40 types of lymphatic system disease blood vessels arteries and veins lymph nodes most important disease treatment investigation finding differential diagnosis contraindication ICO management OPD prescription
Reading File
Finding Sources
Finding Sources
Finding Sources
Finding Sources
Finding Sources
Based on the available textbook references (Harrison's, Robbins, Andrews' Dermatology, Braunwald's, Fitzpatrick's), here is a comprehensive clinical reference covering 40 diseases of the lymphatic system, blood vessels (arteries & veins), and lymph nodes.
40 Diseases of the Lymphatic System, Blood Vessels & Lymph Nodes
COMPREHENSIVE CLINICAL REFERENCE
PART I — LYMPHATIC SYSTEM DISEASES
1. PRIMARY LYMPHEDEMA
| Feature | Detail |
|---|---|
| Definition | Developmental aplasia/hypoplasia of lymphatics |
| Types | Milroy disease (congenital), Lymphedema praecox (puberty), Lymphedema tarda (>35 yrs) |
| Findings | Non-pitting edema, dorsum of foot/leg, Kaposi-Stemmer sign positive, recurrent cellulitis |
| Investigation | Lymphoscintigraphy (gold standard), lymphangiography, MRI lymphangiogram |
| DD | Secondary lymphedema, lipedema, chronic venous insufficiency, myxedema |
| Treatment | Complete decongestive therapy (CDT): manual lymphatic drainage + compression garments; pneumatic compression |
| OPD Rx | Compression stockings (30–40 mmHg), benzopyrones (coumarin), avoid trauma/infection |
| Contraindication | Diuretics (ineffective long-term, worsen protein concentration) |
2. SECONDARY LYMPHEDEMA
| Feature | Detail |
|---|---|
| Causes | Post-mastectomy, filariasis, radiation, malignancy, recurrent lymphangitis |
| Findings | Brawny non-pitting edema, skin thickening, "orange peel" (peau d'orange) |
| Investigation | Lymphoscintigraphy, duplex USS, CBC, microfilaria smear (filariasis) |
| DD | DVT, CCF, nephrotic syndrome, hypoalbuminemia |
| Treatment | CDT, treat underlying cause; antibiotics if cellulitis; surgery (liposuction/bypass) in refractory cases |
| OPD Rx | Compression bandaging, skin hygiene, antifungal prophylaxis (tinea pedis) |
| Contraindication | Tight compressive dressings without monitoring in active infection |
3. LYMPHANGITIS (Acute Bacterial)
| Feature | Detail |
|---|---|
| Pathogen | Group A Streptococcus (most common), S. aureus |
| Findings | Red streaks from wound toward regional lymph nodes, tender lymph nodes, fever, leukocytosis |
| Investigation | Blood culture, wound swab, CBC, CRP |
| DD | Thrombophlebitis, cellulitis, cat-scratch disease, sporotrichosis |
| Treatment | Penicillin/amoxicillin-clavulanate IV; MRSA: clindamycin or vancomycin |
| OPD Rx | Amoxicillin-clavulanate 875/125 mg BD × 7–10 days, limb elevation |
| Contraindication | Penicillin if allergic (use clindamycin or azithromycin) |
4. LYMPHADENITIS (Acute)
| Feature | Detail |
|---|---|
| Causes | Bacterial (Staph, Strep), viral (EBV, CMV), mycobacterial (TB) |
| Findings | Tender, enlarged, warm lymph node; fluctuance if abscess; constitutional symptoms |
| Investigation | FNA/FNAC, culture, CBC, Monospot test, Mantoux, CXR |
| DD | Lymphoma, metastatic carcinoma, cat-scratch disease, sarcoidosis |
| Treatment | Antibiotics; I&D if abscess; anti-TB therapy if tuberculous |
| OPD Rx | Co-amoxiclav 625 mg TDS × 5–7 days; analgesics |
| Contraindication | Empirical steroids without ruling out TB/lymphoma |
5. TUBERCULOUS LYMPHADENITIS (Scrofula)
| Feature | Detail |
|---|---|
| Site | Cervical (most common), mediastinal |
| Findings | Matted, rubbery nodes; collar-stud abscess; sinus tract; low-grade fever, night sweats, weight loss |
| Investigation | FNAC (caseating granuloma), AFB smear/culture, GeneXpert, Mantoux, CXR, ESR |
| DD | Reactive lymphadenopathy, lymphoma, actinomycosis, sarcoidosis |
| Treatment | 2HRZE / 4HR (standard anti-TB regimen) |
| OPD Rx | Isoniazid 300 mg + Rifampicin 600 mg + Ethambutol 800 mg + Pyrazinamide 1500 mg daily × 2 months, then HR × 4 months |
| Contraindication | Rifampicin in liver disease; Ethambutol in optic neuritis |
6. HODGKIN LYMPHOMA
| Feature | Detail |
|---|---|
