1. This patient had low titer antinuclear antibodies and arthralgias. These lesions are:
a) Discoid lupus erythematosus
b) Morphea
c) Lupus profundus
d) Cutaneous mucinosis secondary to lupus erythematosus
e) Erythema nodosum
2. This patient had these recurrent “burning” lesions, arthralgias, elevated sedimentation rate, and reduced total hemolytic complement. Biopsy showed a dermal necrotizing venulitis. The diagnosis is:
a) Idiopathic chronic urticaria
b) Hypersensitivity angiitis of Zeek
c) Urticarial vasculitis
d) Systemic lupus erythematosus
e) Serum sickness
3. Lesions like these are most characteristic of:
a) Dermatitis herpetiformis
b) Epidermolysis bullosa acquisita
c) Erythema multiforme
d) Bullous pemphigoid
e) Linear IgA bullous dermatosis (LAD)
To learn the answers, go to page 2
{{pagebreak}}
ANSWERS:
1. This patient had low titer antinuclear antibodies and arthralgias. These lesions are:
c) Lupus profundus
Lobular panniculitis may occur with chronic cutaneous or systemic lupus erythematosus; the upper body is commonly involved. Lesions are usually associated with deep dermal and subcutaneous inflammation and subsequent atrophy. The overlying skin may be normal, poikilodermatous, or have features of discoid lupus erythematosus. Characteristic histology is that of a lymphocytic panniculitis with hyalinization of fat lobules and lymphoid nodular structures in the deep dermis and fat. Epithelial changes characteristic of lupus erythematosus may be present and most cases are characterized by immune deposits at the basement membrane zone. Patients with lupus panniculitis usually are associated with relatively benign systemic disease activity. In 2004, a revised “Duesseldorf” classification system for cutaneous lupus erythematosus was published. The classification subdivided cutaneous lupus erythematosus into 4 different categories: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, chronic cutaneous lupus erythematosus, and intermittent cutaneous lupus erythematosus.
References
Sánchez NP, Peters MS, Winkelmann RK. The histopathology of lupus erythematosus panniculitis. J Am Acad Dermatol. 1981;5(6):673-680.
Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin. 2002;20(3):373-385.
Kuhn A, Sontheimer RD. Cutaneous lupus erythematosus: molecular and cellular basis of clinical findings. Curr Dir Autoimmun. 2008;10:119-140.
2. This patient had these recurrent “burning” lesions, arthralgias, elevated sedimentation rate, and reduced total hemolytic complement. Biopsy showed a dermal necrotizing venulitis. The diagnosis is:
c) Urticarial vasculitis
Urticarial vasculitis is a cutaneous necrotizing venulitis which has “urticarial-like” rather than palpable purpuric lesions as its major cutaneous feature. Most patients are young to middle-aged females. The associated signs, symptoms, and serologic abnormalities may include arthralgias, abdominal pain, lymphadenopathy, elevated erythrocyte sedimentation rate, hypocomplementemia, and circulation immune complexes. Some patients have nephritis and pulmonary disease, and some have asthma and hay fever. This type of urticarial differs from nonvasculitic urticaria in that lesions persist for several days, often are characterized by a burning sensation rather than itching, often leave a brownish or bluish discoloration in the skin, and may be purpuric. Angioedema may be present in some patients. All patients with hypocomplementemic urticarial vasculitis should be evaluated for coexistent systemic lupus erythrmatosus or Sjögren syndrome. Drugs are an uncommon cause. Systemic disease is much more prevalent than in patients with nonvasculitic urticaria.
References
Mehregan DR, Hall MJ, Gibson LE. Urticarial vasculitis: a histopathologic and clinical review of 72 cases. J Am Acad Dermatol. 1992;26(3 Pt 2):441-448.
Lotti T, Ghersetich I, Comacchi C, Jorizzo JL. Cutaneous small-vessel vasculitis. J Am Acad Dermatol. 1998;39(5 Pt 1):667-687.
