A 76-year-old woman is brought to the physician because of lesions on her left arm. She first noticed them 3 months ago and they have grown larger since that time. She has not had any pain or pruritus in the area. She has a history of invasive ductal carcinoma of the left breast, which was treated with mastectomy and radiation therapy 27 years ago. Since that time, she has had lymphedema of the left arm. Physical examination shows extensive edema of the left arm. There are four coalescing, firm, purple-blue nodules on the left lateral axillary region and swelling of the surrounding skin. Which of the following is the most likely diagnosis?
Q2
A 45-year-old man comes to the emergency department because of chills and numerous skin lesions for 1 week. He has also had watery diarrhea, nausea, and abdominal pain for the past 2 weeks. The skin lesions are nonpruritic and painless. He was diagnosed with HIV infection approximately 20 years ago. He has not taken any medications for over 5 years. He sleeps in homeless shelters and parks. Vital signs are within normal limits. Examination shows several bright red, friable nodules on his face, trunk, extremities. The liver is palpated 3 cm below the right costal margin. His CD4+ T-lymphocyte count is 180/mm3 (N ≥ 500). A rapid plasma reagin test is negative. Abdominal ultrasonography shows hepatomegaly and a single intrahepatic 1.0 x 1.2-cm hypodense lesion. Biopsy of a skin lesion shows vascular proliferation and abundant neutrophils. Which of the following is the most likely causal organism?
Q3
A 93-year-old woman is brought to the physician because of a purple area on her right arm that has been growing for one month. She has not had any pain or itching of the area. She has hyperlipidemia, a history of basal cell carcinoma treated with Mohs surgery 2 years ago, and a history of invasive ductal carcinoma of the right breast treated with radical mastectomy 57 years ago. She has had chronic lymphedema of the right upper extremity since the mastectomy. Her only medication is simvastatin. She lives in an assisted living facility. She is content with her living arrangement but feels guilty that she is dependent on others. Vital signs are within normal limits. Physical examination shows extensive edema of the right arm. Skin exam of the proximal upper right extremity shows three coalescing, 0.5–1.0 cm heterogeneous, purple-colored plaques with associated ulceration. Which of the following is the most likely diagnosis?
Q4
A 49-year-old man with HIV comes to the physician because of a 1-month history of intermittent diarrhea and abdominal pain. Abdominal examination shows mild, diffuse tenderness throughout the lower quadrants. His CD4+ T-lymphocyte count is 180/mm3 (normal ≥ 500/mm3). Colonoscopy shows multiple hemorrhagic nodules in the rectum and descending colon. Polymerase chain reaction of the lesions is positive for HHV-8. Histologic examination of the lesions is most likely to show which of the following findings?
Q5
Two weeks after undergoing low anterior resection for rectal cancer, a 52-year-old man comes to the physician because of swelling in both feet. He has not had any fever, chills, or shortness of breath. His temperature is 36°C (96.8°F) and pulse is 88/min. Physical examination shows a normal thyroid and no jugular venous distention. Examination of the lower extremities shows bilateral non-pitting edema that extends from the feet to the lower thigh, with deep flexion creases. His skin is warm and dry, and there is no erythema or rash. Microscopic examination of the interstitial space in this patient's lower extremities would be most likely to show the presence of which of the following?
Q6
A 28-year-old man presents for severe abdominal pain and is diagnosed with appendicitis. He is taken for emergent appendectomy. During the procedure, the patient has massive and persistent bleeding requiring a blood transfusion. The preoperative laboratory studies showed a normal bleeding time, normal prothrombin time (PT), an INR of 1.0, and a normal platelet count. Postoperatively, when the patient is told about the complications during the surgery, he recalls that he forgot to mention that he has a family history of an unknown bleeding disorder. The postoperative laboratory tests reveal a prolonged partial thromboplastin time (PTT). Which of the following is the most likely diagnosis in this patient?
