What’s Your Diagnosis?
Patient Presentation
A 52-year-old Caucasian female presented with a 1-year history of papulosquamous plaques, chronic joint pain, and fatigue. On physical examination, there were multiple erythematous 5-cm to 10-cm polycyclic and annular patches and plaques on her arms, legs, abdomen and back. Some lesions were notable for a collarette scale and a few areas of post-inflammatory hyperpigmentation. There were no scalp, mucous membrane, or nail findings. Laboratory investigations, including a complete blood cell count and liver function tests, were within normal limits. Serology tests revealed positive anti-Ro antibody. Lyme titer, rapid plasma reagin and purified protein derivative tests were negative. A biopsy was performed to confirm the diagnosis. Patient’s review of systems was negative for photosensitivity, constitutional symptoms, mouth ulcers, dyspnea, as well as Raynaud’s phenomenon.
Diagnosis: Subacute Cutaneous Lupus Erythematosus
The term subacute cutaneous lupus erythematosus (SCLE) was coined by Gilliam and Sontheimer,1,2 who developed the classification system for cutaneous lupus erythematosus (LE), which includes acute cutaneous LE (ACLE), subacute cutaneous LE, and chronic discoid LE (CDLE) (see Table next page).1,3 Even though these are considered distinct subcategories of LE, patients with systemic lupus erythematosus (SLE) may present with any subtype of LE individually or concurrently.4 Although SLE and cutaneous LE share the same basic underlying disease process, patients with SLE show widespread internal as well as mucocutaneous involvement, whereas patients with cutaneous LE have the disease confined to the skin.
Children, adults, and the elderly may be affected by SCLE; however, it is mostly seen among Caucasian women with the mean age of onset in the fifth decade.3 Overall, there is a three to four times higher incidence of SCLE among women than in men.5
Clinical Features
SCLE initially presents as photodistributed, non-pruritic papulosquamous or annular polycyclic eruptions that can coalesce into large patches and plaques.6 The SCLE lesions are easily photoprovoked and can develop into thick hyperkeratotic plaques.5 Photosensitivity (52% to 85%), periungual telangiectasia (22% to 51%), discoid LE (19% to 35%), and vasculitis (12%) are some dermatological manifestations linked to SCLE.7 Associated systemic symptoms are mild and include arthritis/arthralgia (43% to 94%), renal disease (11% to 19%), serositis (12%), and central nervous system (CNS) symptoms (6% to 19%).7,8,9
Typically, SCLE eruptions are found on sun-exposed areas such as the face, neck, extensor arms, dorsal hands, and the lower limbs, but can also be seen on the abdominal trunk and scalp.5 The individual lesions may last for months and recur with intermittent periods of remission, making its course chronic and recurrent.7,10 A subtle gray hypopigmentation and telangiectasia are seen in the center of annular lesions, becoming more obvious as they resolve without scarring. The telangiectasia fades, but the hypopigmentation remains for years, generating unappealing leukoderma.7 Another possible complication, which was observed in our patient, includes temporary post-inflammatory hyperpigmentation.
The findings that can be attributed to SCLE include the following: elevated Ro(SS-A) and/or La(SS-B) antibodies; positive LE immunofluorescence band test in both uninvolved and lesional skin; low level of complement; an increase in IgG antibodies and erythrocyte sedimentation rate; symptomatic myalgia and phototesting that demonstrates persistence of erythema.11 In effect, SCLE is diagnosed using a combination of clinical, serologic and histological criteria.