3. Lesions like these are most characteristic of:
e) Linear IgA bullous dermatosis (LAD)
These elongated, annular, and fusiform (or sausage-shaped) blisters are most characteristic of LAD, especially the childhood variant (chronic bullous disease of childhood); they are often referred to as a “cluster of jewels.” The prototypic finding in direct immunofluorescence of perilesional skin is IgA (primarily IgA1 subclass) in continuous, linear, homogenous array along the dermoepidermal junction (not to be confused with the linear granular deposition of IgA seen in rare patients with dermatitis herpetiformis). Infrequently, weaker staining of IgG may also accompany IgA in LAD patients. Circulating IgA antiskin basement membrane autoantibodies are detected in the majority of patients; most of these antibodies bind to the epidermal roof of 1M sodium chloride-split human skin substrate. These autoantibodies react against the 120-kD fragment of an ectodomain of type XVII collagen. A mouse model confirms the pathogenicity of these autoantibodies. Some patients with LAD have lymphoid or nonlymphoid malignancies. A number of drugs have been associated with LAD in adults, most notably vancomycin. Furthermore, there is an association between LAD and the HLA-B8 haplotype.
References
Beutner EH, Chorzelski TP, Kumar V, eds. Immunopathology of the Skin. 3rd ed. New York, NY: John Wiley and Sons; 1987:407-420.
Olasz EB, Yancey KB. Bullous pemphigoid and related subepidermal autoimmune blistering diseases. Curr Dir Autoimmun. 2008;10:141-166.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine served on the faculty of the Departments of Dermatology and Epidemiology in the Schools of Medicine and Public Health at the University of North Carolina at Chapel Hill.
Ron J. Feldman, MD, PhD, is assistant professor of dermatology at Emory University Hospital in Atlanta, GA.
1. This patient had low titer antinuclear antibodies and arthralgias. These lesions are:
a) Discoid lupus erythematosus
b) Morphea
c) Lupus profundus
d) Cutaneous mucinosis secondary to lupus erythematosus
e) Erythema nodosum
2. This patient had these recurrent “burning” lesions, arthralgias, elevated sedimentation rate, and reduced total hemolytic complement. Biopsy showed a dermal necrotizing venulitis. The diagnosis is:
a) Idiopathic chronic urticaria
b) Hypersensitivity angiitis of Zeek
c) Urticarial vasculitis
d) Systemic lupus erythematosus
e) Serum sickness
3. Lesions like these are most characteristic of:
a) Dermatitis herpetiformis
b) Epidermolysis bullosa acquisita
c) Erythema multiforme
d) Bullous pemphigoid
e) Linear IgA bullous dermatosis (LAD)
ANSWERS:
1. This patient had low titer antinuclear antibodies and arthralgias. These lesions are:
c) Lupus profundus
Lobular panniculitis may occur with chronic cutaneous or systemic lupus erythematosus; the upper body is commonly involved. Lesions are usually associated with deep dermal and subcutaneous inflammation and subsequent atrophy. The overlying skin may be normal, poikilodermatous, or have features of discoid lupus erythematosus. Characteristic histology is that of a lymphocytic panniculitis with hyalinization of fat lobules and lymphoid nodular structures in the deep dermis and fat. Epithelial changes characteristic of lupus erythematosus may be present and most cases are characterized by immune deposits at the basement membrane zone. Patients with lupus panniculitis usually are associated with relatively benign systemic disease activity. In 2004, a revised “Duesseldorf” classification system for cutaneous lupus erythematosus was published. The classification subdivided cutaneous lupus erythematosus into 4 different categories: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, chronic cutaneous lupus erythematosus, and intermittent cutaneous lupus erythematosus.
References
Sánchez NP, Peters MS, Winkelmann RK. The histopathology of lupus erythematosus panniculitis. J Am Acad Dermatol. 1981;5(6):673-680.
Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin. 2002;20(3):373-385.
Kuhn A, Sontheimer RD. Cutaneous lupus erythematosus: molecular and cellular basis of clinical findings. Curr Dir Autoimmun. 2008;10:119-140.