Q7
A previously healthy 4-year-old boy is brought to the emergency department because of a 1-day history of pain and swelling of his left knee joint. He has not had any trauma to the knee. His family history is unremarkable except for a bleeding disorder in his maternal uncle. His temperature is 36.9°C (98.4°F). The left knee is erythematous, swollen, and tender; range of motion is limited. No other joints are affected. An x-ray of the knee shows an effusion but no structural abnormalities of the joint. Arthrocentesis is conducted. The synovial fluid is bloody. Further evaluation of this patient is most likely to show which of the following findings?
Q8
A 50-year-old woman with rheumatoid arthritis on methotrexate develops rapidly progressive painful ulcers on her legs with violaceous undermined borders. Biopsy shows neutrophilic dermal infiltrate with areas of necrosis, but no vasculitis or infection. Wound cultures are negative. Despite debridement, the ulcers worsen. C-ANCA and P-ANCA are negative. Evaluate the diagnosis and determine the management that addresses both the cutaneous condition and systemic disease.
Q9
A 25-year-old woman presents with painful oral ulcers and a pustular rash at venipuncture sites. She has genital ulcers and a history of recurrent uveitis. Skin biopsy from a pustule shows neutrophilic infiltrate in the dermis without vasculitis or infection. HLA-B51 testing is positive. She is planning pregnancy. Evaluate the management strategy considering disease control and pregnancy planning.
Q10
A 70-year-old man on chronic warfarin therapy presents with sudden onset of painful purpura on his thighs and buttocks three days after starting warfarin for atrial fibrillation. He has a history of multiple DVTs. Skin biopsy shows thrombosis of dermal blood vessels with minimal inflammation. Laboratory studies show an INR of 3.5. Evaluate the pathophysiology and determine the most appropriate immediate management.
Vascular disorders of skin US Medical PG Practice Questions and MCQs
Question 1: A 76-year-old woman is brought to the physician because of lesions on her left arm. She first noticed them 3 months ago and they have grown larger since that time. She has not had any pain or pruritus in the area. She has a history of invasive ductal carcinoma of the left breast, which was treated with mastectomy and radiation therapy 27 years ago. Since that time, she has had lymphedema of the left arm. Physical examination shows extensive edema of the left arm. There are four coalescing, firm, purple-blue nodules on the left lateral axillary region and swelling of the surrounding skin. Which of the following is the most likely diagnosis?
A. Thrombophlebitis
B. Cellulitis
C. Melanoma
D. Angiosarcoma (Correct Answer)
E. Kaposi sarcoma
Explanation: ***Angiosarcoma***
- The presence of **firm, purple-blue nodules** in a patient with **chronic lymphedema** following **mastectomy and radiation** for breast cancer is highly suggestive of **angiosarcoma (Stewart-Treves syndrome)**.
- This rare but aggressive vascular malignancy often presents as skin lesions in the setting of long-standing lymphedema, particularly in the upper extremity after breast cancer treatment.
*Thrombophlebitis*
- Typically presents with **erythema**, **tenderness**, and **pain** along the course of a superficial vein, which is not described here.
- The lesions would likely blanch with pressure and feel more like a cord or streak, rather than firm, discrete nodules.
*Cellulitis*
- Would present with **warmth**, **tenderness**, **erythema with ill-defined borders**, and often **fever**, none of which are mentioned.
- While lymphedema is a risk factor for cellulitis, the description of discrete, firm, purple-blue nodules growing over time is not characteristic.
*Melanoma*
- Although melanoma can present as a dark lesion, it often has **irregular borders**, **asymmetry**, and **color variegation**, and is typically associated with UV exposure.
- While it can be nodular, the specific context of chronic lymphedema and the description of purple-blue lesions are more indicative of a vascular tumor.
*Kaposi sarcoma*
- Characterized by **purple-brown skin lesions** and is primarily associated with **HIV infection** or **immunosuppression**, neither of which is indicated in this patient.
- While it is a vascular tumor, the unique presentation in the context of chronic lymphedema post-breast cancer therapy makes angiosarcoma a more fitting diagnosis.
