Patient Presentation
A 53-year-old African-American male presented with a persistent pruritic eruption on his upper body. The patient described intense pruritus on his head, face, arms and hands that caused multiple excoriations on his arms and hands. Sunlight intensified the pruritus. The patient was clothed in heavy material including gloves, jacket and a mask to protect him from the sun.
The patient noted that pruritic lesions first appeared years earlier when he worked in a textile mill. He was exposed to chemicals at the mill, which he believed to be responsible for the lesions on his arms. His condition worsened as he worked as a day laborer in the fields of Georgia. He had been seen by many dermatologists and received multiple courses of corticosteroids, oral and topical, without benefit. Multiple biopsies were performed at various medical centers. He denied any history of connective tissue diseases and previous testing (ANA, anti-Ro, anti-La) were normal.
Physical examination revealed diffuse xerosis, lichenification and alopecia involving the face, neck, upper chest, arms and hands. His legs, abdomen, back, shoulders and submental region were spared. The patient had liberally applied white petrolatum to all areas of lichenification. Multiple excoriations in different stages of healing were present on the hands and forearms.
Complete blood cell count, complete metabolic panel, liver function tests, renal function tests, thyroid stimulating hormone, HIV and rapid plasma reagin testing and urine and stool porphyrins were normal. Skin biopsy revealed a superficial dermal perivascular lymphohistiocytic infiltrate consistent with actinic reticuloid.
What is Your Diagnosis?
Diagnosis: Actinic Reticuloid (Chronic Actinic Dermatitis)
Actinic reticuloid, or chronic actinic dermatitis (CAD), describes a group of serious persistent photodermatoses. In 1961, Wilkinson described a photodermatosis as a “persistent light reaction”.1
A variety of terminology is used to describe this photodermatosis, including photosensitive eczema, chronic photosensitive dermatitis, persistent light reactivity and actinic reticuloid. These terms occur along a continuum of disease.2 In order to resolve this, chronic actinic dermatitis (CAD) has been proposed to encompass all these entities.2,3 Actinic reticuloid represents the most severe form of CAD. The histologic features of actinic reticuloid have been described as resembling lymphoma.4 Although many hypotheses for the pathologic basis of CAD have been proposed, no consensus exists. Like-wise, a consensus on treatment of such a severe disease is also lacking.
Clinical Presentation
Chronic actinic dermatitis occurs in multiple races.5,6 In all races, most patients tend to be middle-aged or elderly.
The condition generally begins with persistent erythema of sun-exposed areas. Lesions are characterized by pruritus and worsening with sun exposure. As the condition progresses, lichenification occurs; however, papules and plaques may be seen.7 Cutaneous edema and vesicles may also be present. Severe pruritus of the affected area is a hallmark of the disease process. Alopecia may occur as a consequence of prolonged and vigorous rubbing. Large areas of skin may be hypo- or hyperpigmented.6
Evolution of this disease may be linked to the patient’s work outdoors and thus to the degree of ultraviolet light exposure. However, despite decreased sun exposure, severe disease can persist during winter months.5,8
Histologic examination of early CAD may resemble contact dermatitis.5 In later stages with lichenification, the histologic pattern may resemble pseudolymphoma.4 The dermal infiltrate consists primarily of suppressor T cells.9 A constellation of changes has been described in the dermis and epidermis but these changes generally represent prolonged rubbing of the skin.
Diagnosis
The diagnosis of CAD can be challenging. Several factors add to the frustration with this diagnosis including confusing terminology, indistinct histopathology and clinical variability. CAD is rare when compared to other photodermatoses affecting the elderly.10 A diagnostic criterion proposed in the past includes persistent facial erythema, a middle-aged or elderly patient and severe photosensitivity with a broad action spectrum for at least 1 year.5 However, the diagnosis of CAD is made with a constellation of clinical features, histologic findings and phototest abnormalities.8
Clinical history of light sensitivity or photosensitivity caused by a photoallergen is most important in establishing a diagnosis of CAD. Phototesting is useful to confirm the diagnosis.11 Patch and photopatch tests are essential ancillary tests.6 Many studies have indicated a contact allergic source for CAD including plants and chemicals.5 As such, allergic testing may be done to aid the patient in allergen avoidance.
