Case Report Leg Edema in Hiv

7/28/2019 Case Report Leg Edema in Hiv

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Case 5 Leg Edema

26 year-old woman presents to a clinic in rural Malawi with chief complaint of bilateral

lower extremity edema. She noted persistent bipedal swelling one year ago when

pregnant with her fourth child, and is now worried about a few “ugly bumps” which

appeared several months ago on both feet. She complains of tightness and mild pain inher legs and thighs, especially with long walks. The swelling does not improve when

recumbent. There is no history of leg ulcers. She has had no fever, night sweats,

shortness of breath, dyspnea on exertion, orthopnea, cough, jaundice, abdominal pain,dysuria, hematuria, nausea, vomiting, weight loss, melena or hematochezia. Her urine has

been clear, and she also denies any prolonged febrile illnesses before and since her edema

started.

Her prior pregnancies were not associated with edema, and her last pregnancy and

delivery were normal. Of note, aside from a child who died recently after developingedema all over his body, she does not recall anyone in her village having similar swelling

in their legs. She lives in a relatively flat region, with no hills and no rivers/lakes. Her

HIV status is unknown. There is no personal history of TB, and no known TB contacts.She has never smoked and does not drink alcohol. Her husband died 4 months ago from a

diarrheal illness, but had been chronically ill with weight loss and weakness for some

time before that.

Physical Exam:

T: 37.3 C HR: 72, BP 110/65, RR: 14, Wt: 47.5 kg, Ht: 1.55m

General: No acute distress, thin appearing, but not wasted

HEENT: Pupils equally reactive to light, extraocular movements intact,conjunctiva slighty pale. No icterus, cervical lymphadenopathy, thrush or

oral lesions.

CHEST: Lungs clear bilaterally. Heart sounds regular without murmurs, rubs or gallops. Normal PMI. No JVP visible above sternum at 20 degrees

elevation.

ABDOMEN: Soft, without tenderness, distention, or shifting dullness. Liver is 2cm below costal margin, spleen tip barely palpable, no CVA tenderness

NEURO: No focal abnormalities are found

EXTREMITIES: Normal upper extremities. Lower extremities grossly edematous,non-pitting, non-tender, firm/woody to touch in both feet and up to mid-

shin bilaterally. +Bilateral inguinal lymphadenopathy, with ~10cm area of

firm, hyperpigmented, edematous skin in groin

SKIN: ~0.5 to 1cm verrucous growths on both feet (totaling 8 lesions), non-

umbilicated, non-ulcerated, non-tender. On upper right upper thigh ~15hyperpigmented, firm, nodular lesions ~1-1.5cm in diameter noted,

nontender, non-pruritic. (See pictures)



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Figure 1. Right foot.

Figure 2. Right thigh.



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1. What is the “frame” of this case (i.e. the key clinical features the final diagnosis

has to be consistent with or explain)?

-- 26 year old woman in rural Malawi

-- husband’s recent death from chronic illness -- bilateral lower extremity swelling, non-pitting,-- swelling is progressive over 1 year, and appeared before skin lesions appeared

-- no shortness of breath or cough, JVP flat, no crackles or ascites

-- verrucous lesions on feet with hyperpigmented nodules bilaterally,-- non-tender lymphadenopathy with 10 cm of firm edema in groin

2. What is your differential diagnosis?

What elements of the history and physical support or reject your diagnoses?

Differential Diagnosis:

Kaposi Sarcoma, filarial elephantiasis, fungal infection (e.g. chromoblastomycosis), podoconiosis, chronic renal failure, congestive heart disease, chronic liver failure,

chronic venous stasis.

Filarial disease is found extensively in sub-Saharan African countries, and often resultsin woody edema of the lower extremities. However, edema generally progresses very

slowly, over the course of years, so it would be unusual for this patient to develop such

extensive edema in just one year unless the history is (very) inaccurate. Also, filarial edema usually presents unilaterally, and this patient has bilateral disease. The absence

of similar symptoms among other people in patient’s village also points away from

filarial disease, as it tends to occur in clusters.