Question 2: A 45-year-old man comes to the emergency department because of chills and numerous skin lesions for 1 week. He has also had watery diarrhea, nausea, and abdominal pain for the past 2 weeks. The skin lesions are nonpruritic and painless. He was diagnosed with HIV infection approximately 20 years ago. He has not taken any medications for over 5 years. He sleeps in homeless shelters and parks. Vital signs are within normal limits. Examination shows several bright red, friable nodules on his face, trunk, extremities. The liver is palpated 3 cm below the right costal margin. His CD4+ T-lymphocyte count is 180/mm3 (N ≥ 500). A rapid plasma reagin test is negative. Abdominal ultrasonography shows hepatomegaly and a single intrahepatic 1.0 x 1.2-cm hypodense lesion. Biopsy of a skin lesion shows vascular proliferation and abundant neutrophils. Which of the following is the most likely causal organism?
A. Bartonella henselae (Correct Answer)
B. Treponema pallidum
C. HHV-8 virus
D. Mycobacterium avium
E. Candida albicans
Explanation: ***Bartonella henselae***
- The patient's presentation with **bright red, friable nodules** (consistent with **bacillary angiomatosis**) in an HIV-positive individual with a low **CD4+ count** strongly suggests infection with *Bartonella henselae*.
- **Hepatomegaly** and **intrahepatic lesions** further support disseminated bartonellosis, and skin biopsy showing vascular proliferation with **abundant neutrophils** is characteristic.
*Treponema pallidum*
- While *Treponema pallidum* (syphilis) can cause various skin lesions, the **rapid plasma reagin (RPR) test** was negative, making syphilis highly unlikely.
- Syphilitic lesions typically do not present as brightly friable nodules with prominent vascular proliferation and neutrophils characteristic of bacillary angiomatosis.
*HHV-8 virus*
- **HHV-8** is the causative agent of **Kaposi sarcoma**, which also presents with vascular lesions. However, Kaposi sarcoma lesions are typically **violaceous plaques or nodules** and histologically show spindle cells and extravasated red blood cells, not the prominent neutrophils seen here.
- The patient's clinical presentation, particularly the friable nature and specific histology, steers away from Kaposi sarcoma.
*Mycobacterium avium*
- *Mycobacterium avium* complex (MAC) can cause disseminated disease in HIV patients with low CD4 counts, often presenting with fever, weight loss, and gastrointestinal symptoms.
- However, MAC infection rarely causes specific nonpruritic, bright red, friable skin nodules like those described, and hepatic lesions would typically be granulomatous, not necessarily angiomatous.
*Candida albicans*
- While *Candida albicans* can cause various infections in immunocompromised individuals, including esophagitis and mucocutaneous candidiasis, it does not typically present with these specific bright red, friable vascular skin nodules.
- Disseminated candidiasis would more likely involve fungemia and widespread organ involvement, often with more subtle or different skin manifestations (e.g., maculopapular rash).
Question 3: A 93-year-old woman is brought to the physician because of a purple area on her right arm that has been growing for one month. She has not had any pain or itching of the area. She has hyperlipidemia, a history of basal cell carcinoma treated with Mohs surgery 2 years ago, and a history of invasive ductal carcinoma of the right breast treated with radical mastectomy 57 years ago. She has had chronic lymphedema of the right upper extremity since the mastectomy. Her only medication is simvastatin. She lives in an assisted living facility. She is content with her living arrangement but feels guilty that she is dependent on others. Vital signs are within normal limits. Physical examination shows extensive edema of the right arm. Skin exam of the proximal upper right extremity shows three coalescing, 0.5–1.0 cm heterogeneous, purple-colored plaques with associated ulceration. Which of the following is the most likely diagnosis?
A. Lymphangiosarcoma (Correct Answer)
B. Cellulitis
C. Thrombophlebitis
D. Kaposi sarcoma
E. Lichen planus
Explanation: ***Lymphangiosarcoma***
- This patient presents with a **purple area** with **ulceration** on an arm affected by chronic **lymphedema** following a mastectomy. This constellation of findings is highly suggestive of **Stewart-Treves syndrome**, which is a rare but aggressive form of angiosarcoma (lymphangiosarcoma) arising in the setting of chronic lymphedema.