Histopathology of SCLE
There are several histological features that may indicate the diagnosis of SCLE. Usually, the epidermal ridge pattern is effaced and hyperkeratosis tends to be mild, while atrophy is more marked.12,13 Parakeratosis and mild liquefactive degeneration of the basal layer may be the other epidermal features. Often, hair follicles are unaffected; however, slight keratin plugging may be seen. Basement membrane thickening is absent or at best minimal.12,13,14
In SCLE, satellite cell necrosis may present and lymphocytic exocytosis may be prominent to the point of subepidermal vesiculation.12,14,15,16 Homogenization of the papillary dermal collagen with mild mucin deposition is occasionally seen.12,14 Colloid body formation and pigmentary incontinence are often inconspicuous, but sometimes they are a major finding.12,13 The inflammatory cell tends to present as a lichenoid band; however, infiltrate is typically mild, superficially located and perivascular
in distribution.12,14,16,17
How does SCLE develop?
SCLE has been linked to HLA-DR3, QA1*0501, DQB1*0502 haplotypes. Gene polymorphisms in the interleukin (IL)-1 receptor antagonist and tumor necrosis factor-a (TNF-a) have also been implicated in SCLE. Ultraviolet (UV) light has demonstrated to be the most significant environmental trigger in cutaneous lupus erythematosus. Pathological photoprovocation reaction to UVA and UVB has been reported in 63% to 100% of SCLE patients.5
Exposure to medications such as the following may induce SCLE: hydro-chlorothiazide, calcium channel blockers, angiotensin-converting enzyme inhibitors, terbinafine, cinnarizine, gold therapy, piroxicam, d-penicillamine, sulfonylureas, oxprenolol, naproxen, spironolactone, griseofulvin, interferon-beta, and ranitidine.3,19,20 Infections by alphaviruses, sindbis, rubella, and cytomegalovirus demonstrate the ability to cause cell surface expression of Ro/SS-A and related autoantigens in cells undergoing virus-induced apoptosis.3 This observation has implicated a viral pathogenesis of SCLE. Since interplay of a number of predisposing genetic and environmental risk factors cause autoimmune diseases, it is common to associate SCLE development with multiple autoimmune disorders.20
Differential Diagnosis of SCLE
The differential diagnoses of SCLE that are important to consider include drug eruptions, dermatomyositis, secondary syphilis, polymorphous light eruptions, dermatophyte infections, nummular eczema, contact dermatitis, pityriasis rubra pilaris, disseminated superficial actinic porokeratosis, cutaneous T-cell lymphoma/mycosis fungoides, psoriasis, and seborrheic dermatitis.3,21 Systemic involvement must also be considered since patients with SLE exhibit similar findings to those with SCLE. Both ACLE and/or CDLE may occur in 15% to 20% of patients with SCLE, which makes the diagnosis more difficult.3 In addition, about half of the patients with SCLE meet the American Rheumatism Association criteria for the diagnostic classification of SLE.12
Managing SCLE: Standard Therapy
A practical approach to SCLE management encompasses a thorough clinical evaluation. Concomitant medications should be reviewed in order to assess whether any drug may be exacerbating or causing the lesions. Information on the smoking status of the patient is important, since smoking seems to worsen the disease or even lower the efficacy of anti-malarial medications indicated for the treatment of SCLE.19,22,23 Hence, smoking cessation or avoidance should be encouraged.
Patients should also be educated on the effects of the sun in relation to their disease; strongly recommended should be the utility of broad-spectrum sunscreens, sun-protective clothing, and lifestyle alterations as to limit sun exposure.
