1.This lesion developed 24 to 48 hours after beginning warfarin therapy for recurrent thromboembolic episodes. There was a positive family history of recurrent pulmonary embolism. Which one of the following statements is true of this condition?
a) The disorder is recessively inherited
b) The disorder is due to a complete deficiency of protein C production
c) The disorder is due to a partial deficiency of protein C production
d) Lesions are usually due to arterial thrombosis
e) Patient can never take warfarin again
2. This histology suggests a diagnosis of:
a) Pemphigus foliaceus
b) IgA pemphigus
c) Pemiphgus vulgaris
d) Familial benign pemphigus (Hailey-Hailey disease)
e) Subcorneal pustular dermatosis
BOARD REVIEW ANSWERS
1. This lesion developed 24 to 48 hours after beginning warfarin therapy for recurrent thromboembolic episodes. There was a positive family history of recurrent pulmonary embolism. Which one of the following statements is true of this condition?
c) The disorder is due to a partial deficiency of protein C production
This patient has warfarin or coumarin-induced skin necrosis due to partial deficiency of protein C. Partial or heterozygous protein C deficiency is dominantly inherited and characterized clinically by recurrent thromboembolic disease which usually begins in early or middle adult life and may follow trauma. Homozygous protein C deficiency (recessive) is characterized by overwhelming thrombosis and embolism leading to death in infancy. Anticoagulation with warfarin is the long-term treatment of choice, but initiation of therapy may cause thrombosis. This complication may be preventable with heparin and/or fresh frozen plasma administered during initiation of therapy. The mechanism of warfarin necrosis in protein C deficiency is thought to be due to the fact that warfarin causes a faster drop in protein C (anticoagulant) levels than in levels of the vitamin K dependent procoagulants, factor II, IX, and X, resulting in transient hypercoagulable state. Veins are the usual site of thrombosis but arteries may be involved.
References
Essex DW, Wynn SS, Jin DK. Late-onset warfarin-induced skin necrosis: case report and review of the literature. Am J Hematol. 1998;57(3):233-237.
Segel GB, Francis CA. Anticoagulant proteins in childhood venous and arterial thrombosis: a review. Blood Cells Mol Dis. 2000;26(5):540-560.
Chan YC, Valenti D, Mansfield AO, Stansby G. Warfarin induced skin necrosis. Br J Surg. 2000;87(3):266-272.
Kottke-Marchant K, Comp P. Laboratory issues in diagnosing abnormalities of protein C, thrombomodulin, and endothelial cell protein C receptor. Arch Pathol Lab Med. 2002;126(11):1337-1348.
2. This histology suggests a diagnosis of:
a) Pemphigus foliaceus
Granular layer vesicle formation with acantholysis is characteristic of pemphigus foliaceus and pemphigus erythematosus (Senear-Usher syndrome). It is also seen in IgA pemphigus and subcorneal pustular dermatosis; however, the latter disorders often demonstrate an acute neutrophil-predominant infiltrate within the dermis and epidermis.
Reference
Lever WF, Schaumburg-Lever G. Histopathology of the Skin. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1990:122-125.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine is currently professor of medicine (dermatology) and pediatrics at Vanderbilt University School of Medicine in Nashville, TN.
Ron J. Feldman, MD, PhD, is assistant professor in the department of dermatology at Emory University School of Medicine in Atlanta, GA.
1.This lesion developed 24 to 48 hours after beginning warfarin therapy for recurrent thromboembolic episodes. There was a positive family history of recurrent pulmonary embolism. Which one of the following statements is true of this condition?
a) The disorder is recessively inherited
b) The disorder is due to a complete deficiency of protein C production
c) The disorder is due to a partial deficiency of protein C production
d) Lesions are usually due to arterial thrombosis
e) Patient can never take warfarin again
2. This histology suggests a diagnosis of:
a) Pemphigus foliaceus
b) IgA pemphigus
c) Pemiphgus vulgaris
d) Familial benign pemphigus (Hailey-Hailey disease)
e) Subcorneal pustular dermatosis
BOARD REVIEW ANSWERS
1. This lesion developed 24 to 48 hours after beginning warfarin therapy for recurrent thromboembolic episodes. There was a positive family history of recurrent pulmonary embolism. Which one of the following statements is true of this condition?