| Hallmark | Reed-Sternberg (RS) cell: "owl-eye" nuclei |
| Findings | Painless cervical lymphadenopathy, B symptoms (fever >38°C, night sweats, >10% wt loss), alcohol-induced pain |
| Staging | Ann Arbor I–IV (A/B) |
| Investigation | Excisional biopsy (RS cells), CT chest/abdomen/pelvis, PET scan, bone marrow biopsy, CBC, LDH, ESR |
| DD | NHL, infectious mononucleosis, sarcoidosis, metastatic carcinoma |
| Treatment | Early stage: ABVD × 2–4 cycles + IFRT; Advanced: ABVD × 6 cycles; Refractory: BEACOPP, autologous SCT |
| OPD Rx | ABVD: Adriamycin, Bleomycin, Vinblastine, Dacarbazine |
| Contraindication | Bleomycin in pre-existing lung disease (pulmonary toxicity) |
7. NON-HODGKIN LYMPHOMA (NHL)
| Feature | Detail |
|---|---|
| Types | Diffuse large B-cell (DLBCL, most common), Follicular, Burkitt, Mantle cell, T-cell |
| Findings | Painless lymphadenopathy (extranodal involvement common), B symptoms, hepatosplenomegaly |
| Investigation | Biopsy + immunohistochemistry (CD20, CD10, bcl-2), flow cytometry, CT/PET, LDH, β2-microglobulin |
| DD | Hodgkin lymphoma, leukemia, reactive lymphadenopathy |
| Treatment | DLBCL: R-CHOP (Rituximab + Cyclophosphamide, Doxorubicin, Vincristine, Prednisolone); Follicular: R-CVP or watchful waiting |
| OPD Rx | Rituximab-based protocols; CNS prophylaxis in high-risk |
| Contraindication | Rituximab in Hepatitis B (reactivation risk — screen first) |
8. FILARIASIS (Lymphatic Filariasis)
| Feature | Detail |
|---|---|
| Pathogen | Wuchereria bancrofti (most common), Brugia malayi |
| Findings | Lymphedema, elephantiasis, hydrocele, chyluria; retrograde lymphangitis (pathognomonic) |
| Investigation | Nocturnal blood smear for microfilariae, ICT card test, ELISA antigen, USS (filarial dance sign) |
| DD | Primary lymphedema, tuberculosis, podoconiosis |
| Treatment | Diethylcarbamazine (DEC) 6 mg/kg/day × 12 days or single-dose DEC + albendazole; Ivermectin in co-endemic areas |
| OPD Rx | DEC 100 mg TDS × 12 days; limb hygiene; compression |
| Contraindication | DEC in onchocerciasis co-infection (Mazzotti reaction); in pregnancy |
9. CASTLEMAN DISEASE (Angiofollicular Lymph Node Hyperplasia)
| Feature | Detail |
|---|---|
| Types | Unicentric (UCD), Multicentric (MCD), HHV-8-associated |
| Findings | Large mediastinal/abdominal mass, constitutional symptoms in MCD; hyaline-vascular pattern (UCD) vs plasma cell (MCD) |
| Investigation | Excisional biopsy, CT PET, IL-6 levels, HHV-8 PCR, VEGF |
| DD | Lymphoma, sarcoidosis, Kikuchi disease, IgG4 disease |
| Treatment | UCD: surgical excision (curative); MCD: Siltuximab (anti-IL-6) or rituximab; HHV-8: antivirals + rituximab |
| OPD Rx | Siltuximab 11 mg/kg IV q3w (MCD) |
| Contraindication | Live vaccines during immunosuppressive therapy |
10. KIKUCHI-FUJIMOTO DISEASE
| Feature | Detail |
|---|---|
| Definition | Self-limiting necrotizing histiocytic lymphadenitis |
| Findings | Young women; posterior cervical lymphadenopathy, fever, leukopenia |
| Investigation | Excisional biopsy (karyorrhectic debris, histiocytes, no neutrophils), CBC, ANA |
| DD | SLE lymphadenopathy, lymphoma, TB lymphadenitis |
| Treatment | Self-limiting; NSAIDs for fever/pain; hydroxychloroquine in severe/recurrent cases |
| OPD Rx | Ibuprofen 400 mg TDS × 1–3 weeks |
| Contraindication | Steroids without excluding lymphoma/SLE |
PART II — ARTERIAL DISEASES
11. ATHEROSCLEROSIS
| Feature | Detail |
|---|---|
| Pathology | Lipid-laden foam cells → fibrous plaque → calcification; vulnerable plaque → rupture |
| Risk factors | Hypertension, dyslipidemia, DM, smoking, family history |
| Findings | Angina, claudication, TIA/stroke, xanthelasma, corneal arcus, reduced pulses |
| Investigation | Lipid profile, ABI (ankle-brachial index), Doppler USS, CT angiography, coronary angiogram |
| DD | Vasculitis, fibromuscular dysplasia, Buerger disease |
| Treatment | Risk factor modification, statins, antiplatelet; revascularization (PCI/CABG/bypass) |
| OPD Rx | Aspirin 75 mg OD + Atorvastatin 40–80 mg ON + BP/glucose control |
| Contraindication | Fibrates with statins (rhabdomyolysis risk) |
