific lesions, including acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), and chronic cutaneous LE (CCLE). CCLE encompasses discoid LE (DLE), LE profundus (LEP), chilblain LE (CHLE), and LE tumidus (LET) [7]. The Duesseldorf Classification in 2004 proposed a separate category for LET, entitled intermittent cutaneous LE (ICLE), although this division is not universally accepted [8]. LE-nonspecific lesions, on the other hand, include findings that are not characteristic of, but are frequently seen in SLE. Such lesions include Raynauds phenomenon, periungual telangiectasias, livedo reticularis, and leukocytoclastic vasculitis. C. How can we differentiate the CLE subtypes? a. Acute Cutaneous Lupus Erythematosus Acute cutaneous LE typically presents in the third decade of life and is frequently associated with active SLE [9] and [10]. There are localized and generalized forms of ACLE. The localized form is the frequently described malar, or butterfly rash, which refers to erythema that occurs over both cheeks, extends over the nasal bridge, and spares the nasolabial folds [11]. These lesions are classically transient, sun-induced, and non-scarring, although dyspigmentation can occur [12]. Patients may initially mistake this rash for a sunburn, and only seek medical attention when it persists for several days. A fine surface scale and/or edema may be associated with the erythema. Malar rashes have been reported to be present in up to 52% of SLE patients at the time of diagnosis, with clinical activity of the rash paralleling that of the systemic disease. This rash can be confused with acne rosacea and seborrheic dermatitis, TC-DAPK6 however the former is usually associated with the formation of papules and pustules, and the latter occurs within the nasolabial folds [13]. The more rare generalized form occurs above and below the neck, and has been referred to as a maculopapular rash of lupus or photosensitive lupus dermatitis. This presents as an often pruritic, widespread eruption of symmetric macules and papules that is photosensitive and may resemble a drug rash. Patients may have associated mucosal ulcerations/apthae, as well as diffuse hair thinning [14]. Generalized ACLE may resemble dermatomyositis as both diseases involve the dorsum of the hands, however, Rabbit polyclonal to OPG dermatomyositis affects the distal interphalangeal, proximal interphalangeal, and metacarpophalangeal joints, while they are spared in ACLE [13]. Cuticular overgrowth, as well as erythema or dilated vessels and drop-out of vessels in the periungual area are frequently seen. Lesions resembling erythema multiforme in ACLE or SCLE patients have been termed Rowells syndrome [15]. Rarely, a severe acute form can resemble toxic epidermal necrolysis. Other differentials include drug-induced photosensitivity, pemphigus erythematosus, atopic dermatitis, contact dermatitis, and photocontact dermatitis. Histologically, ACLE lesions show liquefactive degeneration of the basal layer, edema of the upper dermis, and a scattered interface, perivascular, and periadnexal lymphocytic infiltrate, all of which are generally less pronounced as compared to other CLE subtypes. Immunologically, a positive ANA is found in 95% of ACLE patients, as well as a high incidence of anti-dsDNA and anti-Sm antibodies [16]. Lesional direct immunofluorescence reveals granular immune deposits at the dermal-epidermal junction and perivascular deposits in the upper dermis, most commonly IgM [9]. b. Subacute Cutaneous Lupus Erythematosus As with SLE, Subacute Cutaneous Lupus Erythematosus (SCLE) occurs primarily in young to middle aged women [11]. SCLE is highly photosensitive, with 70-90% of patients meeting the ACR definition of abnormal photosensitivity [17]. There are two morphologic variants of SCLE: annular and papulosquamous. A study of 58 SCLE patients found that 42% had annular SCLE and 39% exhibited papulosquamous SCLE, while 16% of patients showed features of both [18]. Other studies have found more.In a 12-week cross-over study of 78 DLE patients, excellent improvement or resolution of lesions was seen in 27% of patients treated with fluocononide 0.05% cream, as compared to 10% of patients treated with hydrocortisone 1% cream at 6 weeks. with appropriate topical and systemic brokers. Systemic brokers are indicated in cases of widespread, scarring, or treatment-refractory disease. In this review, we discuss issues in classification and diagnosis of the various subtypes of CLE, as well as provide an update on