A 49-year-old African American man presented for the first time to our outpatient dermatology clinic for slightly pruritic lesions on the scalp that had appeared over the span of a few months. On examination, the patient had multiple annular hyperpigmented plaques with central clearing, some with atrophy and mild scaling (Figures 1 and 2). The plaques were localized on the scalp with no other skin or mucosal involvement. The patient denied any similar symptoms in the past; no other family members had similar skin findings, and he did not have pets in the household. A biopsy was performed for further evaluation.
WHAT IS YOUR DIAGNOSIS?
Answer on page 2
{{pagebreak}}
Diagnosis: Scalp Sarcoidosis Mimicking Discoid Lupus Erythematosus
Sarcoidosis is a multisystem granulomatous disease characterized by noncaseating granulomas found in the skin as well as many other organ systems. The most commonly affected organs include the lungs, lymph nodes, skin, and eyes. There is a bimodal age distribution seen in patients presenting with sarcoidosis, with peaks at 25 to 35 years and 45 to 65 years.1 In the United States, there is an increased prevalence of sarcoidosis in African Americans, specifically African American women in their fifth decade of life.1 Additionally, the disease course is often more severe and chronic for African American patients compared with Caucasians.2,3 Five percent of patients with sarcoidosis ultimately die from their disease; pulmonary, cardiac, and neurologic involvements are the most common underlying causes of mortality.3
Clinical Presentation
Cutaneous lesions are seen in sarcoidosis in 20% to 35% of cases.3,4 Clinically, cutaneous lesions of sarcoidosis may display a variety of different morphologies including but not limited to macules, patches, papules, plaques, subcutaneous nodules, ulcerations, and diffuse infiltrates.5 Classically, lesions of sarcoidosis are red-brown to erythematous dermal papules and plaques. Usually there is little secondary change such as scaling or ulceration, illustrating the uniqueness of our case and the difficulty in our diagnosis.5
There are many less common types of cutaneous sarcoidosis, which have specific and important associations. Uncommon variants include lupus pernio, in which papules and plaques are localized to areas affected by the cold, including ears, cheeks, and nose.1 Darier-Roussy is another uncommon variant, where lesions appear as subcutaneous nodules. Additionally, cutaneous sarcoidosis often develops at sites of prior trauma, including scar sites and tattoos.1
Pathogenesis
Although the exact immunopathogenesis of sarcoidosis has yet to be elucidated, it has been proposed that extrinsic antigens may serve as a potential trigger for the disease.4 The interaction of these antigens with the patient’s helper T cells is thought to lead to dysregulation of T cells; this process is thought to lead to development of and progression of the disease.4 At this time, although many viral and bacterial antigens have been hypothesized as potential culprits, the specific antigen remains unknown.
Pathology
Biopsies of cutaneous lesions “specific” for sarcoidosis exhibit noncaseating granulomas. In contrast, “nonspecific” lesions, which are a result of a reactive process, do not show underlying granulomatous inflammation.4,6 The most common “nonspecific” presentation of sarcoidosis is erythema nodosum.6
Differential Diagnosis
The differential diagnosis for sarcoidosis is extensive and based on the clinical presentation. In this case, the patient had annular sarcoidal lesions on the scalp which resembled discoid lupus erythematosus, which also occurs in African Americans and is typically localized to the scalp as well (Figures 1 and 2). Other entities that may be considered as part of the differential diagnosis for annular sarcoidosis include granuloma annulare (annular elastolytic giant-cell granuloma), tinea corporis, Hansen disease, and annular psoriasis.5
Management
Therapeutic options in cutaneous sarcoidosis are guided by the impact of disfigurement by lesions and the patient’s motivation and desire to treat. Treatment for cutaneous sarcoidosis is not necessary for all patients. For localized lesions, ultrapotent topical corticosteroids are thought to be beneficial for cutaneous lesions, although published evidence is lacking.4 Monthly intra- lesional injections with triamcinolone acetonide at concentrations ranging from 3 to 20 mg/mL into the reticular dermis are thought to be more effective than topical preparations, again for localized lesions.1,4
For rapidly progressive, generalized, or highly disfiguring disease, systemic corticosteroids are considered the initial treatment of choice, with responses seen generally within 1 to 3 months.4 Other systemic agents which have been shown to be helpful in both cutaneous and internal involvement include antimalarials, methotrexate, tetracyclines, thalidomide (Thalomid), infliximab (Remicade), and adalimumab (Humira).4 The benefit of treatments must be weighed against the considerable risks that come with the use of these immunosuppressive and immunomodulatory agents.