2. This patient had these recurrent “burning” lesions, arthralgias, elevated sedimentation rate, and reduced total hemolytic complement. Biopsy showed a dermal necrotizing venulitis. The diagnosis is:
c) Urticarial vasculitis
Urticarial vasculitis is a cutaneous necrotizing venulitis which has “urticarial-like” rather than palpable purpuric lesions as its major cutaneous feature. Most patients are young to middle-aged females. The associated signs, symptoms, and serologic abnormalities may include arthralgias, abdominal pain, lymphadenopathy, elevated erythrocyte sedimentation rate, hypocomplementemia, and circulation immune complexes. Some patients have nephritis and pulmonary disease, and some have asthma and hay fever. This type of urticarial differs from nonvasculitic urticaria in that lesions persist for several days, often are characterized by a burning sensation rather than itching, often leave a brownish or bluish discoloration in the skin, and may be purpuric. Angioedema may be present in some patients. All patients with hypocomplementemic urticarial vasculitis should be evaluated for coexistent systemic lupus erythrmatosus or Sjögren syndrome. Drugs are an uncommon cause. Systemic disease is much more prevalent than in patients with nonvasculitic urticaria.
References
Mehregan DR, Hall MJ, Gibson LE. Urticarial vasculitis: a histopathologic and clinical review of 72 cases. J Am Acad Dermatol. 1992;26(3 Pt 2):441-448.
Lotti T, Ghersetich I, Comacchi C, Jorizzo JL. Cutaneous small-vessel vasculitis. J Am Acad Dermatol. 1998;39(5 Pt 1):667-687.
3. Lesions like these are most characteristic of:
e) Linear IgA bullous dermatosis (LAD)
These elongated, annular, and fusiform (or sausage-shaped) blisters are most characteristic of LAD, especially the childhood variant (chronic bullous disease of childhood); they are often referred to as a “cluster of jewels.” The prototypic finding in direct immunofluorescence of perilesional skin is IgA (primarily IgA1 subclass) in continuous, linear, homogenous array along the dermoepidermal junction (not to be confused with the linear granular deposition of IgA seen in rare patients with dermatitis herpetiformis). Infrequently, weaker staining of IgG may also accompany IgA in LAD patients. Circulating IgA antiskin basement membrane autoantibodies are detected in the majority of patients; most of these antibodies bind to the epidermal roof of 1M sodium chloride-split human skin substrate. These autoantibodies react against the 120-kD fragment of an ectodomain of type XVII collagen. A mouse model confirms the pathogenicity of these autoantibodies. Some patients with LAD have lymphoid or nonlymphoid malignancies. A number of drugs have been associated with LAD in adults, most notably vancomycin. Furthermore, there is an association between LAD and the HLA-B8 haplotype.
References
Beutner EH, Chorzelski TP, Kumar V, eds. Immunopathology of the Skin. 3rd ed. New York, NY: John Wiley and Sons; 1987:407-420.
Olasz EB, Yancey KB. Bullous pemphigoid and related subepidermal autoimmune blistering diseases. Curr Dir Autoimmun. 2008;10:141-166.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine served on the faculty of the Departments of Dermatology and Epidemiology in the Schools of Medicine and Public Health at the University of North Carolina at Chapel Hill.
Ron J. Feldman, MD, PhD, is assistant professor of dermatology at Emory University Hospital in Atlanta, GA.