therapeutic management. observed that patients with CLE who progress to SLE typically meet the mildest of SLE criteria [5]. In 2012, the Systemic Lupus International Collaborating Clinics Classification Criteria (SLICC) was proposed as an updated method for diagnosing SLE, including the revised dermatologic criteria of ACLE, CCLE, oral ulcers, and nonscarring alopecia. This validated SLICC criteria is undergoing further comparative testing with the ACR SLE criteria in various populations [6]. In addition, there is ongoing controversy over the classification of the cutaneous manifestations of LE from a dermatologic vantage point. Gilliam proposed a classification system that separated LE-specific lesions from LE-nonspecific lesions, based on histopathology. The various morphologies of CLE fall under the umbrella of LE-specific lesions, including acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), and chronic cutaneous LE (CCLE). CCLE encompasses discoid LE (DLE), LE profundus (LEP), chilblain LE (CHLE), and LE tumidus (LET) [7]. The Duesseldorf Classification in 2004 proposed a separate category for LET, entitled intermittent cutaneous LE (ICLE), although this division is not universally accepted [8]. LE-nonspecific lesions, on the other hand, include findings that are not characteristic of, but are frequently seen in SLE. Such lesions include Raynauds phenomenon, periungual telangiectasias, livedo reticularis, and leukocytoclastic vasculitis. C. How can we differentiate the CLE subtypes? a. Acute Cutaneous Lupus Erythematosus Acute cutaneous LE typically presents in the third decade of life and is frequently associated with active SLE [9] and [10]. There are localized and generalized forms of ACLE. The localized form is the frequently described malar, or butterfly rash, which refers to erythema that occurs over both cheeks, extends over the nasal bridge, and spares the nasolabial folds [11]. These lesions are classically transient, sun-induced, and non-scarring, although dyspigmentation can occur [12]. Patients may initially mistake this rash for a sunburn, and only seek medical attention when it persists for several days. A fine surface scale and/or edema may be associated with the erythema. Malar rashes have been reported to be present in up to 52% of SLE patients at the time of diagnosis, with clinical activity of the rash paralleling that of the systemic disease. This rash can be confused with acne rosacea and seborrheic dermatitis, however the former is associated with the formation of papules and pustules, and the latter occurs within the nasolabial folds [13]. The more rare generalized form occurs above and below the throat, and continues to be known as a maculopapular rash of lupus or photosensitive lupus dermatitis. This presents as an frequently pruritic, wide-spread eruption of symmetric macules and papules that’s photosensitive and could resemble a medication rash. Individuals may have connected mucosal ulcerations/apthae, aswell as diffuse thinning hair [14]. Generalized ACLE look like dermatomyositis as both illnesses involve the dorsum from the hands, nevertheless, dermatomyositis impacts the distal interphalangeal, proximal interphalangeal, and metacarpophalangeal bones, while they may be spared in ACLE [13]. Cuticular overgrowth, aswell as erythema or dilated vessels and drop-out of vessels in the periungual region are frequently noticed. Lesions resembling erythema multiforme in ACLE or SCLE individuals have already been termed Rowells symptoms [15]. Hardly ever, a severe severe type can resemble poisonous epidermal necrolysis. Additional differentials consist of drug-induced photosensitivity, pemphigus erythematosus, atopic dermatitis, get in touch with dermatitis, and photocontact dermatitis. Histologically, ACLE lesions display liquefactive degeneration from the basal coating, edema from the top dermis, and a spread user interface, perivascular, and periadnexal lymphocytic infiltrate, which are generally much less pronounced when compared with additional CLE subtypes. Immunologically, an optimistic ANA is situated in 95% of ACLE individuals, and a high occurrence of anti-dsDNA and anti-Sm antibodies [16]. Lesional immediate immunofluorescence reveals granular immune system debris in the dermal-epidermal junction and perivascular TC-DAPK6 debris in the top dermis, mostly IgM [9]. b. Subacute Cutaneous Lupus Erythematosus Much like SLE, Subacute Cutaneous Lupus Erythematosus (SCLE) happens primarily in youthful to middle aged ladies [11]..