Article continues on page 3
{{pagebreak}}
Our Patient
Pathology revealed a dermal infiltrate composed of naked or sarcoidal noncaseating granulomas (Figures 3 and 4). These granulomas contained epithelioid histiocytes and multinucleated giant cells. Appropriate fungal and bacterial stains, performed to rule out infectious etiology, were negative. The clinical presentation along with pathology findings confirmed the diagnosis of sarcoidosis.
Our patient was evaluated by an ophthalmologist, and there was no ophthalmic involvement found on exam. The patient will be followed yearly. The patient was also evaluated in pulmonology, with pulmonary function tests to assess and monitor for pulmonary involvement.
Given that our patient had localized lesions on the scalp,without prior treatment, he was started on clobetasol 0.05% gel daily to affected areas. He denied pulmonary or ocular symptoms. He used the clobetasol gel with initial clearing of scalp lesions; however, he was lost to follow-up for approximately 9 months and subsequently developed additional lesions on the scalp and upper chest.
Conclusion
This case illustrates the importance of keeping sarcoidosis on your differential diagnosis when a patient presents with annular plaques. If clinically suspected, a biopsy should be performed in order to obtain a tissue diagnosis. Sarcoidosis is often a multisystem disease; if left untreated, it can lead to severe end-organ damage. In this case, a definitive diagnosis was determined, allowing for appropriate management of our patient.
Dr. Curreri is chief resident in the department of dermatology at SUNY Downstate in Brooklyn, NY.
Dr. Liebman is a resident in the department of dermatology at SUNY Downstate in Brooklyn, NY.
Dr. Alapati is chief of dermatology at Brooklyn Veterans Affairs Medical Center Dermatology Service in Brooklyn, NY.
Dr. Khachemoune, the Section Editor of Derm DX, is with the Department of Dermatology at the State University of New York Downstate in Brooklyn, NY.
Disclosure: The authors report no relevant financial relationships.
References
1. Bolognia JL, Jorizzo JL, Schaffer JV. Dermatology. 3rd ed. St. Louis, MO: Mosby/Elsevier; 2012:1558-1563.
2. Heath CR, David J, Taylor SC. Sarcoidosis: Are there differences in your skin of color patients? J Am Acad Dermatol. 2012;66(1):121.e1-14.
3. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357(21):2153-2165.
4. Haimovic A, Sanchez M, Judson MA, Prystowsky S. Sarcoidosis: A comprehensive review and update for the dermatologist. Part II. Extracutaneous disease. J Am Acad Dermatol 2012;66(5):719.e2-10.
5. Fernandez-Faith E, McDonnell J. Cutaneous sarcoidosis: differential diagnosis. Clin Dermatol 2007;25(3):276-287.
6. Mana J, Marcoval J. Skin manifestations of sarcoidosis. La Presse Médicale. 2012;41(6):e355-374.
A 49-year-old African American man presented for the first time to our outpatient dermatology clinic for slightly pruritic lesions on the scalp that had appeared over the span of a few months. On examination, the patient had multiple annular hyperpigmented plaques with central clearing, some with atrophy and mild scaling (Figures 1 and 2). The plaques were localized on the scalp with no other skin or mucosal involvement. The patient denied any similar symptoms in the past; no other family members had similar skin findings, and he did not have pets in the household. A biopsy was performed for further evaluation.
WHAT IS YOUR DIAGNOSIS?
Diagnosis: Scalp Sarcoidosis Mimicking Discoid Lupus Erythematosus
Sarcoidosis is a multisystem granulomatous disease characterized by noncaseating granulomas found in the skin as well as many other organ systems. The most commonly affected organs include the lungs, lymph nodes, skin, and eyes. There is a bimodal age distribution seen in patients presenting with sarcoidosis, with peaks at 25 to 35 years and 45 to 65 years.1 In the United States, there is an increased prevalence of sarcoidosis in African Americans, specifically African American women in their fifth decade of life.1 Additionally, the disease course is often more severe and chronic for African American patients compared with Caucasians.2,3 Five percent of patients with sarcoidosis ultimately die from their disease; pulmonary, cardiac, and neurologic involvements are the most common underlying causes of mortality.3
Clinical Presentation
Cutaneous lesions are seen in sarcoidosis in 20% to 35% of cases.3,4 Clinically, cutaneous lesions of sarcoidosis may display a variety of different morphologies including but not limited to macules, patches, papules, plaques, subcutaneous nodules, ulcerations, and diffuse infiltrates.5 Classically, lesions of sarcoidosis are red-brown to erythematous dermal papules and plaques. Usually there is little secondary change such as scaling or ulceration, illustrating the uniqueness of our case and the difficulty in our diagnosis.5
There are many less common types of cutaneous sarcoidosis, which have specific and important associations. Uncommon variants include lupus pernio, in which papules and plaques are localized to areas affected by the cold, including ears, cheeks, and nose.1 Darier-Roussy is another uncommon variant, where lesions appear as subcutaneous nodules. Additionally, cutaneous sarcoidosis often develops at sites of prior trauma, including scar sites and tattoos.1
Pathogenesis
Although the exact immunopathogenesis of sarcoidosis has yet to be elucidated, it has been proposed that extrinsic antigens may serve as a potential trigger for the disease.4 The interaction of these antigens with the patient’s helper T cells is thought to lead to dysregulation of T cells; this process is thought to lead to development of and progression of the disease.4 At this time, although many viral and bacterial antigens have been hypothesized as potential culprits, the specific antigen remains unknown.