1. This patient had low titer antinuclear antibodies and arthralgias. These lesions are:
a) Discoid lupus erythematosus
b) Morphea
c) Lupus profundus
d) Cutaneous mucinosis secondary to lupus erythematosus
e) Erythema nodosum
2. This patient had these recurrent “burning” lesions, arthralgias, elevated sedimentation rate, and reduced total hemolytic complement. Biopsy showed a dermal necrotizing venulitis. The diagnosis is:
a) Idiopathic chronic urticaria
b) Hypersensitivity angiitis of Zeek
c) Urticarial vasculitis
d) Systemic lupus erythematosus
e) Serum sickness
3. Lesions like these are most characteristic of:
a) Dermatitis herpetiformis
b) Epidermolysis bullosa acquisita
c) Erythema multiforme
d) Bullous pemphigoid
e) Linear IgA bullous dermatosis (LAD)
,
1. This patient had low titer antinuclear antibodies and arthralgias. These lesions are:
a) Discoid lupus erythematosus
b) Morphea
c) Lupus profundus
d) Cutaneous mucinosis secondary to lupus erythematosus
e) Erythema nodosum
2. This patient had these recurrent “burning” lesions, arthralgias, elevated sedimentation rate, and reduced total hemolytic complement. Biopsy showed a dermal necrotizing venulitis. The diagnosis is:
a) Idiopathic chronic urticaria
b) Hypersensitivity angiitis of Zeek
c) Urticarial vasculitis
d) Systemic lupus erythematosus
e) Serum sickness
3. Lesions like these are most characteristic of:
a) Dermatitis herpetiformis
b) Epidermolysis bullosa acquisita
c) Erythema multiforme
d) Bullous pemphigoid
e) Linear IgA bullous dermatosis (LAD)
To learn the answers, go to page 2
{{pagebreak}}
ANSWERS:
1. This patient had low titer antinuclear antibodies and arthralgias. These lesions are:
c) Lupus profundus
Lobular panniculitis may occur with chronic cutaneous or systemic lupus erythematosus; the upper body is commonly involved. Lesions are usually associated with deep dermal and subcutaneous inflammation and subsequent atrophy. The overlying skin may be normal, poikilodermatous, or have features of discoid lupus erythematosus. Characteristic histology is that of a lymphocytic panniculitis with hyalinization of fat lobules and lymphoid nodular structures in the deep dermis and fat. Epithelial changes characteristic of lupus erythematosus may be present and most cases are characterized by immune deposits at the basement membrane zone. Patients with lupus panniculitis usually are associated with relatively benign systemic disease activity. In 2004, a revised “Duesseldorf” classification system for cutaneous lupus erythematosus was published. The classification subdivided cutaneous lupus erythematosus into 4 different categories: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, chronic cutaneous lupus erythematosus, and intermittent cutaneous lupus erythematosus.
References
Sánchez NP, Peters MS, Winkelmann RK. The histopathology of lupus erythematosus panniculitis. J Am Acad Dermatol. 1981;5(6):673-680.
Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin. 2002;20(3):373-385.
Kuhn A, Sontheimer RD. Cutaneous lupus erythematosus: molecular and cellular basis of clinical findings. Curr Dir Autoimmun. 2008;10:119-140.
2. This patient had these recurrent “burning” lesions, arthralgias, elevated sedimentation rate, and reduced total hemolytic complement. Biopsy showed a dermal necrotizing venulitis. The diagnosis is:
c) Urticarial vasculitis
Urticarial vasculitis is a cutaneous necrotizing venulitis which has “urticarial-like” rather than palpable purpuric lesions as its major cutaneous feature. Most patients are young to middle-aged females. The associated signs, symptoms, and serologic abnormalities may include arthralgias, abdominal pain, lymphadenopathy, elevated erythrocyte sedimentation rate, hypocomplementemia, and circulation immune complexes. Some patients have nephritis and pulmonary disease, and some have asthma and hay fever. This type of urticarial differs from nonvasculitic urticaria in that lesions persist for several days, often are characterized by a burning sensation rather than itching, often leave a brownish or bluish discoloration in the skin, and may be purpuric. Angioedema may be present in some patients. All patients with hypocomplementemic urticarial vasculitis should be evaluated for coexistent systemic lupus erythrmatosus or Sjögren syndrome. Drugs are an uncommon cause. Systemic disease is much more prevalent than in patients with nonvasculitic urticaria.
References
Mehregan DR, Hall MJ, Gibson LE. Urticarial vasculitis: a histopathologic and clinical review of 72 cases. J Am Acad Dermatol. 1992;26(3 Pt 2):441-448.
Lotti T, Ghersetich I, Comacchi C, Jorizzo JL. Cutaneous small-vessel vasculitis. J Am Acad Dermatol. 1998;39(5 Pt 1):667-687.