Initially, topical agents, such as corticosteroids, intralesional corticosteroids or retinoids should be used.19,23 However, if no response is noted, steps can then be taken to incorporate systemic anti-malarial agents (including hydroxychloroquine, chloroquine, and quinacrine) into the regimen.19,23 Feldmann et al observed adding quinacrine to hydroxycholoroquine resulted in 80% response rate in a group of SLE, DLE, and SCLE patients.24
Alternative Therapy
When standard therapy fails, patients should be questioned to determine if they are utilizing the therapy appropriately and gauge the level of compliance. If so, evidence obtained from a series of case reports shows that the use of thalidomide is a viable alternative to the standard therapy.25,26 In addition, isolated reports exist of various antibiotics such as cefuroxime axetil and sulfasalazine used to clear SCLE skin lesions.27,28 Moreover, auranofin, an oral form of gold, has also been utilized in SCLE with some therapeutic success.29 There are also anecdotal reports of using dapsone, retinoids (isotretinoin and acitretin), immunosuppressive/cytotoxic agents (azathioprine, methotrexate, mycophenolate mofetil, cyclosporine) and other systemic agents, intravenous immunoglobulin, and biological agents (TNF-a inhibitors, a-Interferon, chimeric CD4 monoclonal antibodies) to treat SCLE when standard therapy fails and the disease is recalcitrant.30-36 Since there is no standardized therapy for SCLE at present, future clinical assessment tools such as CLASI score proposed by Pelle will be a valuable measurement of treatment response of SCLE.37
Our patient’s antinuclear antibody (ANA) panel was positive for ANA and anti-Ro antibodies. Her skin biopsy revealed mild hyperkeratosis and vacuolar interface dermatitis confirmed the diagnosis of SCLE. She was initially managed with smoking cessation, sun avoidance measures, and topical corticosteroids with minimal improvement. Next, trials of antimalarials (plaquenil and chloroquine, individually) were attempted, but because the patient’s depression worsened with each, these medications were discontinued. Then, dapsone was given for 2 months without improvement. Finally, we treated her with methotrexate 10 mg/week, which resulted in complete resolution of her SCLE in several months.
What’s Your Diagnosis?
Patient Presentation
A 52-year-old Caucasian female presented with a 1-year history of papulosquamous plaques, chronic joint pain, and fatigue. On physical examination, there were multiple erythematous 5-cm to 10-cm polycyclic and annular patches and plaques on her arms, legs, abdomen and back. Some lesions were notable for a collarette scale and a few areas of post-inflammatory hyperpigmentation. There were no scalp, mucous membrane, or nail findings. Laboratory investigations, including a complete blood cell count and liver function tests, were within normal limits. Serology tests revealed positive anti-Ro antibody. Lyme titer, rapid plasma reagin and purified protein derivative tests were negative. A biopsy was performed to confirm the diagnosis. Patient’s review of systems was negative for photosensitivity, constitutional symptoms, mouth ulcers, dyspnea, as well as Raynaud’s phenomenon.
Diagnosis: Subacute Cutaneous Lupus Erythematosus
The term subacute cutaneous lupus erythematosus (SCLE) was coined by Gilliam and Sontheimer,1,2 who developed the classification system for cutaneous lupus erythematosus (LE), which includes acute cutaneous LE (ACLE), subacute cutaneous LE, and chronic discoid LE (CDLE) (see Table next page).1,3 Even though these are considered distinct subcategories of LE, patients with systemic lupus erythematosus (SLE) may present with any subtype of LE individually or concurrently.4 Although SLE and cutaneous LE share the same basic underlying disease process, patients with SLE show widespread internal as well as mucocutaneous involvement, whereas patients with cutaneous LE have the disease confined to the skin.
Children, adults, and the elderly may be affected by SCLE; however, it is mostly seen among Caucasian women with the mean age of onset in the fifth decade.3 Overall, there is a three to four times higher incidence of SCLE among women than in men.5
Clinical Features
SCLE initially presents as photodistributed, non-pruritic papulosquamous or annular polycyclic eruptions that can coalesce into large patches and plaques.6 The SCLE lesions are easily photoprovoked and can develop into thick hyperkeratotic plaques.5 Photosensitivity (52% to 85%), periungual telangiectasia (22% to 51%), discoid LE (19% to 35%), and vasculitis (12%) are some dermatological manifestations linked to SCLE.7 Associated systemic symptoms are mild and include arthritis/arthralgia (43% to 94%), renal disease (11% to 19%), serositis (12%), and central nervous system (CNS) symptoms (6% to 19%).7,8,9
Typically, SCLE eruptions are found on sun-exposed areas such as the face, neck, extensor arms, dorsal hands, and the lower limbs, but can also be seen on the abdominal trunk and scalp.5 The individual lesions may last for months and recur with intermittent periods of remission, making its course chronic and recurrent.7,10 A subtle gray hypopigmentation and telangiectasia are seen in the center of annular lesions, becoming more obvious as they resolve without scarring. The telangiectasia fades, but the hypopigmentation remains for years, generating unappealing leukoderma.7 Another possible complication, which was observed in our patient, includes temporary post-inflammatory hyperpigmentation.