Differential diagnoses should include airborne dermatitis, atopic dermatitis, seborrheic dermatitis, actinic prurigo, hydroa vacciniforme, solar urticaria, mycosis fungoides, lupus erythematosus, drug or chemical-induced dermatitis and porphyria cutanea tarda.4,6,8,10,11
Treatment
First and foremost, treatment involves abstaining from any sun exposure. Sunscreens are no longer beneficial once the disease is advanced.5 Clothing with a tight-weave pattern may provide adequate protection from the sun.12 As this disease may be associated with allergens, avoidance of such allergens is necessary.
Treatments in the past with beta-carotene and hydroxychloroquine (Plaquenil) have been of limited benefit.13,14 More effective treatment options may include azathioprine (Imuran) and PUVA.5,8
For severe disease, topical and oral corticosteroids are generally necessary. However, corticosteroids may not be able to control the disease process alone.8 Corticosteroids are often used in conjunction with PUVA.13 Cyclosporine (Neoral, Sandimmune) and mycophenolate mofetil (CellCept) have also been used as steroid sparing agents for CAD.7,16 Tacrolimus (Protopic) has also been used with limited success.10 The use of emollients remains a required treatment.
Discussion
Chronic actinic dermatitis represents a serious, persistent and rare photodermatoses. Multiple light wavelengths are implicated, including UVA and UVB.7 Several hypotheses have been proposed to explain CAD but none have gained favor. The hypotheses include continued presence of photosensitizing drugs in the skin, autosensitization to skin proteins, allergies to plants and household goods and chronic hyperirritability of the patient’s skin.5,8
Other factors that can predispose a patient to CAD include male gender, age, outdoor activity, atopic eczema, HIV infection and allergic contact dermatitis.6
Many systemic diseases present with a similar clinical picture and must be ruled out. A clear history is needed but is insufficient for making the diagnosis of CAD. A biopsy is needed to further define the disease process. Phototesting may also be necessary although not always helpful.
Once the disease has become severe, as in our patient, few treatment options exist. Although this patient was tried on potent topical steroids for years, his condition did not improve. Given his hepatitis C status, therapeutic regimens with immunosuppressive medications were not utilized. Etanercept (Enbrel) may be reasonable as it has been used safely in patients with hepatitis C.17
As in our patient, CAD often takes an unremitting course. Early diagnosis and aggressive treatment is needed for this disease to improve a patient’s quality of life. The condition often fails to respond to conventional treatment and immunosuppressive therapy may be required.
A 53-year-old African-American male presented with a persistent pruritic eruption on his upper body. The patient described intense pruritus on his head, face, arms and hands that caused multiple excoriations on his arms and hands. Sunlight intensified the pruritus. The patient was clothed in heavy material including gloves, jacket and a mask to protect him from the sun.
The patient noted that pruritic lesions first appeared years earlier when he worked in a textile mill. He was exposed to chemicals at the mill, which he believed to be responsible for the lesions on his arms. His condition worsened as he worked as a day laborer in the fields of Georgia. He had been seen by many dermatologists and received multiple courses of corticosteroids, oral and topical, without benefit. Multiple biopsies were performed at various medical centers. He denied any history of connective tissue diseases and previous testing (ANA, anti-Ro, anti-La) were normal.
Physical examination revealed diffuse xerosis, lichenification and alopecia involving the face, neck, upper chest, arms and hands. His legs, abdomen, back, shoulders and submental region were spared. The patient had liberally applied white petrolatum to all areas of lichenification. Multiple excoriations in different stages of healing were present on the hands and forearms.
Complete blood cell count, complete metabolic panel, liver function tests, renal function tests, thyroid stimulating hormone, HIV and rapid plasma reagin testing and urine and stool porphyrins were normal. Skin biopsy revealed a superficial dermal perivascular lymphohistiocytic infiltrate consistent with actinic reticuloid.
What is Your Diagnosis?