c) The disorder is due to a partial deficiency of protein C production
This patient has warfarin or coumarin-induced skin necrosis due to partial deficiency of protein C. Partial or heterozygous protein C deficiency is dominantly inherited and characterized clinically by recurrent thromboembolic disease which usually begins in early or middle adult life and may follow trauma. Homozygous protein C deficiency (recessive) is characterized by overwhelming thrombosis and embolism leading to death in infancy. Anticoagulation with warfarin is the long-term treatment of choice, but initiation of therapy may cause thrombosis. This complication may be preventable with heparin and/or fresh frozen plasma administered during initiation of therapy. The mechanism of warfarin necrosis in protein C deficiency is thought to be due to the fact that warfarin causes a faster drop in protein C (anticoagulant) levels than in levels of the vitamin K dependent procoagulants, factor II, IX, and X, resulting in transient hypercoagulable state. Veins are the usual site of thrombosis but arteries may be involved.
References
Essex DW, Wynn SS, Jin DK. Late-onset warfarin-induced skin necrosis: case report and review of the literature. Am J Hematol. 1998;57(3):233-237.
Segel GB, Francis CA. Anticoagulant proteins in childhood venous and arterial thrombosis: a review. Blood Cells Mol Dis. 2000;26(5):540-560.
Chan YC, Valenti D, Mansfield AO, Stansby G. Warfarin induced skin necrosis. Br J Surg. 2000;87(3):266-272.
Kottke-Marchant K, Comp P. Laboratory issues in diagnosing abnormalities of protein C, thrombomodulin, and endothelial cell protein C receptor. Arch Pathol Lab Med. 2002;126(11):1337-1348.
2. This histology suggests a diagnosis of:
a) Pemphigus foliaceus
Granular layer vesicle formation with acantholysis is characteristic of pemphigus foliaceus and pemphigus erythematosus (Senear-Usher syndrome). It is also seen in IgA pemphigus and subcorneal pustular dermatosis; however, the latter disorders often demonstrate an acute neutrophil-predominant infiltrate within the dermis and epidermis.
Reference
Lever WF, Schaumburg-Lever G. Histopathology of the Skin. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1990:122-125.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine is currently professor of medicine (dermatology) and pediatrics at Vanderbilt University School of Medicine in Nashville, TN.
Ron J. Feldman, MD, PhD, is assistant professor in the department of dermatology at Emory University School of Medicine in Atlanta, GA.
1.This lesion developed 24 to 48 hours after beginning warfarin therapy for recurrent thromboembolic episodes. There was a positive family history of recurrent pulmonary embolism. Which one of the following statements is true of this condition?
a) The disorder is recessively inherited
b) The disorder is due to a complete deficiency of protein C production
c) The disorder is due to a partial deficiency of protein C production
d) Lesions are usually due to arterial thrombosis
e) Patient can never take warfarin again
2. This histology suggests a diagnosis of:
a) Pemphigus foliaceus
b) IgA pemphigus
c) Pemiphgus vulgaris
d) Familial benign pemphigus (Hailey-Hailey disease)
e) Subcorneal pustular dermatosis
,
1.This lesion developed 24 to 48 hours after beginning warfarin therapy for recurrent thromboembolic episodes. There was a positive family history of recurrent pulmonary embolism. Which one of the following statements is true of this condition?
a) The disorder is recessively inherited
b) The disorder is due to a complete deficiency of protein C production
c) The disorder is due to a partial deficiency of protein C production
d) Lesions are usually due to arterial thrombosis
e) Patient can never take warfarin again
2. This histology suggests a diagnosis of:
a) Pemphigus foliaceus
b) IgA pemphigus
c) Pemiphgus vulgaris
d) Familial benign pemphigus (Hailey-Hailey disease)
e) Subcorneal pustular dermatosis
BOARD REVIEW ANSWERS
1. This lesion developed 24 to 48 hours after beginning warfarin therapy for recurrent thromboembolic episodes. There was a positive family history of recurrent pulmonary embolism. Which one of the following statements is true of this condition?