- The history of **radical mastectomy 57 years ago** with subsequent **chronic right upper extremity lymphedema** strongly predisposes to this condition, especially given the lack of pain or itching and the progressive nature of the lesion.
*Cellulitis*
- Characterized by **erythema**, **warmth**, **pain**, and **tenderness**, often with fever and systemic symptoms, none of which are described.
- While lymphedema is a risk factor for cellulitis, the description of a **purple, ulcerated plaque** with a duration of one month is inconsistent with acute bacterial infection.
*Thrombophlebitis*
- Typically presents with **pain**, **tenderness**, **erythema**, and a palpable cord-like vein along the course of a superficial vein, which is not described.
- The lesion described is a spreading, **ulcerated plaque**, not a discrete inflamed vein.
*Kaposi sarcoma*
- While it can present with **purple lesions**, Kaposi sarcoma is typically associated with **immunosuppression** (e.g., HIV infection) or certain endemic regions, neither of which is mentioned.
- The strong association with **chronic lymphedema** in this case makes lymphangiosarcoma a more specific diagnosis.
*Lichen planus*
- A chronic inflammatory condition characterized by **pruritic, purple, polygonal, planar papules and plaques (the 6 Ps)**, often with fine white lines (Wickham's striae).
- It does not typically present as a growing, **ulcerated purple plaque** in the setting of chronic lymphedema, and itching is a prominent symptom.
Question 4: A 49-year-old man with HIV comes to the physician because of a 1-month history of intermittent diarrhea and abdominal pain. Abdominal examination shows mild, diffuse tenderness throughout the lower quadrants. His CD4+ T-lymphocyte count is 180/mm3 (normal ≥ 500/mm3). Colonoscopy shows multiple hemorrhagic nodules in the rectum and descending colon. Polymerase chain reaction of the lesions is positive for HHV-8. Histologic examination of the lesions is most likely to show which of the following findings?
A. Spindle-shaped cells with leukocytic infiltration (Correct Answer)
B. Mucin-filled cell with peripheral nucleus
C. Cords of atypical cells with extracellular mucin
D. Enlarged cells with intranuclear inclusion bodies
E. Polygonal cells with racket-shaped organelles
Explanation: ***Spindle-shaped cells with leukocytic infiltration***
- The presentation of an HIV-positive patient with a low CD4+ count, hemorrhagic nodules in the colon, and positive HHV-8 PCR is highly suggestive of **Kaposi sarcoma**.
- Histologically, Kaposi sarcoma is characterized by proliferation of **spindle-shaped endothelial cells**, often forming slit-like vascular channels, and an inflammatory infiltrate rich in lymphocytes and plasma cells.
*Mucin-filled cell with peripheral nucleus*
- This describes a **signet ring cell**, which is characteristic of diffuse-type gastric carcinoma and other mucinous adenocarcinomas, not Kaposi sarcoma.
- Signet ring cells contain a large **mucin vacuole** that pushes the nucleus to the periphery.
*Cords of atypical cells with extracellular mucin*
- This finding is typical of certain types of **adenocarcinoma**, particularly those with a mucinous component, such as mucinous colorectal carcinoma.
- It does not align with the known histopathology of Kaposi sarcoma, which is a vascular tumor.
*Enlarged cells with intranuclear inclusion bodies*
- This description is characteristic of a **cytomegalovirus (CMV) infection**, particularly in immunocompromised patients. Inclusions are often described as "owl's eye" inclusions.
- While CMV can cause gastrointestinal symptoms in HIV patients, the positive HHV-8 PCR points specifically to Kaposi sarcoma.
*Polygonal cells with racket-shaped organelles*
- This describes the characteristic cells of **Langerhans cell histiocytosis**, where the cells are Langerhans cells containing Birbeck granules (racket-shaped organelles).