Pathology
Biopsies of cutaneous lesions “specific” for sarcoidosis exhibit noncaseating granulomas. In contrast, “nonspecific” lesions, which are a result of a reactive process, do not show underlying granulomatous inflammation.4,6 The most common “nonspecific” presentation of sarcoidosis is erythema nodosum.6
Differential Diagnosis
The differential diagnosis for sarcoidosis is extensive and based on the clinical presentation. In this case, the patient had annular sarcoidal lesions on the scalp which resembled discoid lupus erythematosus, which also occurs in African Americans and is typically localized to the scalp as well (Figures 1 and 2). Other entities that may be considered as part of the differential diagnosis for annular sarcoidosis include granuloma annulare (annular elastolytic giant-cell granuloma), tinea corporis, Hansen disease, and annular psoriasis.5
Management
Therapeutic options in cutaneous sarcoidosis are guided by the impact of disfigurement by lesions and the patient’s motivation and desire to treat. Treatment for cutaneous sarcoidosis is not necessary for all patients. For localized lesions, ultrapotent topical corticosteroids are thought to be beneficial for cutaneous lesions, although published evidence is lacking.4 Monthly intra- lesional injections with triamcinolone acetonide at concentrations ranging from 3 to 20 mg/mL into the reticular dermis are thought to be more effective than topical preparations, again for localized lesions.1,4
For rapidly progressive, generalized, or highly disfiguring disease, systemic corticosteroids are considered the initial treatment of choice, with responses seen generally within 1 to 3 months.4 Other systemic agents which have been shown to be helpful in both cutaneous and internal involvement include antimalarials, methotrexate, tetracyclines, thalidomide (Thalomid), infliximab (Remicade), and adalimumab (Humira).4 The benefit of treatments must be weighed against the considerable risks that come with the use of these immunosuppressive and immunomodulatory agents.
Article continues on page 3
{{pagebreak}}
Our Patient
Pathology revealed a dermal infiltrate composed of naked or sarcoidal noncaseating granulomas (Figures 3 and 4). These granulomas contained epithelioid histiocytes and multinucleated giant cells. Appropriate fungal and bacterial stains, performed to rule out infectious etiology, were negative. The clinical presentation along with pathology findings confirmed the diagnosis of sarcoidosis.
Our patient was evaluated by an ophthalmologist, and there was no ophthalmic involvement found on exam. The patient will be followed yearly. The patient was also evaluated in pulmonology, with pulmonary function tests to assess and monitor for pulmonary involvement.
Given that our patient had localized lesions on the scalp,without prior treatment, he was started on clobetasol 0.05% gel daily to affected areas. He denied pulmonary or ocular symptoms. He used the clobetasol gel with initial clearing of scalp lesions; however, he was lost to follow-up for approximately 9 months and subsequently developed additional lesions on the scalp and upper chest.
Conclusion
This case illustrates the importance of keeping sarcoidosis on your differential diagnosis when a patient presents with annular plaques. If clinically suspected, a biopsy should be performed in order to obtain a tissue diagnosis. Sarcoidosis is often a multisystem disease; if left untreated, it can lead to severe end-organ damage. In this case, a definitive diagnosis was determined, allowing for appropriate management of our patient.
Dr. Curreri is chief resident in the department of dermatology at SUNY Downstate in Brooklyn, NY.
Dr. Liebman is a resident in the department of dermatology at SUNY Downstate in Brooklyn, NY.
Dr. Alapati is chief of dermatology at Brooklyn Veterans Affairs Medical Center Dermatology Service in Brooklyn, NY.
Dr. Khachemoune, the Section Editor of Derm DX, is with the Department of Dermatology at the State University of New York Downstate in Brooklyn, NY.
Disclosure: The authors report no relevant financial relationships.