12. PERIPHERAL ARTERIAL DISEASE (PAD)
| Feature | Detail |
|---|---|
| Fontaine Stages | I: Asymptomatic → II: Claudication → III: Rest pain → IV: Necrosis/gangrene |
| Findings | Claudication, weak/absent pulses, pallor on elevation, dependent rubor, ABI < 0.9 |
| Investigation | ABI (gold standard screening), Duplex USS, CT/MR angiography |
| DD | Spinal stenosis (neurogenic claudication), venous claudication, Buerger disease |
| Treatment | Exercise therapy, cilostazol, antiplatelet; endovascular (PTA/stenting) or bypass surgery |
| OPD Rx | Cilostazol 100 mg BD, Aspirin 75 mg OD, Clopidogrel 75 mg OD, Atorvastatin |
| Contraindication | Cilostazol in heart failure; beta-blockers (worsen claudication) |
13. AORTIC ANEURYSM (AAA / TAA)
| Feature | Detail |
|---|---|
| Definition | Permanent dilation >50% of normal diameter; AAA >3 cm |
| Findings | Often asymptomatic; pulsatile abdominal mass; rupture: hypotension + pulsatile mass + back/abdominal pain (Triad) |
| Investigation | USS (screening), CT aortogram (operative planning), MRA |
| DD | Retroperitoneal mass, horseshoe kidney, dissection |
| Treatment | <5.5 cm: surveillance; ≥5.5 cm or rapid growth >1 cm/yr or symptomatic: EVAR or open repair |
| OPD Rx | Beta-blocker (propranolol), statin, smoking cessation, BP control |
| Contraindication | Vigorous exertion with known large AAA |
14. AORTIC DISSECTION
| Feature | Detail |
|---|---|
| Classification | Stanford: Type A (ascending) — surgical emergency; Type B (descending) — medical management |
| Findings | Sudden tearing/ripping chest pain radiating to back, unequal BP in arms, pulse deficit, aortic regurgitation (Type A) |
| Investigation | CT aortogram (gold standard), TEE, CXR (widened mediastinum), ECG (rule out MI) |
| DD | STEMI, PE, pneumothorax, aortic aneurysm |
| Treatment | Type A: Emergency surgery; Type B: IV labetalol + sodium nitroprusside; TEVAR if complicated |
| OPD Rx | Beta-blocker (Metoprolol succinate 50–100 mg BD) + CCB to target SBP 100–120 mmHg |
| Contraindication | Pure vasodilators (nitroprusside alone) without beta-blockade — reflex tachycardia worsens dissection |
15. TAKAYASU ARTERITIS
| Feature | Detail |
|---|---|
| Population | Young Asian women ("pulseless disease") |
| Findings | Absent/unequal pulses, claudication of arms, Takayasu retinopathy, hypertension (renal artery), bruits |
| Investigation | ESR/CRP, CTA/MRA (luminal irregularity, stenosis of aorta & branches), PET-CT (active inflammation) |
| DD | Giant cell arteritis, fibromuscular dysplasia, atherosclerosis |
| Treatment | Prednisolone 1 mg/kg/day; steroid-sparing: methotrexate, azathioprine, tocilizumab; revascularization in stable disease |
| OPD Rx | Prednisolone + Methotrexate 15–25 mg weekly + folic acid 5 mg weekly |
| Contraindication | NSAIDs (mask inflammation); revascularization during active inflammation |
16. GIANT CELL ARTERITIS (GCA / Temporal Arteritis)
| Feature | Detail |
|---|---|
| Population | >50 years, female predominance |
| Findings | Temporal headache, scalp tenderness, jaw claudication, visual loss (anterior ischemic optic neuropathy), ESR >50 |
| Investigation | Temporal artery biopsy (gold standard: granulomatous inflammation with giant cells), ESR, CRP, USS temporal artery |
| DD | Takayasu arteritis, trigeminal neuralgia, migraine, TIA |
| Treatment | High-dose prednisolone 40–60 mg/day IMMEDIATELY (before biopsy if visual threat); tocilizumab as steroid-sparing |
| OPD Rx | Prednisolone 40–60 mg OD, slow taper over 12–24 months; PPI cover; calcium + vitamin D |
| Contraindication | Delay in steroids when visual loss threatens (ophthalmologic emergency) |
17. BUERGER DISEASE (Thromboangiitis Obliterans)
| Feature | Detail |
|---|---|
| Population | Young male smokers |
| Findings | Distal extremity ischemia, migratory thrombophlebitis, Raynaud phenomenon, rest pain, digital gangrene |
| Investigation | Arteriography (corkscrew collaterals), Allen test, ABI, no atherosclerosis markers, tobacco exposure |
| DD | Atherosclerosis, embolic disease, connective tissue disease, diabetes |