The findings that can be attributed to SCLE include the following: elevated Ro(SS-A) and/or La(SS-B) antibodies; positive LE immunofluorescence band test in both uninvolved and lesional skin; low level of complement; an increase in IgG antibodies and erythrocyte sedimentation rate; symptomatic myalgia and phototesting that demonstrates persistence of erythema.11 In effect, SCLE is diagnosed using a combination of clinical, serologic and histological criteria.
Histopathology of SCLE
There are several histological features that may indicate the diagnosis of SCLE. Usually, the epidermal ridge pattern is effaced and hyperkeratosis tends to be mild, while atrophy is more marked.12,13 Parakeratosis and mild liquefactive degeneration of the basal layer may be the other epidermal features. Often, hair follicles are unaffected; however, slight keratin plugging may be seen. Basement membrane thickening is absent or at best minimal.12,13,14
In SCLE, satellite cell necrosis may present and lymphocytic exocytosis may be prominent to the point of subepidermal vesiculation.12,14,15,16 Homogenization of the papillary dermal collagen with mild mucin deposition is occasionally seen.12,14 Colloid body formation and pigmentary incontinence are often inconspicuous, but sometimes they are a major finding.12,13 The inflammatory cell tends to present as a lichenoid band; however, infiltrate is typically mild, superficially located and perivascular
in distribution.12,14,16,17
How does SCLE develop?
SCLE has been linked to HLA-DR3, QA1*0501, DQB1*0502 haplotypes. Gene polymorphisms in the interleukin (IL)-1 receptor antagonist and tumor necrosis factor-a (TNF-a) have also been implicated in SCLE. Ultraviolet (UV) light has demonstrated to be the most significant environmental trigger in cutaneous lupus erythematosus. Pathological photoprovocation reaction to UVA and UVB has been reported in 63% to 100% of SCLE patients.5
Exposure to medications such as the following may induce SCLE: hydro-chlorothiazide, calcium channel blockers, angiotensin-converting enzyme inhibitors, terbinafine, cinnarizine, gold therapy, piroxicam, d-penicillamine, sulfonylureas, oxprenolol, naproxen, spironolactone, griseofulvin, interferon-beta, and ranitidine.3,19,20 Infections by alphaviruses, sindbis, rubella, and cytomegalovirus demonstrate the ability to cause cell surface expression of Ro/SS-A and related autoantigens in cells undergoing virus-induced apoptosis.3 This observation has implicated a viral pathogenesis of SCLE. Since interplay of a number of predisposing genetic and environmental risk factors cause autoimmune diseases, it is common to associate SCLE development with multiple autoimmune disorders.20
Differential Diagnosis of SCLE
The differential diagnoses of SCLE that are important to consider include drug eruptions, dermatomyositis, secondary syphilis, polymorphous light eruptions, dermatophyte infections, nummular eczema, contact dermatitis, pityriasis rubra pilaris, disseminated superficial actinic porokeratosis, cutaneous T-cell lymphoma/mycosis fungoides, psoriasis, and seborrheic dermatitis.3,21 Systemic involvement must also be considered since patients with SLE exhibit similar findings to those with SCLE. Both ACLE and/or CDLE may occur in 15% to 20% of patients with SCLE, which makes the diagnosis more difficult.3 In addition, about half of the patients with SCLE meet the American Rheumatism Association criteria for the diagnostic classification of SLE.12
Managing SCLE: Standard Therapy
A practical approach to SCLE management encompasses a thorough clinical evaluation. Concomitant medications should be reviewed in order to assess whether any drug may be exacerbating or causing the lesions. Information on the smoking status of the patient is important, since smoking seems to worsen the disease or even lower the efficacy of anti-malarial medications indicated for the treatment of SCLE.19,22,23 Hence, smoking cessation or avoidance should be encouraged.