References
1. Bolognia JL, Jorizzo JL, Schaffer JV. Dermatology. 3rd ed. St. Louis, MO: Mosby/Elsevier; 2012:1558-1563.
2. Heath CR, David J, Taylor SC. Sarcoidosis: Are there differences in your skin of color patients? J Am Acad Dermatol. 2012;66(1):121.e1-14.
3. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357(21):2153-2165.
4. Haimovic A, Sanchez M, Judson MA, Prystowsky S. Sarcoidosis: A comprehensive review and update for the dermatologist. Part II. Extracutaneous disease. J Am Acad Dermatol 2012;66(5):719.e2-10.
5. Fernandez-Faith E, McDonnell J. Cutaneous sarcoidosis: differential diagnosis. Clin Dermatol 2007;25(3):276-287.
6. Mana J, Marcoval J. Skin manifestations of sarcoidosis. La Presse Médicale. 2012;41(6):e355-374.
A 49-year-old African American man presented for the first time to our outpatient dermatology clinic for slightly pruritic lesions on the scalp that had appeared over the span of a few months. On examination, the patient had multiple annular hyperpigmented plaques with central clearing, some with atrophy and mild scaling (Figures 1 and 2). The plaques were localized on the scalp with no other skin or mucosal involvement. The patient denied any similar symptoms in the past; no other family members had similar skin findings, and he did not have pets in the household. A biopsy was performed for further evaluation.
WHAT IS YOUR DIAGNOSIS?
,
A 49-year-old African American man presented for the first time to our outpatient dermatology clinic for slightly pruritic lesions on the scalp that had appeared over the span of a few months. On examination, the patient had multiple annular hyperpigmented plaques with central clearing, some with atrophy and mild scaling (Figures 1 and 2). The plaques were localized on the scalp with no other skin or mucosal involvement. The patient denied any similar symptoms in the past; no other family members had similar skin findings, and he did not have pets in the household. A biopsy was performed for further evaluation.
WHAT IS YOUR DIAGNOSIS?
Answer on page 2
{{pagebreak}}
Diagnosis: Scalp Sarcoidosis Mimicking Discoid Lupus Erythematosus
Sarcoidosis is a multisystem granulomatous disease characterized by noncaseating granulomas found in the skin as well as many other organ systems. The most commonly affected organs include the lungs, lymph nodes, skin, and eyes. There is a bimodal age distribution seen in patients presenting with sarcoidosis, with peaks at 25 to 35 years and 45 to 65 years.1 In the United States, there is an increased prevalence of sarcoidosis in African Americans, specifically African American women in their fifth decade of life.1 Additionally, the disease course is often more severe and chronic for African American patients compared with Caucasians.2,3 Five percent of patients with sarcoidosis ultimately die from their disease; pulmonary, cardiac, and neurologic involvements are the most common underlying causes of mortality.3
Clinical Presentation
Cutaneous lesions are seen in sarcoidosis in 20% to 35% of cases.3,4 Clinically, cutaneous lesions of sarcoidosis may display a variety of different morphologies including but not limited to macules, patches, papules, plaques, subcutaneous nodules, ulcerations, and diffuse infiltrates.5 Classically, lesions of sarcoidosis are red-brown to erythematous dermal papules and plaques. Usually there is little secondary change such as scaling or ulceration, illustrating the uniqueness of our case and the difficulty in our diagnosis.5
There are many less common types of cutaneous sarcoidosis, which have specific and important associations. Uncommon variants include lupus pernio, in which papules and plaques are localized to areas affected by the cold, including ears, cheeks, and nose.1 Darier-Roussy is another uncommon variant, where lesions appear as subcutaneous nodules. Additionally, cutaneous sarcoidosis often develops at sites of prior trauma, including scar sites and tattoos.1
Pathogenesis
Although the exact immunopathogenesis of sarcoidosis has yet to be elucidated, it has been proposed that extrinsic antigens may serve as a potential trigger for the disease.4 The interaction of these antigens with the patient’s helper T cells is thought to lead to dysregulation of T cells; this process is thought to lead to development of and progression of the disease.4 At this time, although many viral and bacterial antigens have been hypothesized as potential culprits, the specific antigen remains unknown.