| Treatment | Complete smoking cessation (only effective treatment); prostanoids (iloprost IV); sympathectomy; amputation |
| OPD Rx | Iloprost infusions; wound care; absolute smoking cessation |
| Contraindication | Nicotine replacement therapy (still delivers nicotine — prolongs disease) |
18. RAYNAUD PHENOMENON
| Feature | Detail |
|---|---|
| Types | Primary (Raynaud disease) vs Secondary (connective tissue disease, drugs) |
| Findings | Triphasic color change: White (pallor) → Blue (cyanosis) → Red (rubor); cold/emotional triggers |
| Investigation | Nail fold capillaroscopy (secondary), ANA, anti-SCl-70, anti-centromere, CBC |
| DD | Acrocyanosis, pernio (chilblains), vibration white finger, digital ischemia |
| Treatment | Avoid cold; calcium channel blockers (nifedipine); prostanoids; phosphodiesterase inhibitors; sympathectomy |
| OPD Rx | Nifedipine LA 30–60 mg OD or Amlodipine 5–10 mg OD; hand warmers |
| Contraindication | Beta-blockers (worsen vasospasm) |
19. FIBROMUSCULAR DYSPLASIA (FMD)
| Feature | Detail |
|---|---|
| Population | Young/middle-aged women; renal and carotid arteries |
| Findings | Renovascular hypertension, TIA/stroke, cervical bruit, headache, pulsatile tinnitus |
| Investigation | CTA/MRA — "string of beads" appearance; renal angiography |
| DD | Atherosclerosis, Takayasu arteritis, neurofibromatosis |
| Treatment | Renal: Percutaneous transluminal angioplasty (PTA); carotid: PTA or CEA |
| OPD Rx | Aspirin 75 mg OD; antihypertensives (ACE-I for renal FMD) |
| Contraindication | Stenting as first-line (PTA preferred) |
20. POLYARTERITIS NODOSA (PAN)
| Feature | Detail |
|---|---|
| Vessel | Medium-sized arteries (non-capillary, no lung involvement) |
| Findings | Fever, weight loss, mononeuritis multiplex, livedo reticularis, renal/mesenteric/coronary artery involvement, HBV association |
| Investigation | Biopsy (necrotizing arteritis), mesenteric/renal angiography (microaneurysms), HBsAg, ANCA (usually negative) |
| DD | MPA (ANCA+), Kawasaki disease, SLE vasculitis |
| Treatment | Prednisolone + cyclophosphamide; HBV-associated: antivirals + plasma exchange |
| OPD Rx | Prednisolone 1 mg/kg/day + Azathioprine (maintenance) |
| Contraindication | Long-term cyclophosphamide (hemorrhagic cystitis, bladder cancer — use mesna) |
PART III — VENOUS DISEASES
21. DEEP VEIN THROMBOSIS (DVT)
| Feature | Detail |
|---|---|
| Virchow's Triad | Stasis, hypercoagulability, endothelial injury |
| Findings | Unilateral leg swelling, calf pain, Homan sign (nonspecific), erythema, warmth |
| Investigation | D-dimer (high sensitivity, low specificity), Duplex USS (gold standard), Well's score |
| DD | Cellulitis, Baker's cyst rupture, lymphedema, muscular hematoma |
| Treatment | LMWH bridging to warfarin OR DOAC (rivaroxaban, apixaban) × ≥3 months; IVC filter if anticoagulation contraindicated |
| OPD Rx | Rivaroxaban 15 mg BD × 21 days then 20 mg OD; compression stockings |
| Contraindication | DOACs in pregnancy (use LMWH); anticoagulation with active major bleeding |
22. PULMONARY EMBOLISM (PE)
| Feature | Detail |
|---|---|
| Massive PE | Hemodynamic instability (SBP <90 mmHg) |
| Findings | Dyspnea, pleuritic chest pain, tachycardia, hemoptysis; S1Q3T3 on ECG; right heart strain |
| Investigation | CT pulmonary angiography (gold standard), D-dimer, V/Q scan, ECHO (right heart strain), troponin/BNP (prognosis) |
| DD | STEMI, pneumothorax, aortic dissection, pericarditis |
| Treatment | Massive PE: thrombolysis (tPA); submassive/low-risk: anticoagulation; surgical embolectomy if thrombolysis fails |
| OPD Rx | Rivaroxaban or Apixaban; warfarin with INR 2–3; duration per risk stratification |
| Contraindication | Thrombolysis in active intracranial bleed or recent surgery |
23. VARICOSE VEINS
| Feature | Detail |
|---|---|
| Definition | Dilated, tortuous superficial veins due to valve incompetence |
| Findings | Aching, heaviness, prominent veins, ankle edema; Trendelenburg test positive; complications: phlebitis, bleeding, ulceration |
| Investigation | Duplex USS (saphenofemoral/saphenopopliteal junction incompetence), CEAP classification |