- This condition is a proliferative disorder of dendritic cells and does not fit the clinical or pathological findings of this case.
Question 5: Two weeks after undergoing low anterior resection for rectal cancer, a 52-year-old man comes to the physician because of swelling in both feet. He has not had any fever, chills, or shortness of breath. His temperature is 36°C (96.8°F) and pulse is 88/min. Physical examination shows a normal thyroid and no jugular venous distention. Examination of the lower extremities shows bilateral non-pitting edema that extends from the feet to the lower thigh, with deep flexion creases. His skin is warm and dry, and there is no erythema or rash. Microscopic examination of the interstitial space in this patient's lower extremities would be most likely to show the presence of which of the following?
A. Neutrophilic, protein-rich fluid
B. Lymphocytic, hemosiderin-rich fluid
C. Lipid-rich, protein-rich fluid (Correct Answer)
D. Protein-rich, glycosaminoglycan-rich fluid
E. Acellular, protein-poor fluid
Explanation: ***Lipid-rich, protein-rich fluid***
- The presentation of bilateral non-pitting edema extending to the thigh, with deep flexion creases, in a patient post-**low anterior resection** for rectal cancer, strongly suggests **lymphedema**.
- Lymphedema results from impaired lymphatic drainage, leading to the accumulation of **protein-rich fluid**, **macromolecules**, and **adipose tissue** in the interstitial space, which eventually becomes lipid-rich due to chronic inflammation and fibroblasts stimulating adipogenesis.
*Neutrophilic, protein-rich fluid*
- This description is characteristic of **inflammatory edema** or **purulent exudate**, typically seen in infections.
- The patient's lack of fever, chills, erythema, or warmth makes an infectious or acute inflammatory process unlikely.
*Lymphocytic, hemosiderin-rich fluid*
- **Hemosiderin deposits** are indicative of chronic **venous insufficiency** or recurrent hemorrhages, leading to red blood cell extravasation and breakdown.
- While lymphocytes can be present in chronic inflammation, the primary issue here is lymphatic obstruction, not venous stasis or bleeding.
*Protein-rich, glycosaminoglycan-rich fluid*
- While lymphedema is indeed **protein-rich**, the primary accumulation in mature lymphedema involves **adipose tissue** and fibrosis.
- **Glycosaminoglycans** accumulate significantly in conditions like **myxedema** (hypothyroidism), which was ruled out by the normal thyroid examination.
*Acellular, protein-poor fluid*
- This describes a **transudate**, typically seen in conditions like **heart failure**, **liver cirrhosis**, or **nephrotic syndrome** where there's an imbalance of hydrostatic and oncotic pressures.
- The edema in this case is **non-pitting**, suggesting a higher protein content and tissue changes characteristic of lymphatic dysfunction, not systemic fluid overload leading to protein-poor fluid.
Question 6: A 28-year-old man presents for severe abdominal pain and is diagnosed with appendicitis. He is taken for emergent appendectomy. During the procedure, the patient has massive and persistent bleeding requiring a blood transfusion. The preoperative laboratory studies showed a normal bleeding time, normal prothrombin time (PT), an INR of 1.0, and a normal platelet count. Postoperatively, when the patient is told about the complications during the surgery, he recalls that he forgot to mention that he has a family history of an unknown bleeding disorder. The postoperative laboratory tests reveal a prolonged partial thromboplastin time (PTT). Which of the following is the most likely diagnosis in this patient?
A. von Willebrand disease
B. Bernard-Soulier syndrome
C. Thrombotic thrombocytopenic purpura
D. Hemophilia A (Correct Answer)
E. Glanzmann thrombasthenia
Explanation: ***Hemophilia A***
- The patient's presentation with **severe, persistent bleeding** during surgery despite normal preoperative coagulation studies (PT, INR, platelet count) and a subsequent **prolonged PTT** strongly indicates a **factor deficiency in the intrinsic pathway**.