Patients should also be educated on the effects of the sun in relation to their disease; strongly recommended should be the utility of broad-spectrum sunscreens, sun-protective clothing, and lifestyle alterations as to limit sun exposure.
Initially, topical agents, such as corticosteroids, intralesional corticosteroids or retinoids should be used.19,23 However, if no response is noted, steps can then be taken to incorporate systemic anti-malarial agents (including hydroxychloroquine, chloroquine, and quinacrine) into the regimen.19,23 Feldmann et al observed adding quinacrine to hydroxycholoroquine resulted in 80% response rate in a group of SLE, DLE, and SCLE patients.24
Alternative Therapy
When standard therapy fails, patients should be questioned to determine if they are utilizing the therapy appropriately and gauge the level of compliance. If so, evidence obtained from a series of case reports shows that the use of thalidomide is a viable alternative to the standard therapy.25,26 In addition, isolated reports exist of various antibiotics such as cefuroxime axetil and sulfasalazine used to clear SCLE skin lesions.27,28 Moreover, auranofin, an oral form of gold, has also been utilized in SCLE with some therapeutic success.29 There are also anecdotal reports of using dapsone, retinoids (isotretinoin and acitretin), immunosuppressive/cytotoxic agents (azathioprine, methotrexate, mycophenolate mofetil, cyclosporine) and other systemic agents, intravenous immunoglobulin, and biological agents (TNF-a inhibitors, a-Interferon, chimeric CD4 monoclonal antibodies) to treat SCLE when standard therapy fails and the disease is recalcitrant.30-36 Since there is no standardized therapy for SCLE at present, future clinical assessment tools such as CLASI score proposed by Pelle will be a valuable measurement of treatment response of SCLE.37
Our patient’s antinuclear antibody (ANA) panel was positive for ANA and anti-Ro antibodies. Her skin biopsy revealed mild hyperkeratosis and vacuolar interface dermatitis confirmed the diagnosis of SCLE. She was initially managed with smoking cessation, sun avoidance measures, and topical corticosteroids with minimal improvement. Next, trials of antimalarials (plaquenil and chloroquine, individually) were attempted, but because the patient’s depression worsened with each, these medications were discontinued. Then, dapsone was given for 2 months without improvement. Finally, we treated her with methotrexate 10 mg/week, which resulted in complete resolution of her SCLE in several months.
What’s Your Diagnosis?
Patient Presentation
A 52-year-old Caucasian female presented with a 1-year history of papulosquamous plaques, chronic joint pain, and fatigue. On physical examination, there were multiple erythematous 5-cm to 10-cm polycyclic and annular patches and plaques on her arms, legs, abdomen and back. Some lesions were notable for a collarette scale and a few areas of post-inflammatory hyperpigmentation. There were no scalp, mucous membrane, or nail findings. Laboratory investigations, including a complete blood cell count and liver function tests, were within normal limits. Serology tests revealed positive anti-Ro antibody. Lyme titer, rapid plasma reagin and purified protein derivative tests were negative. A biopsy was performed to confirm the diagnosis. Patient’s review of systems was negative for photosensitivity, constitutional symptoms, mouth ulcers, dyspnea, as well as Raynaud’s phenomenon.
Diagnosis: Subacute Cutaneous Lupus Erythematosus
The term subacute cutaneous lupus erythematosus (SCLE) was coined by Gilliam and Sontheimer,1,2 who developed the classification system for cutaneous lupus erythematosus (LE), which includes acute cutaneous LE (ACLE), subacute cutaneous LE, and chronic discoid LE (CDLE) (see Table next page).1,3 Even though these are considered distinct subcategories of LE, patients with systemic lupus erythematosus (SLE) may present with any subtype of LE individually or concurrently.4 Although SLE and cutaneous LE share the same basic underlying disease process, patients with SLE show widespread internal as well as mucocutaneous involvement, whereas patients with cutaneous LE have the disease confined to the skin.