3. Lesions like these are most characteristic of:
e) Linear IgA bullous dermatosis (LAD)
These elongated, annular, and fusiform (or sausage-shaped) blisters are most characteristic of LAD, especially the childhood variant (chronic bullous disease of childhood); they are often referred to as a “cluster of jewels.” The prototypic finding in direct immunofluorescence of perilesional skin is IgA (primarily IgA1 subclass) in continuous, linear, homogenous array along the dermoepidermal junction (not to be confused with the linear granular deposition of IgA seen in rare patients with dermatitis herpetiformis). Infrequently, weaker staining of IgG may also accompany IgA in LAD patients. Circulating IgA antiskin basement membrane autoantibodies are detected in the majority of patients; most of these antibodies bind to the epidermal roof of 1M sodium chloride-split human skin substrate. These autoantibodies react against the 120-kD fragment of an ectodomain of type XVII collagen. A mouse model confirms the pathogenicity of these autoantibodies. Some patients with LAD have lymphoid or nonlymphoid malignancies. A number of drugs have been associated with LAD in adults, most notably vancomycin. Furthermore, there is an association between LAD and the HLA-B8 haplotype.
References
Beutner EH, Chorzelski TP, Kumar V, eds. Immunopathology of the Skin. 3rd ed. New York, NY: John Wiley and Sons; 1987:407-420.
Olasz EB, Yancey KB. Bullous pemphigoid and related subepidermal autoimmune blistering diseases. Curr Dir Autoimmun. 2008;10:141-166.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine served on the faculty of the Departments of Dermatology and Epidemiology in the Schools of Medicine and Public Health at the University of North Carolina at Chapel Hill.
Ron J. Feldman, MD, PhD, is assistant professor of dermatology at Emory University Hospital in Atlanta, GA.
1. This patient had low titer antinuclear antibodies and arthralgias. These lesions are:
a) Discoid lupus erythematosus
b) Morphea
c) Lupus profundus
d) Cutaneous mucinosis secondary to lupus erythematosus
e) Erythema nodosum
2. This patient had these recurrent “burning” lesions, arthralgias, elevated sedimentation rate, and reduced total hemolytic complement. Biopsy showed a dermal necrotizing venulitis. The diagnosis is:
a) Idiopathic chronic urticaria
b) Hypersensitivity angiitis of Zeek
c) Urticarial vasculitis
d) Systemic lupus erythematosus
e) Serum sickness
3. Lesions like these are most characteristic of:
a) Dermatitis herpetiformis
b) Epidermolysis bullosa acquisita
c) Erythema multiforme
d) Bullous pemphigoid
e) Linear IgA bullous dermatosis (LAD)
ANSWERS:
1. This patient had low titer antinuclear antibodies and arthralgias. These lesions are:
c) Lupus profundus
Lobular panniculitis may occur with chronic cutaneous or systemic lupus erythematosus; the upper body is commonly involved. Lesions are usually associated with deep dermal and subcutaneous inflammation and subsequent atrophy. The overlying skin may be normal, poikilodermatous, or have features of discoid lupus erythematosus. Characteristic histology is that of a lymphocytic panniculitis with hyalinization of fat lobules and lymphoid nodular structures in the deep dermis and fat. Epithelial changes characteristic of lupus erythematosus may be present and most cases are characterized by immune deposits at the basement membrane zone. Patients with lupus panniculitis usually are associated with relatively benign systemic disease activity. In 2004, a revised “Duesseldorf” classification system for cutaneous lupus erythematosus was published. The classification subdivided cutaneous lupus erythematosus into 4 different categories: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, chronic cutaneous lupus erythematosus, and intermittent cutaneous lupus erythematosus.
References
Sánchez NP, Peters MS, Winkelmann RK. The histopathology of lupus erythematosus panniculitis. J Am Acad Dermatol. 1981;5(6):673-680.
Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin. 2002;20(3):373-385.
Kuhn A, Sontheimer RD. Cutaneous lupus erythematosus: molecular and cellular basis of clinical findings. Curr Dir Autoimmun. 2008;10:119-140.