- **Hemophilia A**, an X-linked recessive disorder, is caused by a deficiency of **Factor VIII**, leading to a prolonged PTT and deep tissue bleeding, which fits the clinical picture and family history.
*von Willebrand disease*
- This condition typically presents with **mucocutaneous bleeding** (e.g., nosebleeds, menorrhagia) and can have a prolonged bleeding time, but the primary defect is in **platelet adhesion**, not usually massive operative bleeding with normal platelet count.
- While **von Willebrand factor (vWF)** carries Factor VIII, a primary deficiency of vWF would affect factor VIII levels but the presentation and normal bleeding time here make it less likely than direct factor VIII deficiency.
*Bernard-Soulier syndrome*
- This is a **platelet disorder** characterized by defective **glycoprotein Ib/IX/V complex**, leading to impaired platelet adhesion and often **thrombocytopenia** with unusually large platelets.
- The patient had a **normal platelet count** and a subsequent prolonged PTT, which points away from a primary platelet adhesion defect.
*Thrombotic thrombocytopenic purpura*
- This is a microangiopathic hemolytic anemia characterized by a **pentad of symptoms**: fever, neurologic symptoms, renal dysfunction, thrombocytopenia, and microangiopathic hemolytic anemia.
- It involves widespread **thrombosis** and **low platelet count**, which does not align with the patient's presentation of massive bleeding with normal platelet counts.
*Glanzmann thrombasthenia*
- This is a rare **platelet aggregation disorder** caused by a defect in **glycoprotein IIb/IIIa**, leading to impaired fibrinogen binding and platelet aggregation.
- While it causes severe bleeding, it would be associated with a **prolonged bleeding time** and normal PTT, which contradicts the patient's normal bleeding time and prolonged PTT.
Question 7: A previously healthy 4-year-old boy is brought to the emergency department because of a 1-day history of pain and swelling of his left knee joint. He has not had any trauma to the knee. His family history is unremarkable except for a bleeding disorder in his maternal uncle. His temperature is 36.9°C (98.4°F). The left knee is erythematous, swollen, and tender; range of motion is limited. No other joints are affected. An x-ray of the knee shows an effusion but no structural abnormalities of the joint. Arthrocentesis is conducted. The synovial fluid is bloody. Further evaluation of this patient is most likely to show which of the following findings?
A. Prolonged prothrombin time
B. Synovial fluid leukocytosis
C. Elevated antinuclear antibody levels
D. Prolonged partial thromboplastin time (Correct Answer)
E. Decreased platelet count
Explanation: ***Prolonged partial thromboplastin time***
- The clinical presentation of a young boy with **spontaneous hemarthrosis** (bloody synovial fluid in the knee) and a family history of a **bleeding disorder in a maternal uncle** is highly suggestive of **hemophilia A or B**.
- Both hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency) present with a **prolonged PTT** due to impaired intrinsic coagulation pathway, while PT and platelet count typically remain normal.
*Prolonged prothrombin time*
- A prolonged **prothrombin time (PT)** primarily indicates a deficiency in the **extrinsic coagulation pathway**, involving factor VII, or severe deficiencies in common pathway factors (II, V, X, fibrinogen).
- This is not characteristic of hemophilia A or B, which are deficiencies in the **intrinsic pathway**.
*Synovial fluid leukocytosis*
- **Synovial fluid leukocytosis** (increased white blood cells) is typically seen in **inflammatory or infectious arthritis**, such as septic arthritis or juvenile idiopathic arthritis.
- While the knee is swollen and tender, the **bloody synovial fluid without trauma** points away from infection and towards a bleeding disorder.
*Elevated antinuclear antibody levels*
- **Elevated antinuclear antibody (ANA) levels** are a hallmark of **systemic autoimmune diseases** like systemic lupus erythematosus (SLE) or certain forms of juvenile idiopathic arthritis.
- These conditions do not typically present with spontaneous gross hemarthrosis as the primary symptom, nor would they explain the family history of a bleeding disorder.