| DD | Venous malformation, Klippel-Trenaunay syndrome, DVT |
| Treatment | Conservative: compression stockings; Endovenous: EVLA (laser), RFA; Surgical: stripping/ligation; Sclerotherapy |
| OPD Rx | Compression stockings grade II (23–32 mmHg); leg elevation; MICRONIZED PURIFIED FLAVONOID FRACTION (MPFF) |
| Contraindication | Compression in PAD (ABI <0.8); sclerotherapy in allergy to sclerosant |
24. CHRONIC VENOUS INSUFFICIENCY (CVI)
| Feature | Detail |
|---|---|
| Findings | Varicose veins, edema, lipodermatosclerosis, venous eczema, haemosiderin staining, venous ulcer (medial malleolus) |
| Investigation | Duplex USS, plethysmography; CEAP classification (C0–C6) |
| DD | Arterial ulcer (lateral malleolus, punched out), lymphedema, vasculitis ulcers |
| Treatment | Compression therapy (cornerstone), wound care; venotonics; surgical correction of reflux |
| OPD Rx | 4-layer compression bandaging; pentoxifylline 400 mg TDS; topical hydrocolloid dressings |
| Contraindication | High-compression in ABI <0.8 |
25. SUPERIOR VENA CAVA SYNDROME (SVC Syndrome)
| Feature | Detail |
|---|---|
| Causes | Lung cancer (most common), lymphoma, SVC thrombosis, fibrosing mediastinitis |
| Findings | Facial/arm edema, venous distension in neck/chest wall collaterals, dyspnea, Pemberton sign, plethora |
| Investigation | CT chest with contrast (obstruction level), biopsy, bronchoscopy if malignant |
| DD | Cardiac tamponade, tension pneumothorax, SVC compression vs thrombosis |
| Treatment | Malignant: chemotherapy/radiotherapy ± SVC stenting; benign: anticoagulation; dexamethasone for edema |
| OPD Rx | Dexamethasone 4–8 mg BD; diuretics (furosemide); head elevation |
| Contraindication | Biopsy delay if rapidly deteriorating — treat empirically |
26. INFERIOR VENA CAVA SYNDROME
| Feature | Detail |
|---|---|
| Causes | Tumor compression (RCC, HCC), thrombosis, IVC filter complication |
| Findings | Bilateral leg edema, prominent abdominal wall collaterals, DVT, back pain |
| Investigation | CT abdomen/pelvis with contrast, MR venography, echocardiography |
| DD | Bilateral DVT, hepatic venous obstruction, right heart failure |
| Treatment | Anticoagulation; IVC stenting; treat underlying cause (tumor resection) |
| OPD Rx | Warfarin (INR 2–3) or DOAC; compression stockings |
| Contraindication | IVC filter in coagulopathy without anticoagulation bridge |
27. THROMBOPHLEBITIS (Superficial Venous Thrombosis)
| Feature | Detail |
|---|---|
| Findings | Tender, erythematous, palpable cord along superficial vein; warmth; migratory thrombophlebitis (malignancy sign) |
| Investigation | Duplex USS (rule out DVT extension), CBC, coagulation screen; if migratory — tumor markers |
| DD | DVT, cellulitis, lymphangitis |
| Treatment | NSAIDs + compression + LMWH if near saphenofemoral junction or extensive |
| OPD Rx | Fondaparinux 2.5 mg SC OD × 45 days (if >5 cm) or NSAID + topical heparin |
| Contraindication | Anticoagulation in thrombocytopenia (HIT risk) |
28. MAY-THURNER SYNDROME (Iliac Vein Compression)
| Feature | Detail |
|---|---|
| Definition | Right iliac artery compresses left iliac vein → left-sided DVT |
| Findings | Left leg DVT (young woman), pelvic congestion syndrome |
| Investigation | CT/MR venography, IVUS (intravascular USS) — gold standard |
| DD | Thrombophilia-related DVT, pelvic mass |
| Treatment | Catheter-directed thrombolysis + iliac vein stenting |
| OPD Rx | Anticoagulation + follow-up imaging |
| Contraindication | Anticoagulation alone (stent needed to relieve compression) |
PART IV — ADDITIONAL VASCULAR & LYMPH NODE DISEASES
29. KAWASAKI DISEASE
| Feature | Detail |
|---|---|
| Population | Children <5 years; medium vessel vasculitis |
| Diagnostic Criteria | Fever ≥5 days + 4 of: conjunctivitis, rash, lymphadenopathy, oral changes (strawberry tongue), extremity changes |
| Investigation | Echo (coronary artery aneurysms), CBC (thrombocytosis in subacute), ESR, CRP, UA |
| DD | Scarlet fever, measles, juvenile idiopathic arthritis, staphylococcal toxic shock |