Diagnosis: Actinic Reticuloid (Chronic Actinic Dermatitis)
Actinic reticuloid, or chronic actinic dermatitis (CAD), describes a group of serious persistent photodermatoses. In 1961, Wilkinson described a photodermatosis as a “persistent light reaction”.1
A variety of terminology is used to describe this photodermatosis, including photosensitive eczema, chronic photosensitive dermatitis, persistent light reactivity and actinic reticuloid. These terms occur along a continuum of disease.2 In order to resolve this, chronic actinic dermatitis (CAD) has been proposed to encompass all these entities.2,3 Actinic reticuloid represents the most severe form of CAD. The histologic features of actinic reticuloid have been described as resembling lymphoma.4 Although many hypotheses for the pathologic basis of CAD have been proposed, no consensus exists. Like-wise, a consensus on treatment of such a severe disease is also lacking.
Clinical Presentation
Chronic actinic dermatitis occurs in multiple races.5,6 In all races, most patients tend to be middle-aged or elderly.
The condition generally begins with persistent erythema of sun-exposed areas. Lesions are characterized by pruritus and worsening with sun exposure. As the condition progresses, lichenification occurs; however, papules and plaques may be seen.7 Cutaneous edema and vesicles may also be present. Severe pruritus of the affected area is a hallmark of the disease process. Alopecia may occur as a consequence of prolonged and vigorous rubbing. Large areas of skin may be hypo- or hyperpigmented.6
Evolution of this disease may be linked to the patient’s work outdoors and thus to the degree of ultraviolet light exposure. However, despite decreased sun exposure, severe disease can persist during winter months.5,8
Histologic examination of early CAD may resemble contact dermatitis.5 In later stages with lichenification, the histologic pattern may resemble pseudolymphoma.4 The dermal infiltrate consists primarily of suppressor T cells.9 A constellation of changes has been described in the dermis and epidermis but these changes generally represent prolonged rubbing of the skin.
Diagnosis
The diagnosis of CAD can be challenging. Several factors add to the frustration with this diagnosis including confusing terminology, indistinct histopathology and clinical variability. CAD is rare when compared to other photodermatoses affecting the elderly.10 A diagnostic criterion proposed in the past includes persistent facial erythema, a middle-aged or elderly patient and severe photosensitivity with a broad action spectrum for at least 1 year.5 However, the diagnosis of CAD is made with a constellation of clinical features, histologic findings and phototest abnormalities.8
Clinical history of light sensitivity or photosensitivity caused by a photoallergen is most important in establishing a diagnosis of CAD. Phototesting is useful to confirm the diagnosis.11 Patch and photopatch tests are essential ancillary tests.6 Many studies have indicated a contact allergic source for CAD including plants and chemicals.5 As such, allergic testing may be done to aid the patient in allergen avoidance.
Differential diagnoses should include airborne dermatitis, atopic dermatitis, seborrheic dermatitis, actinic prurigo, hydroa vacciniforme, solar urticaria, mycosis fungoides, lupus erythematosus, drug or chemical-induced dermatitis and porphyria cutanea tarda.4,6,8,10,11
Treatment
First and foremost, treatment involves abstaining from any sun exposure. Sunscreens are no longer beneficial once the disease is advanced.5 Clothing with a tight-weave pattern may provide adequate protection from the sun.12 As this disease may be associated with allergens, avoidance of such allergens is necessary.
Treatments in the past with beta-carotene and hydroxychloroquine (Plaquenil) have been of limited benefit.13,14 More effective treatment options may include azathioprine (Imuran) and PUVA.5,8
For severe disease, topical and oral corticosteroids are generally necessary. However, corticosteroids may not be able to control the disease process alone.8 Corticosteroids are often used in conjunction with PUVA.13 Cyclosporine (Neoral, Sandimmune) and mycophenolate mofetil (CellCept) have also been used as steroid sparing agents for CAD.7,16 Tacrolimus (Protopic) has also been used with limited success.10 The use of emollients remains a required treatment.
Discussion
Chronic actinic dermatitis represents a serious, persistent and rare photodermatoses. Multiple light wavelengths are implicated, including UVA and UVB.7 Several hypotheses have been proposed to explain CAD but none have gained favor. The hypotheses include continued presence of photosensitizing drugs in the skin, autosensitization to skin proteins, allergies to plants and household goods and chronic hyperirritability of the patient’s skin.5,8
Other factors that can predispose a patient to CAD include male gender, age, outdoor activity, atopic eczema, HIV infection and allergic contact dermatitis.6
Many systemic diseases present with a similar clinical picture and must be ruled out. A clear history is needed but is insufficient for making the diagnosis of CAD. A biopsy is needed to further define the disease process. Phototesting may also be necessary although not always helpful.