Children, adults, and the elderly may be affected by SCLE; however, it is mostly seen among Caucasian women with the mean age of onset in the fifth decade.3 Overall, there is a three to four times higher incidence of SCLE among women than in men.5
Clinical Features
SCLE initially presents as photodistributed, non-pruritic papulosquamous or annular polycyclic eruptions that can coalesce into large patches and plaques.6 The SCLE lesions are easily photoprovoked and can develop into thick hyperkeratotic plaques.5 Photosensitivity (52% to 85%), periungual telangiectasia (22% to 51%), discoid LE (19% to 35%), and vasculitis (12%) are some dermatological manifestations linked to SCLE.7 Associated systemic symptoms are mild and include arthritis/arthralgia (43% to 94%), renal disease (11% to 19%), serositis (12%), and central nervous system (CNS) symptoms (6% to 19%).7,8,9
Typically, SCLE eruptions are found on sun-exposed areas such as the face, neck, extensor arms, dorsal hands, and the lower limbs, but can also be seen on the abdominal trunk and scalp.5 The individual lesions may last for months and recur with intermittent periods of remission, making its course chronic and recurrent.7,10 A subtle gray hypopigmentation and telangiectasia are seen in the center of annular lesions, becoming more obvious as they resolve without scarring. The telangiectasia fades, but the hypopigmentation remains for years, generating unappealing leukoderma.7 Another possible complication, which was observed in our patient, includes temporary post-inflammatory hyperpigmentation.
The findings that can be attributed to SCLE include the following: elevated Ro(SS-A) and/or La(SS-B) antibodies; positive LE immunofluorescence band test in both uninvolved and lesional skin; low level of complement; an increase in IgG antibodies and erythrocyte sedimentation rate; symptomatic myalgia and phototesting that demonstrates persistence of erythema.11 In effect, SCLE is diagnosed using a combination of clinical, serologic and histological criteria.
Histopathology of SCLE
There are several histological features that may indicate the diagnosis of SCLE. Usually, the epidermal ridge pattern is effaced and hyperkeratosis tends to be mild, while atrophy is more marked.12,13 Parakeratosis and mild liquefactive degeneration of the basal layer may be the other epidermal features. Often, hair follicles are unaffected; however, slight keratin plugging may be seen. Basement membrane thickening is absent or at best minimal.12,13,14
In SCLE, satellite cell necrosis may present and lymphocytic exocytosis may be prominent to the point of subepidermal vesiculation.12,14,15,16 Homogenization of the papillary dermal collagen with mild mucin deposition is occasionally seen.12,14 Colloid body formation and pigmentary incontinence are often inconspicuous, but sometimes they are a major finding.12,13 The inflammatory cell tends to present as a lichenoid band; however, infiltrate is typically mild, superficially located and perivascular
in distribution.12,14,16,17
How does SCLE develop?