c) The disorder is due to a partial deficiency of protein C production
This patient has warfarin or coumarin-induced skin necrosis due to partial deficiency of protein C. Partial or heterozygous protein C deficiency is dominantly inherited and characterized clinically by recurrent thromboembolic disease which usually begins in early or middle adult life and may follow trauma. Homozygous protein C deficiency (recessive) is characterized by overwhelming thrombosis and embolism leading to death in infancy. Anticoagulation with warfarin is the long-term treatment of choice, but initiation of therapy may cause thrombosis. This complication may be preventable with heparin and/or fresh frozen plasma administered during initiation of therapy. The mechanism of warfarin necrosis in protein C deficiency is thought to be due to the fact that warfarin causes a faster drop in protein C (anticoagulant) levels than in levels of the vitamin K dependent procoagulants, factor II, IX, and X, resulting in transient hypercoagulable state. Veins are the usual site of thrombosis but arteries may be involved.
References
Essex DW, Wynn SS, Jin DK. Late-onset warfarin-induced skin necrosis: case report and review of the literature. Am J Hematol. 1998;57(3):233-237.
Segel GB, Francis CA. Anticoagulant proteins in childhood venous and arterial thrombosis: a review. Blood Cells Mol Dis. 2000;26(5):540-560.
Chan YC, Valenti D, Mansfield AO, Stansby G. Warfarin induced skin necrosis. Br J Surg. 2000;87(3):266-272.
Kottke-Marchant K, Comp P. Laboratory issues in diagnosing abnormalities of protein C, thrombomodulin, and endothelial cell protein C receptor. Arch Pathol Lab Med. 2002;126(11):1337-1348.
2. This histology suggests a diagnosis of:
a) Pemphigus foliaceus
Granular layer vesicle formation with acantholysis is characteristic of pemphigus foliaceus and pemphigus erythematosus (Senear-Usher syndrome). It is also seen in IgA pemphigus and subcorneal pustular dermatosis; however, the latter disorders often demonstrate an acute neutrophil-predominant infiltrate within the dermis and epidermis.
Reference
Lever WF, Schaumburg-Lever G. Histopathology of the Skin. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1990:122-125.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine is currently professor of medicine (dermatology) and pediatrics at Vanderbilt University School of Medicine in Nashville, TN.
Ron J. Feldman, MD, PhD, is assistant professor in the department of dermatology at Emory University School of Medicine in Atlanta, GA.
1.This lesion developed 24 to 48 hours after beginning warfarin therapy for recurrent thromboembolic episodes. There was a positive family history of recurrent pulmonary embolism. Which one of the following statements is true of this condition?
a) The disorder is recessively inherited
b) The disorder is due to a complete deficiency of protein C production
c) The disorder is due to a partial deficiency of protein C production
d) Lesions are usually due to arterial thrombosis
e) Patient can never take warfarin again
2. This histology suggests a diagnosis of:
a) Pemphigus foliaceus
b) IgA pemphigus
c) Pemiphgus vulgaris
d) Familial benign pemphigus (Hailey-Hailey disease)
e) Subcorneal pustular dermatosis
BOARD REVIEW ANSWERS
1. This lesion developed 24 to 48 hours after beginning warfarin therapy for recurrent thromboembolic episodes. There was a positive family history of recurrent pulmonary embolism. Which one of the following statements is true of this condition?
c) The disorder is due to a partial deficiency of protein C production
This patient has warfarin or coumarin-induced skin necrosis due to partial deficiency of protein C. Partial or heterozygous protein C deficiency is dominantly inherited and characterized clinically by recurrent thromboembolic disease which usually begins in early or middle adult life and may follow trauma. Homozygous protein C deficiency (recessive) is characterized by overwhelming thrombosis and embolism leading to death in infancy. Anticoagulation with warfarin is the long-term treatment of choice, but initiation of therapy may cause thrombosis. This complication may be preventable with heparin and/or fresh frozen plasma administered during initiation of therapy. The mechanism of warfarin necrosis in protein C deficiency is thought to be due to the fact that warfarin causes a faster drop in protein C (anticoagulant) levels than in levels of the vitamin K dependent procoagulants, factor II, IX, and X, resulting in transient hypercoagulable state. Veins are the usual site of thrombosis but arteries may be involved.