Pathology
Biopsies of cutaneous lesions “specific” for sarcoidosis exhibit noncaseating granulomas. In contrast, “nonspecific” lesions, which are a result of a reactive process, do not show underlying granulomatous inflammation.4,6 The most common “nonspecific” presentation of sarcoidosis is erythema nodosum.6
Differential Diagnosis
The differential diagnosis for sarcoidosis is extensive and based on the clinical presentation. In this case, the patient had annular sarcoidal lesions on the scalp which resembled discoid lupus erythematosus, which also occurs in African Americans and is typically localized to the scalp as well (Figures 1 and 2). Other entities that may be considered as part of the differential diagnosis for annular sarcoidosis include granuloma annulare (annular elastolytic giant-cell granuloma), tinea corporis, Hansen disease, and annular psoriasis.5
Management
Therapeutic options in cutaneous sarcoidosis are guided by the impact of disfigurement by lesions and the patient’s motivation and desire to treat. Treatment for cutaneous sarcoidosis is not necessary for all patients. For localized lesions, ultrapotent topical corticosteroids are thought to be beneficial for cutaneous lesions, although published evidence is lacking.4 Monthly intra- lesional injections with triamcinolone acetonide at concentrations ranging from 3 to 20 mg/mL into the reticular dermis are thought to be more effective than topical preparations, again for localized lesions.1,4
For rapidly progressive, generalized, or highly disfiguring disease, systemic corticosteroids are considered the initial treatment of choice, with responses seen generally within 1 to 3 months.4 Other systemic agents which have been shown to be helpful in both cutaneous and internal involvement include antimalarials, methotrexate, tetracyclines, thalidomide (Thalomid), infliximab (Remicade), and adalimumab (Humira).4 The benefit of treatments must be weighed against the considerable risks that come with the use of these immunosuppressive and immunomodulatory agents.
Article continues on page 3
{{pagebreak}}
Our Patient
Pathology revealed a dermal infiltrate composed of naked or sarcoidal noncaseating granulomas (Figures 3 and 4). These granulomas contained epithelioid histiocytes and multinucleated giant cells. Appropriate fungal and bacterial stains, performed to rule out infectious etiology, were negative. The clinical presentation along with pathology findings confirmed the diagnosis of sarcoidosis.
Our patient was evaluated by an ophthalmologist, and there was no ophthalmic involvement found on exam. The patient will be followed yearly. The patient was also evaluated in pulmonology, with pulmonary function tests to assess and monitor for pulmonary involvement.
Given that our patient had localized lesions on the scalp,without prior treatment, he was started on clobetasol 0.05% gel daily to affected areas. He denied pulmonary or ocular symptoms. He used the clobetasol gel with initial clearing of scalp lesions; however, he was lost to follow-up for approximately 9 months and subsequently developed additional lesions on the scalp and upper chest.
Conclusion
This case illustrates the importance of keeping sarcoidosis on your differential diagnosis when a patient presents with annular plaques. If clinically suspected, a biopsy should be performed in order to obtain a tissue diagnosis. Sarcoidosis is often a multisystem disease; if left untreated, it can lead to severe end-organ damage. In this case, a definitive diagnosis was determined, allowing for appropriate management of our patient.
Dr. Curreri is chief resident in the department of dermatology at SUNY Downstate in Brooklyn, NY.
Dr. Liebman is a resident in the department of dermatology at SUNY Downstate in Brooklyn, NY.
Dr. Alapati is chief of dermatology at Brooklyn Veterans Affairs Medical Center Dermatology Service in Brooklyn, NY.
Dr. Khachemoune, the Section Editor of Derm DX, is with the Department of Dermatology at the State University of New York Downstate in Brooklyn, NY.
Disclosure: The authors report no relevant financial relationships.
References
1. Bolognia JL, Jorizzo JL, Schaffer JV. Dermatology. 3rd ed. St. Louis, MO: Mosby/Elsevier; 2012:1558-1563.
2. Heath CR, David J, Taylor SC. Sarcoidosis: Are there differences in your skin of color patients? J Am Acad Dermatol. 2012;66(1):121.e1-14.
3. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357(21):2153-2165.
4. Haimovic A, Sanchez M, Judson MA, Prystowsky S. Sarcoidosis: A comprehensive review and update for the dermatologist. Part II. Extracutaneous disease. J Am Acad Dermatol 2012;66(5):719.e2-10.
5. Fernandez-Faith E, McDonnell J. Cutaneous sarcoidosis: differential diagnosis. Clin Dermatol 2007;25(3):276-287.
6. Mana J, Marcoval J. Skin manifestations of sarcoidosis. La Presse Médicale. 2012;41(6):e355-374.
A 49-year-old African American man presented for the first time to our outpatient dermatology clinic for slightly pruritic lesions on the scalp that had appeared over the span of a few months. On examination, the patient had multiple annular hyperpigmented plaques with central clearing, some with atrophy and mild scaling (Figures 1 and 2). The plaques were localized on the scalp with no other skin or mucosal involvement. The patient denied any similar symptoms in the past; no other family members had similar skin findings, and he did not have pets in the household. A biopsy was performed for further evaluation.