| Treatment | IVIG 2 g/kg single dose + Aspirin 80–100 mg/kg/day (acute) then 3–5 mg/kg/day (maintenance) |
| OPD Rx | Aspirin 3–5 mg/kg OD + Echo follow-up at 2 and 6 weeks |
| Contraindication | Live vaccines for 11 months after IVIG |
30. HENOCH-SCHÖNLEIN PURPURA (IgA Vasculitis)
| Feature | Detail |
|---|---|
| Tetrad | Palpable purpura + arthritis + abdominal pain + renal involvement (IgA nephropathy) |
| Findings | Buttocks/lower limb purpura; intussusception risk; mesangial IgA deposits on biopsy |
| Investigation | Skin/renal biopsy (IgA deposits by IF), urinalysis, IgA levels, coagulation screen |
| DD | Meningococcemia, thrombocytopenic purpura, SLE vasculitis |
| Treatment | Usually self-limiting; NSAIDs for arthritis; steroids for severe abdominal/renal disease |
| OPD Rx | Prednisolone 1 mg/kg/day if nephritis; supportive care; monitor BP and urine |
| Contraindication | NSAIDs in renal impairment |
31. WEGENER GRANULOMATOSIS (GPA — Granulomatosis with Polyangiitis)
| Feature | Detail |
|---|---|
| Triad | Upper respiratory tract + lower respiratory tract + renal involvement |
| Findings | Saddle-nose deformity, epistaxis, hemoptysis, pulmonary nodules, rapidly progressive GN |
| Investigation | c-ANCA (PR3-ANCA) positive, CXR/CT (nodules, cavities), renal biopsy (necrotizing pauci-immune GN) |
| DD | MPA, Churg-Strauss, Goodpasture syndrome, SLE |
| Treatment | Induction: Rituximab or cyclophosphamide + prednisolone; Maintenance: azathioprine + prednisolone |
| OPD Rx | Rituximab 375 mg/m² weekly × 4 doses; Cotrimoxazole (PCP prophylaxis) |
| Contraindication | Cyclophosphamide without mesna (hemorrhagic cystitis) |
32. MICROSCOPIC POLYANGIITIS (MPA)
| Feature | Detail |
|---|---|
| Findings | Rapidly progressive GN, pulmonary hemorrhage (DAH), palpable purpura; p-ANCA (MPO) positive |
| Investigation | p-ANCA, renal biopsy, HRCT chest |
| DD | GPA, PAN, Goodpasture syndrome |
| Treatment | Cyclophosphamide + prednisolone; maintenance: azathioprine; Rituximab alternative |
| OPD Rx | Prednisolone + Azathioprine 2 mg/kg/day maintenance |
| Contraindication | Allopurinol with azathioprine (xanthine oxidase inhibition → toxicity) |
33. CHURG-STRAUSS SYNDROME (EGPA)
| Feature | Detail |
|---|---|
| Findings | Asthma + eosinophilia + vasculitis; mononeuritis multiplex, cardiac involvement, p-ANCA (50%) |
| Investigation | Eosinophil count >1.5×10⁹/L, p-ANCA, tissue biopsy, HRCT, echocardiogram |
| DD | Hypereosinophilic syndrome, PAN, Loeffler syndrome |
| Treatment | Prednisolone + cyclophosphamide (severe); Mepolizumab (anti-IL-5, FDA approved) |
| OPD Rx | Mepolizumab 300 mg SC every 4 weeks; prednisolone taper |
| Contraindication | Leukotriene antagonists (can unmask latent disease) |
34. THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP)
| Feature | Detail |
|---|---|
| Pentad | Microangiopathic hemolytic anemia + thrombocytopenia + fever + renal failure + neurological symptoms |
| Pathophysiology | ADAMTS13 deficiency → uncleaved vWF → platelet microthrombi |
| Investigation | Blood film (schistocytes), ADAMTS13 activity (<10%), LDH, PT/APTT (normal), CBC |
| DD | HUS (Shiga toxin; renal > neuro), DIC, HELLP |
| Treatment | URGENT plasma exchange (PEX); caplacizumab; corticosteroids; rituximab |
| OPD Rx | Rituximab in relapsing/refractory TTP; caplacizumab |
| Contraindication | Platelet transfusion (worsens thrombosis — "fuel on the fire") |
35. ANTIPHOSPHOLIPID SYNDROME (APS)
| Feature | Detail |
|---|---|
| Findings | Recurrent arterial/venous thrombosis, recurrent miscarriage (>3); livedo reticularis; Sapporo criteria |
| Investigation | Lupus anticoagulant, anti-cardiolipin IgG/IgM, anti-β2GP1 (on two occasions ≥12 weeks apart) |
| DD | Inherited thrombophilia (Factor V Leiden), SLE, protein C/S deficiency |
| Treatment | Venous: Warfarin (INR 2–3); Arterial: Warfarin INR 3–4 ± aspirin; Obstetric: LMWH + aspirin |
| OPD Rx | Warfarin long-term; hydroxychloroquine in SLE-associated APS |
| Contraindication | DOACs in triple-positive APS (high thrombosis risk — use warfarin) |