References
Essex DW, Wynn SS, Jin DK. Late-onset warfarin-induced skin necrosis: case report and review of the literature. Am J Hematol. 1998;57(3):233-237.
Segel GB, Francis CA. Anticoagulant proteins in childhood venous and arterial thrombosis: a review. Blood Cells Mol Dis. 2000;26(5):540-560.
Chan YC, Valenti D, Mansfield AO, Stansby G. Warfarin induced skin necrosis. Br J Surg. 2000;87(3):266-272.
Kottke-Marchant K, Comp P. Laboratory issues in diagnosing abnormalities of protein C, thrombomodulin, and endothelial cell protein C receptor. Arch Pathol Lab Med. 2002;126(11):1337-1348.
2. This histology suggests a diagnosis of:
a) Pemphigus foliaceus
Granular layer vesicle formation with acantholysis is characteristic of pemphigus foliaceus and pemphigus erythematosus (Senear-Usher syndrome). It is also seen in IgA pemphigus and subcorneal pustular dermatosis; however, the latter disorders often demonstrate an acute neutrophil-predominant infiltrate within the dermis and epidermis.
Reference
Lever WF, Schaumburg-Lever G. Histopathology of the Skin. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1990:122-125.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine is currently professor of medicine (dermatology) and pediatrics at Vanderbilt University School of Medicine in Nashville, TN.
Ron J. Feldman, MD, PhD, is assistant professor in the department of dermatology at Emory University School of Medicine in Atlanta, GA.
BOARD REVIEW ANSWERS
1. This lesion developed 24 to 48 hours after beginning warfarin therapy for recurrent thromboembolic episodes. There was a positive family history of recurrent pulmonary embolism. Which one of the following statements is true of this condition?
c) The disorder is due to a partial deficiency of protein C production
This patient has warfarin or coumarin-induced skin necrosis due to partial deficiency of protein C. Partial or heterozygous protein C deficiency is dominantly inherited and characterized clinically by recurrent thromboembolic disease which usually begins in early or middle adult life and may follow trauma. Homozygous protein C deficiency (recessive) is characterized by overwhelming thrombosis and embolism leading to death in infancy. Anticoagulation with warfarin is the long-term treatment of choice, but initiation of therapy may cause thrombosis. This complication may be preventable with heparin and/or fresh frozen plasma administered during initiation of therapy. The mechanism of warfarin necrosis in protein C deficiency is thought to be due to the fact that warfarin causes a faster drop in protein C (anticoagulant) levels than in levels of the vitamin K dependent procoagulants, factor II, IX, and X, resulting in transient hypercoagulable state. Veins are the usual site of thrombosis but arteries may be involved.
References
Essex DW, Wynn SS, Jin DK. Late-onset warfarin-induced skin necrosis: case report and review of the literature. Am J Hematol. 1998;57(3):233-237.
Segel GB, Francis CA. Anticoagulant proteins in childhood venous and arterial thrombosis: a review. Blood Cells Mol Dis. 2000;26(5):540-560.
Chan YC, Valenti D, Mansfield AO, Stansby G. Warfarin induced skin necrosis. Br J Surg. 2000;87(3):266-272.
Kottke-Marchant K, Comp P. Laboratory issues in diagnosing abnormalities of protein C, thrombomodulin, and endothelial cell protein C receptor. Arch Pathol Lab Med. 2002;126(11):1337-1348.
2. This histology suggests a diagnosis of:
a) Pemphigus foliaceus
Granular layer vesicle formation with acantholysis is characteristic of pemphigus foliaceus and pemphigus erythematosus (Senear-Usher syndrome). It is also seen in IgA pemphigus and subcorneal pustular dermatosis; however, the latter disorders often demonstrate an acute neutrophil-predominant infiltrate within the dermis and epidermis.
Reference
Lever WF, Schaumburg-Lever G. Histopathology of the Skin. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1990:122-125.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine is currently professor of medicine (dermatology) and pediatrics at Vanderbilt University School of Medicine in Nashville, TN.
Ron J. Feldman, MD, PhD, is assistant professor in the department of dermatology at Emory University School of Medicine in Atlanta, GA.