WHAT IS YOUR DIAGNOSIS?
Diagnosis: Scalp Sarcoidosis Mimicking Discoid Lupus Erythematosus
Sarcoidosis is a multisystem granulomatous disease characterized by noncaseating granulomas found in the skin as well as many other organ systems. The most commonly affected organs include the lungs, lymph nodes, skin, and eyes. There is a bimodal age distribution seen in patients presenting with sarcoidosis, with peaks at 25 to 35 years and 45 to 65 years.1 In the United States, there is an increased prevalence of sarcoidosis in African Americans, specifically African American women in their fifth decade of life.1 Additionally, the disease course is often more severe and chronic for African American patients compared with Caucasians.2,3 Five percent of patients with sarcoidosis ultimately die from their disease; pulmonary, cardiac, and neurologic involvements are the most common underlying causes of mortality.3
Clinical Presentation
Cutaneous lesions are seen in sarcoidosis in 20% to 35% of cases.3,4 Clinically, cutaneous lesions of sarcoidosis may display a variety of different morphologies including but not limited to macules, patches, papules, plaques, subcutaneous nodules, ulcerations, and diffuse infiltrates.5 Classically, lesions of sarcoidosis are red-brown to erythematous dermal papules and plaques. Usually there is little secondary change such as scaling or ulceration, illustrating the uniqueness of our case and the difficulty in our diagnosis.5
There are many less common types of cutaneous sarcoidosis, which have specific and important associations. Uncommon variants include lupus pernio, in which papules and plaques are localized to areas affected by the cold, including ears, cheeks, and nose.1 Darier-Roussy is another uncommon variant, where lesions appear as subcutaneous nodules. Additionally, cutaneous sarcoidosis often develops at sites of prior trauma, including scar sites and tattoos.1
Pathogenesis
Although the exact immunopathogenesis of sarcoidosis has yet to be elucidated, it has been proposed that extrinsic antigens may serve as a potential trigger for the disease.4 The interaction of these antigens with the patient’s helper T cells is thought to lead to dysregulation of T cells; this process is thought to lead to development of and progression of the disease.4 At this time, although many viral and bacterial antigens have been hypothesized as potential culprits, the specific antigen remains unknown.
Pathology
Biopsies of cutaneous lesions “specific” for sarcoidosis exhibit noncaseating granulomas. In contrast, “nonspecific” lesions, which are a result of a reactive process, do not show underlying granulomatous inflammation.4,6 The most common “nonspecific” presentation of sarcoidosis is erythema nodosum.6
Differential Diagnosis
The differential diagnosis for sarcoidosis is extensive and based on the clinical presentation. In this case, the patient had annular sarcoidal lesions on the scalp which resembled discoid lupus erythematosus, which also occurs in African Americans and is typically localized to the scalp as well (Figures 1 and 2). Other entities that may be considered as part of the differential diagnosis for annular sarcoidosis include granuloma annulare (annular elastolytic giant-cell granuloma), tinea corporis, Hansen disease, and annular psoriasis.5
Management
Therapeutic options in cutaneous sarcoidosis are guided by the impact of disfigurement by lesions and the patient’s motivation and desire to treat. Treatment for cutaneous sarcoidosis is not necessary for all patients. For localized lesions, ultrapotent topical corticosteroids are thought to be beneficial for cutaneous lesions, although published evidence is lacking.4 Monthly intra- lesional injections with triamcinolone acetonide at concentrations ranging from 3 to 20 mg/mL into the reticular dermis are thought to be more effective than topical preparations, again for localized lesions.1,4
For rapidly progressive, generalized, or highly disfiguring disease, systemic corticosteroids are considered the initial treatment of choice, with responses seen generally within 1 to 3 months.4 Other systemic agents which have been shown to be helpful in both cutaneous and internal involvement include antimalarials, methotrexate, tetracyclines, thalidomide (Thalomid), infliximab (Remicade), and adalimumab (Humira).4 The benefit of treatments must be weighed against the considerable risks that come with the use of these immunosuppressive and immunomodulatory agents.
Article continues on page 3
{{pagebreak}}
Our Patient
Pathology revealed a dermal infiltrate composed of naked or sarcoidal noncaseating granulomas (Figures 3 and 4). These granulomas contained epithelioid histiocytes and multinucleated giant cells. Appropriate fungal and bacterial stains, performed to rule out infectious etiology, were negative. The clinical presentation along with pathology findings confirmed the diagnosis of sarcoidosis.