36. HEREDITARY HEMORRHAGIC TELANGIECTASIA (Osler-Weber-Rendu)
| Feature | Detail |
|---|---|
| Findings | Mucocutaneous telangiectasias, recurrent epistaxis, GI bleeding, AVM (pulmonary, hepatic, cerebral) |
| Investigation | Clinical diagnosis (Curaçao criteria ≥3), CT angiography for AVM, MRI brain, hemoglobin |
| DD | CREST syndrome, hereditary iron deficiency, von Willebrand disease |
| Treatment | Iron replacement; laser coagulation for telangiectasias; embolization for large AVM; bevacizumab for severe bleeds |
| OPD Rx | Iron sulfate 200 mg TDS; tranexamic acid for bleeds; nasal saline |
| Contraindication | Anticoagulation without careful monitoring (exacerbates bleeding) |
37. KLIPPEL-TRENAUNAY SYNDROME
| Feature | Detail |
|---|---|
| Triad | Port-wine stain + varicose veins + soft tissue/bony hypertrophy of limb |
| Findings | Unilateral limb; marginal vein of Servelle; risk of PE; lymphatic malformation |
| Investigation | MRI (extent), Duplex USS, coagulation screen (DIC in extensive malformation) |
| DD | Parkes-Weber syndrome (AVF component), Sturge-Weber, hemihypertrophy |
| Treatment | Compression therapy; sclerotherapy; laser for port-wine stain; anticoagulation for thrombosis |
| OPD Rx | Compression stocking; sirolimus (mTOR inhibitor) in complex cases |
| Contraindication | Surgery without pre-operative imaging (risk of AVF) |
38. LYMPHANGIOLEIOMYOMATOSIS (LAM)
| Feature | Detail |
|---|---|
| Population | Young women of reproductive age; TSC gene mutation |
| Findings | Progressive dyspnea, chylothorax, pneumothorax, haemoptysis; renal angiomyolipoma |
| Investigation | HRCT chest (bilateral cysts), VEGF-D >800 pg/mL (diagnostic), serum TSC mutation, lung biopsy |
| DD | Emphysema, Langerhans cell histiocytosis, Birt-Hogg-Dubé |
| Treatment | Sirolimus (mTOR inhibitor) — stabilizes or improves lung function; lung transplant in end-stage |
| OPD Rx | Sirolimus 2 mg/day (target trough 5–15 ng/mL); avoid estrogen (promotes disease) |
| Contraindication | Estrogen-containing OCP; progesterone controversial |
39. KAPOSI SARCOMA (Vascular Tumor of Lymphatics)
| Feature | Detail |
|---|---|
| Types | AIDS-related (most common), Classic (elderly Mediterranean men), Endemic (African), Iatrogenic (post-transplant) |
| Findings | Violaceous skin lesions (lower extremities → face), mucosal/pulmonary/GI involvement; HHV-8 driven |
| Investigation | Skin biopsy (spindle cells, HHV-8 LANA staining), HIV viral load, CD4 count, CT staging |
| DD | Bacillary angiomatosis (Bartonella), pyogenic granuloma, hemangioma, melanoma |
| Treatment | AIDS-KS: ART (HAART) ± chemotherapy (liposomal doxorubicin); localized: radiation/cryotherapy |
| OPD Rx | Liposomal doxorubicin 20 mg/m² q3w; ART optimization; imiquimod topical (cutaneous) |
| Contraindication | Immunosuppression continuation in transplant-KS (reduce/change if possible) |
40. LYMPHOMA — PRIMARY CNS (PCNSL)
| Feature | Detail |
|---|---|
| Population | Immunocompromised (AIDS, post-transplant), elderly; diffuse large B-cell most common |
| Findings | Confusion, focal deficits, raised ICP; periventricular ring-enhancing lesions on MRI |
| Investigation | MRI brain (contact ventricular wall), CSF cytology + flow cytometry, vitreoretinal biopsy, stereotactic brain biopsy; HIV test |
| DD | Glioblastoma, metastases, cerebral toxoplasmosis (AIDS), abscess |
| Treatment | High-dose methotrexate (HD-MTX) ± rituximab ± temozolomide; whole brain RT in refractory; consolidation ASCT |
| OPD Rx | HD-MTX 3.5 g/m² IV q2w × 6 cycles; leucovorin rescue; dexamethasone for edema |
| Contraindication | Steroids before biopsy (can cause "vanishing" lymphoma — falsely negative biopsy) |
QUICK REFERENCE SUMMARY TABLE
| # | Disease | Key Investigation | First-line Treatment | Critical Contraindication |
|---|---|---|---|---|
| 1 | Primary Lymphedema | Lymphoscintigraphy | CDT + compression | Diuretics |
| 2 | Secondary Lymphedema | Duplex USS | CDT + treat cause | Tight dressings in infection |
| 3 | Acute Lymphangitis | Blood culture | Penicillin/Amoxiclav | Penicillin if allergic |