Once the disease has become severe, as in our patient, few treatment options exist. Although this patient was tried on potent topical steroids for years, his condition did not improve. Given his hepatitis C status, therapeutic regimens with immunosuppressive medications were not utilized. Etanercept (Enbrel) may be reasonable as it has been used safely in patients with hepatitis C.17
As in our patient, CAD often takes an unremitting course. Early diagnosis and aggressive treatment is needed for this disease to improve a patient’s quality of life. The condition often fails to respond to conventional treatment and immunosuppressive therapy may be required.
A 53-year-old African-American male presented with a persistent pruritic eruption on his upper body. The patient described intense pruritus on his head, face, arms and hands that caused multiple excoriations on his arms and hands. Sunlight intensified the pruritus. The patient was clothed in heavy material including gloves, jacket and a mask to protect him from the sun.
The patient noted that pruritic lesions first appeared years earlier when he worked in a textile mill. He was exposed to chemicals at the mill, which he believed to be responsible for the lesions on his arms. His condition worsened as he worked as a day laborer in the fields of Georgia. He had been seen by many dermatologists and received multiple courses of corticosteroids, oral and topical, without benefit. Multiple biopsies were performed at various medical centers. He denied any history of connective tissue diseases and previous testing (ANA, anti-Ro, anti-La) were normal.
Physical examination revealed diffuse xerosis, lichenification and alopecia involving the face, neck, upper chest, arms and hands. His legs, abdomen, back, shoulders and submental region were spared. The patient had liberally applied white petrolatum to all areas of lichenification. Multiple excoriations in different stages of healing were present on the hands and forearms.
Complete blood cell count, complete metabolic panel, liver function tests, renal function tests, thyroid stimulating hormone, HIV and rapid plasma reagin testing and urine and stool porphyrins were normal. Skin biopsy revealed a superficial dermal perivascular lymphohistiocytic infiltrate consistent with actinic reticuloid.
What is Your Diagnosis?
,
Patient Presentation
A 53-year-old African-American male presented with a persistent pruritic eruption on his upper body. The patient described intense pruritus on his head, face, arms and hands that caused multiple excoriations on his arms and hands. Sunlight intensified the pruritus. The patient was clothed in heavy material including gloves, jacket and a mask to protect him from the sun.
The patient noted that pruritic lesions first appeared years earlier when he worked in a textile mill. He was exposed to chemicals at the mill, which he believed to be responsible for the lesions on his arms. His condition worsened as he worked as a day laborer in the fields of Georgia. He had been seen by many dermatologists and received multiple courses of corticosteroids, oral and topical, without benefit. Multiple biopsies were performed at various medical centers. He denied any history of connective tissue diseases and previous testing (ANA, anti-Ro, anti-La) were normal.
Physical examination revealed diffuse xerosis, lichenification and alopecia involving the face, neck, upper chest, arms and hands. His legs, abdomen, back, shoulders and submental region were spared. The patient had liberally applied white petrolatum to all areas of lichenification. Multiple excoriations in different stages of healing were present on the hands and forearms.
Complete blood cell count, complete metabolic panel, liver function tests, renal function tests, thyroid stimulating hormone, HIV and rapid plasma reagin testing and urine and stool porphyrins were normal. Skin biopsy revealed a superficial dermal perivascular lymphohistiocytic infiltrate consistent with actinic reticuloid.
What is Your Diagnosis?
Diagnosis: Actinic Reticuloid (Chronic Actinic Dermatitis)
Actinic reticuloid, or chronic actinic dermatitis (CAD), describes a group of serious persistent photodermatoses. In 1961, Wilkinson described a photodermatosis as a “persistent light reaction”.1
A variety of terminology is used to describe this photodermatosis, including photosensitive eczema, chronic photosensitive dermatitis, persistent light reactivity and actinic reticuloid. These terms occur along a continuum of disease.2 In order to resolve this, chronic actinic dermatitis (CAD) has been proposed to encompass all these entities.2,3 Actinic reticuloid represents the most severe form of CAD. The histologic features of actinic reticuloid have been described as resembling lymphoma.4 Although many hypotheses for the pathologic basis of CAD have been proposed, no consensus exists. Like-wise, a consensus on treatment of such a severe disease is also lacking.