Fungal infections can also present with this clinical picture, with cauliflower like

coalesced growths, firm, non-pitting edema, and satellite lesions. These advanced

infections are usually seen in tropical and subtropical regions, mostly affecting the rural population. This infection, called chromoblastomycosis, can be caused by a number of

fungal species, most commonly Fonsecaea pedrosoi, Phialophora verrucosa, and

Cladosporium Carrionii. However, fungal infection is also usually a process that takes years to become this advanced, so our suspicion in this patient is less. Another aspect

that points less to fungal infection is the existence of non-pitting edema prior to any skin

findings - which suggests a primary lymphatic/vascular process rather than a primary

skin process.

Podoconiosis, or non-filarial elephantiasis, is a chronic condition caused by exposure of

bare feet to inorganic elements in certain types of soil that over the course of years can

lead to lymphatic fibrosis and asymmetric edema of the lower extremities. This disease ismost prevalent in equatorial regions of Africa and especially in areas with hills. This

condition is uncommon epidemiologically in Malawi.



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Chronic renal failure, heart failure, liver failure, and chronic venous stasis all can

present with woody edema. For this patient, her story has no hallmarks of renal failure

(normal urine, blood pressure is normal), heart failure (no SOB, no rales on lung exam,

no elevated JVP, no cardiac murmur, normal heart rate), nor liver failure (no ascites, no jaundice, no abdominal pain, non-pitting edema instead of pitting). Chronic venous

stasis is also less likely given the time course (usually over many years), non-pitting nature of patient’s edema, and l ack of ulcers.

Kaposi Sarcoma (KS) is a neoplastic process of endothelial cells and is associated with

HHV-8 (Human Herpes Virus-8) infection, and has been described mostly in people withimmunosuppression, especially among HIV patients. KS is one of the most common

tumors in HIV positive patients in Sub-Saharan Africa, and is the most common cause of

unilateral lower extremity edema in Malawi. These tumors are highly vascular and can

grow rapidly. The risk for KS is highest in those with lower CD4 counts, but can occur at any CD4 level. Most commonly, the disease presents with dark, nodular skin lesions,

matted lymphadenopathy (often in the inguinal region), and woody, non-pitting edema of

the areas involved. KS lesions can be found anywhere in the body, including the lungs(often producing hemoptysis and mimicking TB), and gut (causing melena). While most

cases of KS currently occur in HIV infected individuals, there is a slowly progressive

form of KS that predates the HIV pandemic, that is not associated with HIV infection or

immunosuppression, and that is endemic in the so called Kaposi Sarcoma “Belt” inequatorial Africa. In this patient, KS should be one of the top diagnoses on our

differential given the physical findings, the epidemiologic background, and the

circumstances of her husband’s death (very suspicious for AIDS). If patient’s HIV statu sis positive, then the likelihood of KS increases even further.

3. What testing is indicated at this point?

Commonly available tests:

Urine dip analysis Specific Gravity: 1.015, nitrite negative, no WBCs, no RBCs, no

protein, no casts, no glucose, no ketones.

HIV rapid test positive

Creatinine 1.2 mg/dl

4. How do these tests narrow your differential diagnosis?

We can likely rule out chronic renal failure as the creatinine is normal and the urine dip

shows no protein. The HIV seropositivity should greatly increase our suspicion for KS,and should prompt us to further characterize the circumstances of the patient’s

husband’s death as he likel y died of an AIDS-related illness (consider discussing the use

of the verbal autopsy to establish husband’s likely diagnosis). We should also attempt toobtain a more detailed history and exam to give the patient a WHO clinical stage (e.g.