Our patient was evaluated by an ophthalmologist, and there was no ophthalmic involvement found on exam. The patient will be followed yearly. The patient was also evaluated in pulmonology, with pulmonary function tests to assess and monitor for pulmonary involvement.
Given that our patient had localized lesions on the scalp,without prior treatment, he was started on clobetasol 0.05% gel daily to affected areas. He denied pulmonary or ocular symptoms. He used the clobetasol gel with initial clearing of scalp lesions; however, he was lost to follow-up for approximately 9 months and subsequently developed additional lesions on the scalp and upper chest.
Conclusion
This case illustrates the importance of keeping sarcoidosis on your differential diagnosis when a patient presents with annular plaques. If clinically suspected, a biopsy should be performed in order to obtain a tissue diagnosis. Sarcoidosis is often a multisystem disease; if left untreated, it can lead to severe end-organ damage. In this case, a definitive diagnosis was determined, allowing for appropriate management of our patient.
Dr. Curreri is chief resident in the department of dermatology at SUNY Downstate in Brooklyn, NY.
Dr. Liebman is a resident in the department of dermatology at SUNY Downstate in Brooklyn, NY.
Dr. Alapati is chief of dermatology at Brooklyn Veterans Affairs Medical Center Dermatology Service in Brooklyn, NY.
Dr. Khachemoune, the Section Editor of Derm DX, is with the Department of Dermatology at the State University of New York Downstate in Brooklyn, NY.
Disclosure: The authors report no relevant financial relationships.
References
1. Bolognia JL, Jorizzo JL, Schaffer JV. Dermatology. 3rd ed. St. Louis, MO: Mosby/Elsevier; 2012:1558-1563.
2. Heath CR, David J, Taylor SC. Sarcoidosis: Are there differences in your skin of color patients? J Am Acad Dermatol. 2012;66(1):121.e1-14.
3. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357(21):2153-2165.
4. Haimovic A, Sanchez M, Judson MA, Prystowsky S. Sarcoidosis: A comprehensive review and update for the dermatologist. Part II. Extracutaneous disease. J Am Acad Dermatol 2012;66(5):719.e2-10.
5. Fernandez-Faith E, McDonnell J. Cutaneous sarcoidosis: differential diagnosis. Clin Dermatol 2007;25(3):276-287.
6. Mana J, Marcoval J. Skin manifestations of sarcoidosis. La Presse Médicale. 2012;41(6):e355-374.
Diagnosis: Scalp Sarcoidosis Mimicking Discoid Lupus Erythematosus
Sarcoidosis is a multisystem granulomatous disease characterized by noncaseating granulomas found in the skin as well as many other organ systems. The most commonly affected organs include the lungs, lymph nodes, skin, and eyes. There is a bimodal age distribution seen in patients presenting with sarcoidosis, with peaks at 25 to 35 years and 45 to 65 years.1 In the United States, there is an increased prevalence of sarcoidosis in African Americans, specifically African American women in their fifth decade of life.1 Additionally, the disease course is often more severe and chronic for African American patients compared with Caucasians.2,3 Five percent of patients with sarcoidosis ultimately die from their disease; pulmonary, cardiac, and neurologic involvements are the most common underlying causes of mortality.3
Clinical Presentation
Cutaneous lesions are seen in sarcoidosis in 20% to 35% of cases.3,4 Clinically, cutaneous lesions of sarcoidosis may display a variety of different morphologies including but not limited to macules, patches, papules, plaques, subcutaneous nodules, ulcerations, and diffuse infiltrates.5 Classically, lesions of sarcoidosis are red-brown to erythematous dermal papules and plaques. Usually there is little secondary change such as scaling or ulceration, illustrating the uniqueness of our case and the difficulty in our diagnosis.5
There are many less common types of cutaneous sarcoidosis, which have specific and important associations. Uncommon variants include lupus pernio, in which papules and plaques are localized to areas affected by the cold, including ears, cheeks, and nose.1 Darier-Roussy is another uncommon variant, where lesions appear as subcutaneous nodules. Additionally, cutaneous sarcoidosis often develops at sites of prior trauma, including scar sites and tattoos.1
Pathogenesis
Although the exact immunopathogenesis of sarcoidosis has yet to be elucidated, it has been proposed that extrinsic antigens may serve as a potential trigger for the disease.4 The interaction of these antigens with the patient’s helper T cells is thought to lead to dysregulation of T cells; this process is thought to lead to development of and progression of the disease.4 At this time, although many viral and bacterial antigens have been hypothesized as potential culprits, the specific antigen remains unknown.