Clinical Presentation
Chronic actinic dermatitis occurs in multiple races.5,6 In all races, most patients tend to be middle-aged or elderly.
The condition generally begins with persistent erythema of sun-exposed areas. Lesions are characterized by pruritus and worsening with sun exposure. As the condition progresses, lichenification occurs; however, papules and plaques may be seen.7 Cutaneous edema and vesicles may also be present. Severe pruritus of the affected area is a hallmark of the disease process. Alopecia may occur as a consequence of prolonged and vigorous rubbing. Large areas of skin may be hypo- or hyperpigmented.6
Evolution of this disease may be linked to the patient’s work outdoors and thus to the degree of ultraviolet light exposure. However, despite decreased sun exposure, severe disease can persist during winter months.5,8
Histologic examination of early CAD may resemble contact dermatitis.5 In later stages with lichenification, the histologic pattern may resemble pseudolymphoma.4 The dermal infiltrate consists primarily of suppressor T cells.9 A constellation of changes has been described in the dermis and epidermis but these changes generally represent prolonged rubbing of the skin.
Diagnosis
The diagnosis of CAD can be challenging. Several factors add to the frustration with this diagnosis including confusing terminology, indistinct histopathology and clinical variability. CAD is rare when compared to other photodermatoses affecting the elderly.10 A diagnostic criterion proposed in the past includes persistent facial erythema, a middle-aged or elderly patient and severe photosensitivity with a broad action spectrum for at least 1 year.5 However, the diagnosis of CAD is made with a constellation of clinical features, histologic findings and phototest abnormalities.8
Clinical history of light sensitivity or photosensitivity caused by a photoallergen is most important in establishing a diagnosis of CAD. Phototesting is useful to confirm the diagnosis.11 Patch and photopatch tests are essential ancillary tests.6 Many studies have indicated a contact allergic source for CAD including plants and chemicals.5 As such, allergic testing may be done to aid the patient in allergen avoidance.
Differential diagnoses should include airborne dermatitis, atopic dermatitis, seborrheic dermatitis, actinic prurigo, hydroa vacciniforme, solar urticaria, mycosis fungoides, lupus erythematosus, drug or chemical-induced dermatitis and porphyria cutanea tarda.4,6,8,10,11
Treatment
First and foremost, treatment involves abstaining from any sun exposure. Sunscreens are no longer beneficial once the disease is advanced.5 Clothing with a tight-weave pattern may provide adequate protection from the sun.12 As this disease may be associated with allergens, avoidance of such allergens is necessary.
Treatments in the past with beta-carotene and hydroxychloroquine (Plaquenil) have been of limited benefit.13,14 More effective treatment options may include azathioprine (Imuran) and PUVA.5,8
For severe disease, topical and oral corticosteroids are generally necessary. However, corticosteroids may not be able to control the disease process alone.8 Corticosteroids are often used in conjunction with PUVA.13 Cyclosporine (Neoral, Sandimmune) and mycophenolate mofetil (CellCept) have also been used as steroid sparing agents for CAD.7,16 Tacrolimus (Protopic) has also been used with limited success.10 The use of emollients remains a required treatment.
Discussion
Chronic actinic dermatitis represents a serious, persistent and rare photodermatoses. Multiple light wavelengths are implicated, including UVA and UVB.7 Several hypotheses have been proposed to explain CAD but none have gained favor. The hypotheses include continued presence of photosensitizing drugs in the skin, autosensitization to skin proteins, allergies to plants and household goods and chronic hyperirritability of the patient’s skin.5,8
Other factors that can predispose a patient to CAD include male gender, age, outdoor activity, atopic eczema, HIV infection and allergic contact dermatitis.6
Many systemic diseases present with a similar clinical picture and must be ruled out. A clear history is needed but is insufficient for making the diagnosis of CAD. A biopsy is needed to further define the disease process. Phototesting may also be necessary although not always helpful.