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weight loss, recent infections such as abscesses, pneumonia, zoster, etc.). A clinical

diagnosis of KS would have the patient at WHO clinical stage 4 AIDS. It would be

important to document any potential visceral involvement of KS, including detailed exam

of the conjunctiva, oral mucosa, and a fecal occult blood test. At this point, while muchlower on the differential diagnosis (likely < 5% pre-test probability), we still cannot rule

out filarial disease, podoconiosis (though less likely from epidemiologic standpoint), or fungal process.

5. What should be your initial management approach be for this patient? Are there

other tests that can be useful?

A urine pregnancy test should be done as PCP prophylaxis is indicated for this patient at

this time. In many resource-poor settings, CD4 counts are not readily available or are

cost-prohibitive, in which case a clinical diagnosis of KS would prompt us to initiate HAART as soon possible, as KS is a WHO clinical stage 4 diagnosis. The choice of

initial therapy will depend on the patient’s pregnancy status. In most areas, histo-

pathological diagnosis will not be available to confirm a diagnosis of KS, but where possible, a punch biopsy should be performed to diagnosis KS and rule out a fungal

process.

Other testing:

Urine pregnancy (HCG) test negative

Blood film (midnight) for filariasis no filarial forms seen

CD4 120 cells/mm3

Guaiac positive

Skin scraping microscopy after KOH exposure no fungal forms seen

Punch biopsy of thigh lesion no parasites seen, no hyphae, yeast or other fungal forms

seen, hypervascular invasion consistent with Kaposi Sarcoma.

6. Any other steps you would take at this point?

It would be extremely important to arrange for the patient’s children to be tested. We should also encour age the patient’s current and past sexual partners to be tested. We

should also want to know if the patient is breastfeeding her infant, and if so, to determine

the baby’s (depending on age) serologic status using DNA PCR or rapid testing. If the

child is HIV-negative, and there are resources available, the mother should be offered formula and a source of clean water. If the child is seropositive, then the mother should

continue to breastfeed.



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The patient should also have a thorough evaluation to rule out co-infection with sexually

transmitted illnesses (STI), and TB.

7. What are the long-term treatment options for this patient’s condition?

The recommended initial therapy in HIV-positive patients with a new diagnosis of KS isinitiation immediately of HAART. Often, with HAART alone, KS lesions may improve or stabilize. Of note, patients can present shortly (within days to weeks) after HAART with

worsening KS signs and symptoms that could be consistent with KS immune

reconstitution (KS-IRIS). Depending on the severity of the IRIS, treatment withcorticosteroids may be necessary.

For KS lesions in isolation, cryotherapy, surgical excision, radiation, or intralesional

chemotherapy have been used, primarily in resource-rich settings. For more extensive KS that has not responded despite HAART, systemic chemotherapeutics have been found

to be useful (where available). A number of systemic chemotherapies are in use in

resource-rich areas including liposomal doxorubicin and paclitaxel. In resource-poor regions, often only vincristine and bleomycin are available. Use of chemotherapeutic

agents should not be attempted without adequate laboratory abilities (minimum LFTs,

complete blood counts, basic chemistries). Familiarity with pre-hydration techniques

and the management of adverse reactions (e.g. anaphylaxis, fever and neutropenia) isalso crucial before starting chemotherapeutic agents. Thus, because of the level of

resources needed to treat advanced KS, the patient may need to be transferred to a

regional or tertiary health care center if possible.

Further reading:

Szajerka, T., Jabłecki, J. Kaposi’s sarcoma revisited. AIDS Reviews 2007;9:230-6Vanni T, Sprinz E, Machado MW, Santana Rde C, Fonseca BA, Schwartsmann G.

Systemic treatment of AIDS-related Kaposi sarcoma: current status and perspectives.

Cancer Treatment Reviews. 2006 Oct;32(6):445-55.

Phuoc V. Le, MD, MPH, Jonathan Crocker, MD – Partners in Health -- Malawi

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Case Report Leg Edema in Hiv