*Decreased platelet count*
- A **decreased platelet count (thrombocytopenia)** would lead to a bleeding disorder characterized by mucocutaneous bleeding (e.g., petechiae, purpura, epistaxis) rather than deep joint bleeds.
- Platelet dysfunction or thrombocytopenia would cause a **prolonged bleeding time**, not typically a prolonged PTT, and the overall clinical picture does not align with a primary platelet disorder.
Question 8: A 50-year-old woman with rheumatoid arthritis on methotrexate develops rapidly progressive painful ulcers on her legs with violaceous undermined borders. Biopsy shows neutrophilic dermal infiltrate with areas of necrosis, but no vasculitis or infection. Wound cultures are negative. Despite debridement, the ulcers worsen. C-ANCA and P-ANCA are negative. Evaluate the diagnosis and determine the management that addresses both the cutaneous condition and systemic disease.
A. Discontinue all immunosuppression to allow wound healing
B. Increase methotrexate dose and add wound care
C. Discontinue methotrexate, start cyclosporine and prednisone
D. Start broad-spectrum antibiotics and surgical debridement
E. Continue methotrexate, add TNF-alpha inhibitor and systemic corticosteroids (Correct Answer)
Explanation: ***Continue methotrexate, add TNF-alpha inhibitor and systemic corticosteroids***
- This patient presents with **Pyoderma Gangrenosum (PG)**, a neutrophilic dermatosis characterized by **violaceous undermined borders** and **pathergy**, where surgical debridement cause lesion expansion.
- **TNF-alpha inhibitors** (e.g., adalimumab, infliximab) are first-line for recalcitrant PG and simultaneously provide excellent control for the underlying **Rheumatoid Arthritis**.
*Discontinue all immunosuppression to allow wound healing*
- Since PG is an **autoimmune inflammatory condition**, withdrawing immunosuppression would result in rapid progression of the ulcers rather than healing.
- Wound healing in PG requires **suppressing the inflammatory response** rather than the typical wound care approach for infected ulcers.
*Increase methotrexate dose and add wound care*
- While **methotrexate** treats RA, it is often insufficient as a monotherapy for the acute, rapidly progressive phase of **Pyoderma Gangrenosum**.
- Standard wound care alone is ineffective because the primary driver is **neutrophilic infilatration**, which requires targeted biologic or corticosteroid therapy.
*Discontinue methotrexate, start cyclosporine and prednisone*
- While **cyclosporine** and **prednisone** are used for PG, discontinuing methotrexate may lead to a flare of the patient’s **Rheumatoid Arthritis**.
- Maintaining a coordinated regimen that addresses both the skin and the joints, such as adding a **TNF-alpha inhibitor**, is preferred over switching all medications.
*Start broad-spectrum antibiotics and surgical debridement*
- **Surgical debridement** is contraindicated in PG due to **pathergy**, a phenomenon where trauma to the skin induces new or worsening lesions.
- **Antibiotics** are unnecessary as the biopsy and cultures confirmed a **sterile neutrophilic infiltrate** rather than an infectious process.
Question 9: A 25-year-old woman presents with painful oral ulcers and a pustular rash at venipuncture sites. She has genital ulcers and a history of recurrent uveitis. Skin biopsy from a pustule shows neutrophilic infiltrate in the dermis without vasculitis or infection. HLA-B51 testing is positive. She is planning pregnancy. Evaluate the management strategy considering disease control and pregnancy planning.
A. Start methotrexate for disease control
B. Start colchicine monotherapy and proceed with pregnancy
C. Start high-dose corticosteroids and azathioprine, delay pregnancy
D. Start infliximab, use contraception, then transition to low-risk therapy before conception (Correct Answer)
E. Avoid all immunosuppression and manage symptoms only
Explanation: ***Start infliximab, use contraception, then transition to low-risk therapy before conception***
- This patient has **Beh'et's disease** with **recurrent uveitis**, which is **sight-threatening** and requires aggressive biological therapy like **infliximab** or **TNF-inhibitors** for rapid remission.