Once the disease has become severe, as in our patient, few treatment options exist. Although this patient was tried on potent topical steroids for years, his condition did not improve. Given his hepatitis C status, therapeutic regimens with immunosuppressive medications were not utilized. Etanercept (Enbrel) may be reasonable as it has been used safely in patients with hepatitis C.17
As in our patient, CAD often takes an unremitting course. Early diagnosis and aggressive treatment is needed for this disease to improve a patient’s quality of life. The condition often fails to respond to conventional treatment and immunosuppressive therapy may be required.
A 53-year-old African-American male presented with a persistent pruritic eruption on his upper body. The patient described intense pruritus on his head, face, arms and hands that caused multiple excoriations on his arms and hands. Sunlight intensified the pruritus. The patient was clothed in heavy material including gloves, jacket and a mask to protect him from the sun.
The patient noted that pruritic lesions first appeared years earlier when he worked in a textile mill. He was exposed to chemicals at the mill, which he believed to be responsible for the lesions on his arms. His condition worsened as he worked as a day laborer in the fields of Georgia. He had been seen by many dermatologists and received multiple courses of corticosteroids, oral and topical, without benefit. Multiple biopsies were performed at various medical centers. He denied any history of connective tissue diseases and previous testing (ANA, anti-Ro, anti-La) were normal.
Physical examination revealed diffuse xerosis, lichenification and alopecia involving the face, neck, upper chest, arms and hands. His legs, abdomen, back, shoulders and submental region were spared. The patient had liberally applied white petrolatum to all areas of lichenification. Multiple excoriations in different stages of healing were present on the hands and forearms.
Complete blood cell count, complete metabolic panel, liver function tests, renal function tests, thyroid stimulating hormone, HIV and rapid plasma reagin testing and urine and stool porphyrins were normal. Skin biopsy revealed a superficial dermal perivascular lymphohistiocytic infiltrate consistent with actinic reticuloid.
What is Your Diagnosis?
Diagnosis: Actinic Reticuloid (Chronic Actinic Dermatitis)
Actinic reticuloid, or chronic actinic dermatitis (CAD), describes a group of serious persistent photodermatoses. In 1961, Wilkinson described a photodermatosis as a “persistent light reaction”.1
A variety of terminology is used to describe this photodermatosis, including photosensitive eczema, chronic photosensitive dermatitis, persistent light reactivity and actinic reticuloid. These terms occur along a continuum of disease.2 In order to resolve this, chronic actinic dermatitis (CAD) has been proposed to encompass all these entities.2,3 Actinic reticuloid represents the most severe form of CAD. The histologic features of actinic reticuloid have been described as resembling lymphoma.4 Although many hypotheses for the pathologic basis of CAD have been proposed, no consensus exists. Like-wise, a consensus on treatment of such a severe disease is also lacking.
Clinical Presentation
Chronic actinic dermatitis occurs in multiple races.5,6 In all races, most patients tend to be middle-aged or elderly.
The condition generally begins with persistent erythema of sun-exposed areas. Lesions are characterized by pruritus and worsening with sun exposure. As the condition progresses, lichenification occurs; however, papules and plaques may be seen.7 Cutaneous edema and vesicles may also be present. Severe pruritus of the affected area is a hallmark of the disease process. Alopecia may occur as a consequence of prolonged and vigorous rubbing. Large areas of skin may be hypo- or hyperpigmented.6
Evolution of this disease may be linked to the patient’s work outdoors and thus to the degree of ultraviolet light exposure. However, despite decreased sun exposure, severe disease can persist during winter months.5,8
Histologic examination of early CAD may resemble contact dermatitis.5 In later stages with lichenification, the histologic pattern may resemble pseudolymphoma.4 The dermal infiltrate consists primarily of suppressor T cells.9 A constellation of changes has been described in the dermis and epidermis but these changes generally represent prolonged rubbing of the skin.