| 4 | Acute Lymphadenitis | FNAC | Antibiotics ± I&D | Empirical steroids |
| 5 | TB Lymphadenitis | FNAC + GeneXpert | 2HRZE/4HR | Rifampicin in liver disease |
| 6 | Hodgkin Lymphoma | Excisional biopsy | ABVD | Bleomycin in lung disease |
| 7 | Non-Hodgkin Lymphoma | Biopsy + IHC | R-CHOP | Rituximab in HBV |
| 8 | Filariasis | Nocturnal blood smear | DEC + Albendazole | DEC in onchocerciasis |
| 9 | Castleman Disease | Excisional biopsy | Siltuximab/Surgery | Live vaccines |
| 10 | Kikuchi Disease | Excisional biopsy | NSAIDs | Steroids without ruling out lymphoma |
| 11 | Atherosclerosis | Lipid profile, ABI | Statin + Aspirin | Fibrate + statin combo |
| 12 | PAD | ABI + Duplex USS | Cilostazol + Aspirin | Cilostazol in CCF |
| 13 | Aortic Aneurysm | CT Aortogram | EVAR/open repair | Vigorous exertion |
| 14 | Aortic Dissection | CT Aortogram | Surgery (A) / Labetalol (B) | Vasodilator alone |
| 15 | Takayasu Arteritis | CTA/MRA + PET | Prednisolone + MTX | Revascularization in active disease |
| 16 | Giant Cell Arteritis | Temporal artery biopsy | Prednisolone 40–60 mg | Delay in treatment |
| 17 | Buerger Disease | Arteriography | Smoking cessation | Nicotine replacement |
| 18 | Raynaud Phenomenon | Nailfold capillaroscopy | Nifedipine | Beta-blockers |
| 19 | Fibromuscular Dysplasia | CTA (string of beads) | PTA | Stenting first-line |
| 20 | Polyarteritis Nodosa | Angiography/biopsy | Prednisolone + CYC | CYC without mesna |
| 21 | DVT | Duplex USS, D-dimer | DOAC (Rivaroxaban) | DOAC in pregnancy |
| 22 | Pulmonary Embolism | CTPA | Anticoagulation/tPA | Thrombolysis in ICH |
| 23 | Varicose Veins | Duplex USS | Compression/EVLA/RFA | Compression in PAD |
| 24 | CVI | Duplex USS (CEAP) | Compression + pentoxifylline | High compression in ABI <0.8 |
| 25 | SVC Syndrome | CT chest | Stenting ± chemoRT | Biopsy delay if deteriorating |
| 26 | IVC Syndrome | CT abdomen | Stenting + anticoagulation | IVC filter without bridge |
| 27 | Superficial Thrombophlebitis | Duplex USS | Fondaparinux/NSAIDs | Anticoagulation in HIT |
| 28 | May-Thurner Syndrome | IVUS | CDT + Stenting | Anticoagulation alone |
| 29 | Kawasaki Disease | Echocardiogram | IVIG + Aspirin | Live vaccines post-IVIG |
| 30 | HSP / IgA Vasculitis | Skin/renal biopsy | NSAIDs/steroids | NSAIDs in renal failure |
| 31 | GPA (Wegener) | c-ANCA, renal biopsy | Rituximab + prednisolone | CYC without mesna |
| 32 | Microscopic Polyangiitis | p-ANCA | CYC + prednisolone | Allopurinol + azathioprine |
| 33 | EGPA (Churg-Strauss) | Eosinophilia + p-ANCA | Prednisolone + mepolizumab | Leukotriene antagonists |
| 34 | TTP | ADAMTS13, blood film | Plasma exchange | Platelet transfusion |
| 35 | Antiphospholipid Syndrome | LA, aCL, anti-β2GP1 | Warfarin (INR 2–3) | DOACs in triple-positive |
| 36 | HHT (Osler-Weber-Rendu) | CTA (AVM), Curaçao | Iron + embolization | Uncontrolled anticoagulation |
| 37 | Klippel-Trenaunay | MRI + Duplex USS | Compression + sirolimus | Surgery without imaging |
| 38 | LAM | HRCT + VEGF-D | Sirolimus | Estrogen-containing OCP |
| 39 | Kaposi Sarcoma | Biopsy (HHV-8) | ART + liposomal doxorubicin | Continue immunosuppression in transplant KS |
| 40 | Primary CNS Lymphoma | MRI + CSF cytology | HD-Methotrexate + rituximab | Steroids before biopsy |
KEY ICO (International Classification & Coding) / ICD-10 CODES
| Disease | ICD-10 |
|---|---|
| Primary lymphedema | Q82.0 |
| Secondary lymphedema | I89.0 |
| Lymphangitis | I89.1 |
| Hodgkin lymphoma | C81 |
| Non-Hodgkin lymphoma | C82–C86 |
| Atherosclerosis | I70 |
| Aortic aneurysm | I71 |
| Aortic dissection | I71.0 |
| DVT | I80.2 |
| Pulmonary embolism | I26 |
| Varicose veins | I83 |
| Takayasu arteritis | M31.4 |
| Giant cell arteritis | M31.6 |
| Buerger disease | I73.1 |
| Kawasaki disease | M30.3 |
| TTP | M31.1 |
| Kaposi sarcoma | C46 |
Sources: Harrison's Principles of Internal Medicine 22e, Robbins & Kumar Pathologic Basis of Disease, Andrews' Diseases of the Skin, Braunwald's Heart Disease, Fitzpatrick's Dermatology
This is a shared conversation. Sign in to Orris to start your own chat.