- Achievng **remission** before pregnancy is vital; while TNF-inhibitors are often continued, transitioning to pregnancy-compatible agents like **azathioprine** or **colchicine** ensures long-term safety.
*Start methotrexate for disease control*
- **Methotrexate** is strictly **teratogenic** and must be avoided in patients planning pregnancy or discontinued months before conception.
- While it can treat some aspects of systemic inflammation, it is not the first-line gold standard for **acute ocular Beh'et's** compared to biologics.
*Start colchicine monotherapy and proceed with pregnancy*
- **Colchicine** is excellent for **mucocutaneous** symptoms (oral and genital ulcers) but is insufficient as monotherapy to prevent blindness from **recurrent uveitis**.
- Relying on monotherapy in a patient with active ocular disease risks **permanent vision loss** during the pregnancy period.
*Start high-dose corticosteroids and azathioprine, delay pregnancy*
- While **azathioprine** is used for maintenance, **high-dose corticosteroids** carry significant side effects and are usually a bridge, not a comprehensive plan for ocular stabilization.
- This strategy lacks the rapid, potent **TNF-alpha inhibition** needed to quickly arrest the neutrophilic inflammation seen in severe Beh'et's flare-ups.
*Avoid all immunosuppression and manage symptoms only*
- **Beh'et's disease** is a multi-system inflammatory disorder; leaving **uveitis** and systemic vasculitis untreated leads to irreversible organ damage and **blindness**.
- Symptomatic management alone ignores the **neutrophilic infiltrate** and underlying autoimmune process, which could also lead to pregnancy complications due to active maternal disease.
Question 10: A 70-year-old man on chronic warfarin therapy presents with sudden onset of painful purpura on his thighs and buttocks three days after starting warfarin for atrial fibrillation. He has a history of multiple DVTs. Skin biopsy shows thrombosis of dermal blood vessels with minimal inflammation. Laboratory studies show an INR of 3.5. Evaluate the pathophysiology and determine the most appropriate immediate management.
A. Reduce warfarin dose and add compression therapy
B. Discontinue all anticoagulation and monitor
C. Continue warfarin and add aspirin for antiplatelet effect
D. Discontinue warfarin, give vitamin K and fresh frozen plasma, start heparin bridge
E. Discontinue warfarin, give protein C concentrate and alternative anticoagulation (Correct Answer)
Explanation: ***Discontinue warfarin, give protein C concentrate and alternative anticoagulation***
- This patient presents with **warfarin-induced skin necrosis**, likely caused by an underlying **Protein C deficiency** accentuated by the rapid drop in Protein C levels during initial warfarin therapy.
- Management requires immediate **discontinuation of warfarin**, replacement of the missing anticoagulant via **Protein C concentrate**, and switching to an **alternative anticoagulant** (like heparin).
*Reduce warfarin dose and add compression therapy*
- Reducing the dose is insufficient because the patient is in a **hypercoagulable state** that requires the complete removal of the offending agent.
- **Compression therapy** is used for venous insufficiency and does not address the underlying **microvascular thrombosis** seen in this condition.
*Discontinue all anticoagulation and monitor*
- Discontinuing all anticoagulation is dangerous as the patient is currently **hypercoagulable** and has a high risk for further **thromboembolic events**.
- Monitoring alone does not address the **dermal vessel thrombosis** already causing tissue necrosis.
*Continue warfarin and add aspirin for antiplatelet effect*
- Continuing warfarin would worsen the condition by further suppressing **Protein C** and **Protein S** levels while procoagulant factors like **Prothrombin** are still present.
- **Aspirin** targets platelets but does not reverse the **clotting factor imbalance** responsible for skin necrosis.
*Discontinue warfarin, give vitamin K and fresh frozen plasma, start heparin bridge*
- While **vitamin K** and **FFP** help reverse warfarin effects, FFP may not contain enough **Protein C** to rapidly stabilize this specific critical deficiency.
- Management must prioritize **specific protein replacement** or therapeutic dose heparin rather than a standard bridge protocol which may be too slow to stop the necrosis.
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