Diagnosis
The diagnosis of CAD can be challenging. Several factors add to the frustration with this diagnosis including confusing terminology, indistinct histopathology and clinical variability. CAD is rare when compared to other photodermatoses affecting the elderly.10 A diagnostic criterion proposed in the past includes persistent facial erythema, a middle-aged or elderly patient and severe photosensitivity with a broad action spectrum for at least 1 year.5 However, the diagnosis of CAD is made with a constellation of clinical features, histologic findings and phototest abnormalities.8
Clinical history of light sensitivity or photosensitivity caused by a photoallergen is most important in establishing a diagnosis of CAD. Phototesting is useful to confirm the diagnosis.11 Patch and photopatch tests are essential ancillary tests.6 Many studies have indicated a contact allergic source for CAD including plants and chemicals.5 As such, allergic testing may be done to aid the patient in allergen avoidance.
Differential diagnoses should include airborne dermatitis, atopic dermatitis, seborrheic dermatitis, actinic prurigo, hydroa vacciniforme, solar urticaria, mycosis fungoides, lupus erythematosus, drug or chemical-induced dermatitis and porphyria cutanea tarda.4,6,8,10,11
Treatment
First and foremost, treatment involves abstaining from any sun exposure. Sunscreens are no longer beneficial once the disease is advanced.5 Clothing with a tight-weave pattern may provide adequate protection from the sun.12 As this disease may be associated with allergens, avoidance of such allergens is necessary.
Treatments in the past with beta-carotene and hydroxychloroquine (Plaquenil) have been of limited benefit.13,14 More effective treatment options may include azathioprine (Imuran) and PUVA.5,8
For severe disease, topical and oral corticosteroids are generally necessary. However, corticosteroids may not be able to control the disease process alone.8 Corticosteroids are often used in conjunction with PUVA.13 Cyclosporine (Neoral, Sandimmune) and mycophenolate mofetil (CellCept) have also been used as steroid sparing agents for CAD.7,16 Tacrolimus (Protopic) has also been used with limited success.10 The use of emollients remains a required treatment.
Discussion
Chronic actinic dermatitis represents a serious, persistent and rare photodermatoses. Multiple light wavelengths are implicated, including UVA and UVB.7 Several hypotheses have been proposed to explain CAD but none have gained favor. The hypotheses include continued presence of photosensitizing drugs in the skin, autosensitization to skin proteins, allergies to plants and household goods and chronic hyperirritability of the patient’s skin.5,8
Other factors that can predispose a patient to CAD include male gender, age, outdoor activity, atopic eczema, HIV infection and allergic contact dermatitis.6
Many systemic diseases present with a similar clinical picture and must be ruled out. A clear history is needed but is insufficient for making the diagnosis of CAD. A biopsy is needed to further define the disease process. Phototesting may also be necessary although not always helpful.
Once the disease has become severe, as in our patient, few treatment options exist. Although this patient was tried on potent topical steroids for years, his condition did not improve. Given his hepatitis C status, therapeutic regimens with immunosuppressive medications were not utilized. Etanercept (Enbrel) may be reasonable as it has been used safely in patients with hepatitis C.17
As in our patient, CAD often takes an unremitting course. Early diagnosis and aggressive treatment is needed for this disease to improve a patient’s quality of life. The condition often fails to respond to conventional treatment and immunosuppressive therapy may be required.
Diagnosis: Actinic Reticuloid (Chronic Actinic Dermatitis)
Actinic reticuloid, or chronic actinic dermatitis (CAD), describes a group of serious persistent photodermatoses. In 1961, Wilkinson described a photodermatosis as a “persistent light reaction”.1
A variety of terminology is used to describe this photodermatosis, including photosensitive eczema, chronic photosensitive dermatitis, persistent light reactivity and actinic reticuloid. These terms occur along a continuum of disease.2 In order to resolve this, chronic actinic dermatitis (CAD) has been proposed to encompass all these entities.2,3 Actinic reticuloid represents the most severe form of CAD. The histologic features of actinic reticuloid have been described as resembling lymphoma.4 Although many hypotheses for the pathologic basis of CAD have been proposed, no consensus exists. Like-wise, a consensus on treatment of such a severe disease is also lacking.
Clinical Presentation
Chronic actinic dermatitis occurs in multiple races.5,6 In all races, most patients tend to be middle-aged or elderly.