SCLE has been linked to HLA-DR3, QA1*0501, DQB1*0502 haplotypes. Gene polymorphisms in the interleukin (IL)-1 receptor antagonist and tumor necrosis factor-a (TNF-a) have also been implicated in SCLE. Ultraviolet (UV) light has demonstrated to be the most significant environmental trigger in cutaneous lupus erythematosus. Pathological photoprovocation reaction to UVA and UVB has been reported in 63% to 100% of SCLE patients.5
Exposure to medications such as the following may induce SCLE: hydro-chlorothiazide, calcium channel blockers, angiotensin-converting enzyme inhibitors, terbinafine, cinnarizine, gold therapy, piroxicam, d-penicillamine, sulfonylureas, oxprenolol, naproxen, spironolactone, griseofulvin, interferon-beta, and ranitidine.3,19,20 Infections by alphaviruses, sindbis, rubella, and cytomegalovirus demonstrate the ability to cause cell surface expression of Ro/SS-A and related autoantigens in cells undergoing virus-induced apoptosis.3 This observation has implicated a viral pathogenesis of SCLE. Since interplay of a number of predisposing genetic and environmental risk factors cause autoimmune diseases, it is common to associate SCLE development with multiple autoimmune disorders.20
Differential Diagnosis of SCLE
The differential diagnoses of SCLE that are important to consider include drug eruptions, dermatomyositis, secondary syphilis, polymorphous light eruptions, dermatophyte infections, nummular eczema, contact dermatitis, pityriasis rubra pilaris, disseminated superficial actinic porokeratosis, cutaneous T-cell lymphoma/mycosis fungoides, psoriasis, and seborrheic dermatitis.3,21 Systemic involvement must also be considered since patients with SLE exhibit similar findings to those with SCLE. Both ACLE and/or CDLE may occur in 15% to 20% of patients with SCLE, which makes the diagnosis more difficult.3 In addition, about half of the patients with SCLE meet the American Rheumatism Association criteria for the diagnostic classification of SLE.12
Managing SCLE: Standard Therapy
A practical approach to SCLE management encompasses a thorough clinical evaluation. Concomitant medications should be reviewed in order to assess whether any drug may be exacerbating or causing the lesions. Information on the smoking status of the patient is important, since smoking seems to worsen the disease or even lower the efficacy of anti-malarial medications indicated for the treatment of SCLE.19,22,23 Hence, smoking cessation or avoidance should be encouraged.
Patients should also be educated on the effects of the sun in relation to their disease; strongly recommended should be the utility of broad-spectrum sunscreens, sun-protective clothing, and lifestyle alterations as to limit sun exposure.
Initially, topical agents, such as corticosteroids, intralesional corticosteroids or retinoids should be used.19,23 However, if no response is noted, steps can then be taken to incorporate systemic anti-malarial agents (including hydroxychloroquine, chloroquine, and quinacrine) into the regimen.19,23 Feldmann et al observed adding quinacrine to hydroxycholoroquine resulted in 80% response rate in a group of SLE, DLE, and SCLE patients.24
Alternative Therapy
When standard therapy fails, patients should be questioned to determine if they are utilizing the therapy appropriately and gauge the level of compliance. If so, evidence obtained from a series of case reports shows that the use of thalidomide is a viable alternative to the standard therapy.25,26 In addition, isolated reports exist of various antibiotics such as cefuroxime axetil and sulfasalazine used to clear SCLE skin lesions.27,28 Moreover, auranofin, an oral form of gold, has also been utilized in SCLE with some therapeutic success.29 There are also anecdotal reports of using dapsone, retinoids (isotretinoin and acitretin), immunosuppressive/cytotoxic agents (azathioprine, methotrexate, mycophenolate mofetil, cyclosporine) and other systemic agents, intravenous immunoglobulin, and biological agents (TNF-a inhibitors, a-Interferon, chimeric CD4 monoclonal antibodies) to treat SCLE when standard therapy fails and the disease is recalcitrant.30-36 Since there is no standardized therapy for SCLE at present, future clinical assessment tools such as CLASI score proposed by Pelle will be a valuable measurement of treatment response of SCLE.37
Our patient’s antinuclear antibody (ANA) panel was positive for ANA and anti-Ro antibodies. Her skin biopsy revealed mild hyperkeratosis and vacuolar interface dermatitis confirmed the diagnosis of SCLE. She was initially managed with smoking cessation, sun avoidance measures, and topical corticosteroids with minimal improvement. Next, trials of antimalarials (plaquenil and chloroquine, individually) were attempted, but because the patient’s depression worsened with each, these medications were discontinued. Then, dapsone was given for 2 months without improvement. Finally, we treated her with methotrexate 10 mg/week, which resulted in complete resolution of her SCLE in several months.