2. This patient had these recurrent “burning” lesions, arthralgias, elevated sedimentation rate, and reduced total hemolytic complement. Biopsy showed a dermal necrotizing venulitis. The diagnosis is:
c) Urticarial vasculitis
Urticarial vasculitis is a cutaneous necrotizing venulitis which has “urticarial-like” rather than palpable purpuric lesions as its major cutaneous feature. Most patients are young to middle-aged females. The associated signs, symptoms, and serologic abnormalities may include arthralgias, abdominal pain, lymphadenopathy, elevated erythrocyte sedimentation rate, hypocomplementemia, and circulation immune complexes. Some patients have nephritis and pulmonary disease, and some have asthma and hay fever. This type of urticarial differs from nonvasculitic urticaria in that lesions persist for several days, often are characterized by a burning sensation rather than itching, often leave a brownish or bluish discoloration in the skin, and may be purpuric. Angioedema may be present in some patients. All patients with hypocomplementemic urticarial vasculitis should be evaluated for coexistent systemic lupus erythrmatosus or Sjögren syndrome. Drugs are an uncommon cause. Systemic disease is much more prevalent than in patients with nonvasculitic urticaria.
References
Mehregan DR, Hall MJ, Gibson LE. Urticarial vasculitis: a histopathologic and clinical review of 72 cases. J Am Acad Dermatol. 1992;26(3 Pt 2):441-448.
Lotti T, Ghersetich I, Comacchi C, Jorizzo JL. Cutaneous small-vessel vasculitis. J Am Acad Dermatol. 1998;39(5 Pt 1):667-687.
3. Lesions like these are most characteristic of:
e) Linear IgA bullous dermatosis (LAD)
These elongated, annular, and fusiform (or sausage-shaped) blisters are most characteristic of LAD, especially the childhood variant (chronic bullous disease of childhood); they are often referred to as a “cluster of jewels.” The prototypic finding in direct immunofluorescence of perilesional skin is IgA (primarily IgA1 subclass) in continuous, linear, homogenous array along the dermoepidermal junction (not to be confused with the linear granular deposition of IgA seen in rare patients with dermatitis herpetiformis). Infrequently, weaker staining of IgG may also accompany IgA in LAD patients. Circulating IgA antiskin basement membrane autoantibodies are detected in the majority of patients; most of these antibodies bind to the epidermal roof of 1M sodium chloride-split human skin substrate. These autoantibodies react against the 120-kD fragment of an ectodomain of type XVII collagen. A mouse model confirms the pathogenicity of these autoantibodies. Some patients with LAD have lymphoid or nonlymphoid malignancies. A number of drugs have been associated with LAD in adults, most notably vancomycin. Furthermore, there is an association between LAD and the HLA-B8 haplotype.
References
Beutner EH, Chorzelski TP, Kumar V, eds. Immunopathology of the Skin. 3rd ed. New York, NY: John Wiley and Sons; 1987:407-420.
Olasz EB, Yancey KB. Bullous pemphigoid and related subepidermal autoimmune blistering diseases. Curr Dir Autoimmun. 2008;10:141-166.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine served on the faculty of the Departments of Dermatology and Epidemiology in the Schools of Medicine and Public Health at the University of North Carolina at Chapel Hill.
Ron J. Feldman, MD, PhD, is assistant professor of dermatology at Emory University Hospital in Atlanta, GA.
ANSWERS:
1. This patient had low titer antinuclear antibodies and arthralgias. These lesions are:
c) Lupus profundus
Lobular panniculitis may occur with chronic cutaneous or systemic lupus erythematosus; the upper body is commonly involved. Lesions are usually associated with deep dermal and subcutaneous inflammation and subsequent atrophy. The overlying skin may be normal, poikilodermatous, or have features of discoid lupus erythematosus. Characteristic histology is that of a lymphocytic panniculitis with hyalinization of fat lobules and lymphoid nodular structures in the deep dermis and fat. Epithelial changes characteristic of lupus erythematosus may be present and most cases are characterized by immune deposits at the basement membrane zone. Patients with lupus panniculitis usually are associated with relatively benign systemic disease activity. In 2004, a revised “Duesseldorf” classification system for cutaneous lupus erythematosus was published. The classification subdivided cutaneous lupus erythematosus into 4 different categories: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, chronic cutaneous lupus erythematosus, and intermittent cutaneous lupus erythematosus.