Pathology
Biopsies of cutaneous lesions “specific” for sarcoidosis exhibit noncaseating granulomas. In contrast, “nonspecific” lesions, which are a result of a reactive process, do not show underlying granulomatous inflammation.4,6 The most common “nonspecific” presentation of sarcoidosis is erythema nodosum.6
Differential Diagnosis
The differential diagnosis for sarcoidosis is extensive and based on the clinical presentation. In this case, the patient had annular sarcoidal lesions on the scalp which resembled discoid lupus erythematosus, which also occurs in African Americans and is typically localized to the scalp as well (Figures 1 and 2). Other entities that may be considered as part of the differential diagnosis for annular sarcoidosis include granuloma annulare (annular elastolytic giant-cell granuloma), tinea corporis, Hansen disease, and annular psoriasis.5
Management
Therapeutic options in cutaneous sarcoidosis are guided by the impact of disfigurement by lesions and the patient’s motivation and desire to treat. Treatment for cutaneous sarcoidosis is not necessary for all patients. For localized lesions, ultrapotent topical corticosteroids are thought to be beneficial for cutaneous lesions, although published evidence is lacking.4 Monthly intra- lesional injections with triamcinolone acetonide at concentrations ranging from 3 to 20 mg/mL into the reticular dermis are thought to be more effective than topical preparations, again for localized lesions.1,4
For rapidly progressive, generalized, or highly disfiguring disease, systemic corticosteroids are considered the initial treatment of choice, with responses seen generally within 1 to 3 months.4 Other systemic agents which have been shown to be helpful in both cutaneous and internal involvement include antimalarials, methotrexate, tetracyclines, thalidomide (Thalomid), infliximab (Remicade), and adalimumab (Humira).4 The benefit of treatments must be weighed against the considerable risks that come with the use of these immunosuppressive and immunomodulatory agents.
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Our Patient
Pathology revealed a dermal infiltrate composed of naked or sarcoidal noncaseating granulomas (Figures 3 and 4). These granulomas contained epithelioid histiocytes and multinucleated giant cells. Appropriate fungal and bacterial stains, performed to rule out infectious etiology, were negative. The clinical presentation along with pathology findings confirmed the diagnosis of sarcoidosis.
Our patient was evaluated by an ophthalmologist, and there was no ophthalmic involvement found on exam. The patient will be followed yearly. The patient was also evaluated in pulmonology, with pulmonary function tests to assess and monitor for pulmonary involvement.
Given that our patient had localized lesions on the scalp,without prior treatment, he was started on clobetasol 0.05% gel daily to affected areas. He denied pulmonary or ocular symptoms. He used the clobetasol gel with initial clearing of scalp lesions; however, he was lost to follow-up for approximately 9 months and subsequently developed additional lesions on the scalp and upper chest.
Conclusion
This case illustrates the importance of keeping sarcoidosis on your differential diagnosis when a patient presents with annular plaques. If clinically suspected, a biopsy should be performed in order to obtain a tissue diagnosis. Sarcoidosis is often a multisystem disease; if left untreated, it can lead to severe end-organ damage. In this case, a definitive diagnosis was determined, allowing for appropriate management of our patient.
Dr. Curreri is chief resident in the department of dermatology at SUNY Downstate in Brooklyn, NY.
Dr. Liebman is a resident in the department of dermatology at SUNY Downstate in Brooklyn, NY.
Dr. Alapati is chief of dermatology at Brooklyn Veterans Affairs Medical Center Dermatology Service in Brooklyn, NY.
Dr. Khachemoune, the Section Editor of Derm DX, is with the Department of Dermatology at the State University of New York Downstate in Brooklyn, NY.
Disclosure: The authors report no relevant financial relationships.
References
1. Bolognia JL, Jorizzo JL, Schaffer JV. Dermatology. 3rd ed. St. Louis, MO: Mosby/Elsevier; 2012:1558-1563.
2. Heath CR, David J, Taylor SC. Sarcoidosis: Are there differences in your skin of color patients? J Am Acad Dermatol. 2012;66(1):121.e1-14.
3. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357(21):2153-2165.
4. Haimovic A, Sanchez M, Judson MA, Prystowsky S. Sarcoidosis: A comprehensive review and update for the dermatologist. Part II. Extracutaneous disease. J Am Acad Dermatol 2012;66(5):719.e2-10.
5. Fernandez-Faith E, McDonnell J. Cutaneous sarcoidosis: differential diagnosis. Clin Dermatol 2007;25(3):276-287.
6. Mana J, Marcoval J. Skin manifestations of sarcoidosis. La Presse Médicale. 2012;41(6):e355-374.