The condition generally begins with persistent erythema of sun-exposed areas. Lesions are characterized by pruritus and worsening with sun exposure. As the condition progresses, lichenification occurs; however, papules and plaques may be seen.7 Cutaneous edema and vesicles may also be present. Severe pruritus of the affected area is a hallmark of the disease process. Alopecia may occur as a consequence of prolonged and vigorous rubbing. Large areas of skin may be hypo- or hyperpigmented.6
Evolution of this disease may be linked to the patient’s work outdoors and thus to the degree of ultraviolet light exposure. However, despite decreased sun exposure, severe disease can persist during winter months.5,8
Histologic examination of early CAD may resemble contact dermatitis.5 In later stages with lichenification, the histologic pattern may resemble pseudolymphoma.4 The dermal infiltrate consists primarily of suppressor T cells.9 A constellation of changes has been described in the dermis and epidermis but these changes generally represent prolonged rubbing of the skin.
Diagnosis
The diagnosis of CAD can be challenging. Several factors add to the frustration with this diagnosis including confusing terminology, indistinct histopathology and clinical variability. CAD is rare when compared to other photodermatoses affecting the elderly.10 A diagnostic criterion proposed in the past includes persistent facial erythema, a middle-aged or elderly patient and severe photosensitivity with a broad action spectrum for at least 1 year.5 However, the diagnosis of CAD is made with a constellation of clinical features, histologic findings and phototest abnormalities.8
Clinical history of light sensitivity or photosensitivity caused by a photoallergen is most important in establishing a diagnosis of CAD. Phototesting is useful to confirm the diagnosis.11 Patch and photopatch tests are essential ancillary tests.6 Many studies have indicated a contact allergic source for CAD including plants and chemicals.5 As such, allergic testing may be done to aid the patient in allergen avoidance.
Differential diagnoses should include airborne dermatitis, atopic dermatitis, seborrheic dermatitis, actinic prurigo, hydroa vacciniforme, solar urticaria, mycosis fungoides, lupus erythematosus, drug or chemical-induced dermatitis and porphyria cutanea tarda.4,6,8,10,11
Treatment
First and foremost, treatment involves abstaining from any sun exposure. Sunscreens are no longer beneficial once the disease is advanced.5 Clothing with a tight-weave pattern may provide adequate protection from the sun.12 As this disease may be associated with allergens, avoidance of such allergens is necessary.
Treatments in the past with beta-carotene and hydroxychloroquine (Plaquenil) have been of limited benefit.13,14 More effective treatment options may include azathioprine (Imuran) and PUVA.5,8
For severe disease, topical and oral corticosteroids are generally necessary. However, corticosteroids may not be able to control the disease process alone.8 Corticosteroids are often used in conjunction with PUVA.13 Cyclosporine (Neoral, Sandimmune) and mycophenolate mofetil (CellCept) have also been used as steroid sparing agents for CAD.7,16 Tacrolimus (Protopic) has also been used with limited success.10 The use of emollients remains a required treatment.
Discussion
Chronic actinic dermatitis represents a serious, persistent and rare photodermatoses. Multiple light wavelengths are implicated, including UVA and UVB.7 Several hypotheses have been proposed to explain CAD but none have gained favor. The hypotheses include continued presence of photosensitizing drugs in the skin, autosensitization to skin proteins, allergies to plants and household goods and chronic hyperirritability of the patient’s skin.5,8
Other factors that can predispose a patient to CAD include male gender, age, outdoor activity, atopic eczema, HIV infection and allergic contact dermatitis.6
Many systemic diseases present with a similar clinical picture and must be ruled out. A clear history is needed but is insufficient for making the diagnosis of CAD. A biopsy is needed to further define the disease process. Phototesting may also be necessary although not always helpful.
Once the disease has become severe, as in our patient, few treatment options exist. Although this patient was tried on potent topical steroids for years, his condition did not improve. Given his hepatitis C status, therapeutic regimens with immunosuppressive medications were not utilized. Etanercept (Enbrel) may be reasonable as it has been used safely in patients with hepatitis C.17
As in our patient, CAD often takes an unremitting course. Early diagnosis and aggressive treatment is needed for this disease to improve a patient’s quality of life. The condition often fails to respond to conventional treatment and immunosuppressive therapy may be required.