References
Sánchez NP, Peters MS, Winkelmann RK. The histopathology of lupus erythematosus panniculitis. J Am Acad Dermatol. 1981;5(6):673-680.
Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin. 2002;20(3):373-385.
Kuhn A, Sontheimer RD. Cutaneous lupus erythematosus: molecular and cellular basis of clinical findings. Curr Dir Autoimmun. 2008;10:119-140.
2. This patient had these recurrent “burning” lesions, arthralgias, elevated sedimentation rate, and reduced total hemolytic complement. Biopsy showed a dermal necrotizing venulitis. The diagnosis is:
c) Urticarial vasculitis
Urticarial vasculitis is a cutaneous necrotizing venulitis which has “urticarial-like” rather than palpable purpuric lesions as its major cutaneous feature. Most patients are young to middle-aged females. The associated signs, symptoms, and serologic abnormalities may include arthralgias, abdominal pain, lymphadenopathy, elevated erythrocyte sedimentation rate, hypocomplementemia, and circulation immune complexes. Some patients have nephritis and pulmonary disease, and some have asthma and hay fever. This type of urticarial differs from nonvasculitic urticaria in that lesions persist for several days, often are characterized by a burning sensation rather than itching, often leave a brownish or bluish discoloration in the skin, and may be purpuric. Angioedema may be present in some patients. All patients with hypocomplementemic urticarial vasculitis should be evaluated for coexistent systemic lupus erythrmatosus or Sjögren syndrome. Drugs are an uncommon cause. Systemic disease is much more prevalent than in patients with nonvasculitic urticaria.
References
Mehregan DR, Hall MJ, Gibson LE. Urticarial vasculitis: a histopathologic and clinical review of 72 cases. J Am Acad Dermatol. 1992;26(3 Pt 2):441-448.
Lotti T, Ghersetich I, Comacchi C, Jorizzo JL. Cutaneous small-vessel vasculitis. J Am Acad Dermatol. 1998;39(5 Pt 1):667-687.
3. Lesions like these are most characteristic of:
e) Linear IgA bullous dermatosis (LAD)
These elongated, annular, and fusiform (or sausage-shaped) blisters are most characteristic of LAD, especially the childhood variant (chronic bullous disease of childhood); they are often referred to as a “cluster of jewels.” The prototypic finding in direct immunofluorescence of perilesional skin is IgA (primarily IgA1 subclass) in continuous, linear, homogenous array along the dermoepidermal junction (not to be confused with the linear granular deposition of IgA seen in rare patients with dermatitis herpetiformis). Infrequently, weaker staining of IgG may also accompany IgA in LAD patients. Circulating IgA antiskin basement membrane autoantibodies are detected in the majority of patients; most of these antibodies bind to the epidermal roof of 1M sodium chloride-split human skin substrate. These autoantibodies react against the 120-kD fragment of an ectodomain of type XVII collagen. A mouse model confirms the pathogenicity of these autoantibodies. Some patients with LAD have lymphoid or nonlymphoid malignancies. A number of drugs have been associated with LAD in adults, most notably vancomycin. Furthermore, there is an association between LAD and the HLA-B8 haplotype.
References
Beutner EH, Chorzelski TP, Kumar V, eds. Immunopathology of the Skin. 3rd ed. New York, NY: John Wiley and Sons; 1987:407-420.
Olasz EB, Yancey KB. Bullous pemphigoid and related subepidermal autoimmune blistering diseases. Curr Dir Autoimmun. 2008;10:141-166.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine served on the faculty of the Departments of Dermatology and Epidemiology in the Schools of Medicine and Public Health at the University of North Carolina at Chapel Hill.
Ron J. Feldman, MD, PhD, is assistant professor of dermatology at Emory University Hospital in Atlanta, GA.
