Acute Lymphedema

Presented by Dr. Henry Foong
Ipoh, Malaysia

The patient is a 50-yr-old man with a history of swelling of the right leg for 2 months. He noted that the right foot was swollen initially and a few weeks later the swelling has progressed to the right leg.  Over the past week the swelling has progressed to the right thigh. The swelling was mildly painful but overall the past week the swelling has progressed a lot.  The swelling was so severe that he was unable to wear his normal shoe. He has a history of hypertension and a history of “stroke” where he lost his consciousness and was told by his doctor he suffered from a minor stroke.  He was treated as cellulitis by his doctor with penicillin but did not improve.  In fact he had developed a generalised maculopapular eruptions over the trunk a week after starting on IV penicillin.

O/E: Shows a severe unilateral swelling of the right leg extending from the foot to the thigh.  There was superficial erythema, desquamation with small blisters. The affected areas are well-demarcated especially on right thigh.  The leg swelling was indurated and mildly pitting.  The measured circumference of the right leg at mid-calf level was 51 cm compared with the corresponding left leg of 39 cm.  His right inguinal nodes were markedly enlarged. Rest of exam was unremarkable.

Images: 

Differential Diagnosis

1.  Erysipelas right leg

2.  Filariasis

3.  Deep vein thrombosis

Lab:

Hb 13.5 gm%, TWBC 17,900 (N87%, L10%, E0.1%, B1% M2%) ESR 79mm/hr Biochemistry unremarkable. Culture from the right leg grew Staphylococcal aureus.  Doppler ultrasound right leg did not show any evidence of deep vein thrombosis but enlarged right inguinal nodes.

He was advised to stop penicillin and started on IV moxifloxacin 400mg od, IV hydrocortisone 200mg qid,  and wet compress. Blood was sent for microfilaria (x3) which was negative.

Follow-up Photo after 5 days of IV moxifloxacin and IV hydrocortisone.

 

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Marked improvement after 5 days of IV moxifloxacin and IV hydrocortisone.  There is a 5 cm reduction in the circumference of the right call. The patient would be scheduled for a MRI lower pelvis/right thigh soon, but he has refused since he is feeling so much better.
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Your thoughts will be appreciated.

Erysipelas in a 69 yo Woman

presented by Henry Foong
Ipoh, Malaysia

A 69-yr-old woman was seen with a 2-day history of rapidly enlarged swelling on the right cheek.  Initially she felt some discomfort on the right ear and then involved the right cheek.  She had no fever.

There was no history of trauma.  She had a history of hypertension. 

Examination showed unilateral swelling on the right cheek with increased temperature, tenderness and redness extending from right forehead to the right lower jaw.  It appeared oedematous, with swelling of the ipsilateral upper eyelid. It had a well defined raised border. The rash did not cross the bridge of the nose to the opposite cheek. No blisters were seen. Her regional nodes were not enlarged.

Clinically she has erysipelas.

TWBC 12,900 (N84%)

blood sugar  8.5 mol/l

BU and serum electrolytes normal

LFT normal

Erysipelas affects the superficial layer of the skin while cellulitis affects the deeper part of the dermis. There were no clusters of vesicles or erosions to suggest herpes zoster. She was not keen to be admitted and preferred oral medication as an outpatient  She was treated with oral cefuroxime 500mg bd for 10 days with wet compress dressing on the affected face. The erysipelas cleared with the treatment.

Toxic Erythema

Presented by Dr. Henry Foong,

Ipoh, Malaysia

Abstract:  41 yo man with short history of toxic erythema

HPI: The patient is a 41 yr old restaurant worker who presented with 5 day history of bilateral and symmetrical erythema over the loins, groins and knees.

About a week ago, he had a furunculosis on upper back and was prescribed Augmentin. Two days later he developed high fever and was treated with ciprofloxacin by a physician. A day later he noticed itchy rashes on both thighs and since then, the macular erythema spread symmetrically to the loins, upper shoulders and knees.  He has no fever.

His past medical history was non-contributory.

Examination showed bilateral and symmetrical diffuse erythema on the thighs, knees, loins and upper shoulders. Over the loins, there was symmetrical and bilateral edematous areas  with vesicles over a background of erythema.  His scalp, oral cavity and genitalia were clear.

Clinical Photos:

Lab: Culture and a skin biopsy has been done. Culture negative.  Biopsy pending.

 TWBC 3200 (N 85.1%  L 10.3%  E0 .5%  M3.4%  B 0.8%)  ESR 36

Diagnosis: Toxic erythema

Differentials:  Streptococcal/Staph cellulitis 

Questions and Comments:  The presence of blisters /edema on the loin is worrying.  In the meantime what would you recommend for this patient? Would you use corticosteroids in this patient? I have stopped both augmentin and ciprofloxacin. In terms of dressing, I used wet compress with dil. KMNO4.Have I missed anything?

References:

1.  Miyahara A, et. al.  A new proposal for a clinical-oriented subclassification of baboon syndrome and a review of baboon syndrome. Asian Pac J Allergy Immunol. 2011 Jun;29(2):150-60

Source: Department of Pediatrics, Tokyo Medical University. miyahara.pediatrics@gmail.com

Abstract

OBJECTIVE: To review baboon syndrome (BS). Data Sources: Date sources were obtained from PubMed and Google Scholar: Photographs of baboon syndrome were obtained from our patient.

STUDY SELECTIONS: PubMed and Google Scholar were searched up to June 30, 2010. The search terms were "baboon syndrome", "SDRIFE" and "thimerosal allergy". Reverse references from relevant articles and Google Scholar were also used. As BS is a classical disease and cases of offending agents were relatively old, some references were more than five years old. In order to gather as many cases of offending agents as possible, more than 50 references were collected.

RESULTS AND CONCLUSION: We divided BS into as 4 groups; classical baboon syndrome, topical drug-induced baboon syndrome, systemic drug-induced baboon syndrome and symmetrical drug-related intertriginous and flexural exanthema (SDRIFE). The pathomechanism of BS is still unknown. A delayed type of hypersensitivity reaction, a recall phenomenon, pharmacologic interaction with immune-receptors and anatomical factors may be involved in the causation of BS.  This valuable article is available in Free full text

2. Tan SC, Tan JW.  Symmetrical drug-related intertriginous and flexural exanthema. Curr Opin Allergy Clin Immunol. 2011 Aug;11(4):313-8.

Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore, Singapore. Sze_Chin_Tan@ttsh.com.sg

Abstract

PURPOSE OF REVIEW: Symmetrical drug-related intertriginous and flexural exanthema (SDRIFE), previously termed drug-related baboon syndrome, is a benign and self-limiting type IV hypersensitivity reaction characterized by symmetrical erythema involving the gluteal and intertriginous areas in the absence of systemic involvement. It may also occur in the absence of previous drug exposure.

RECENT FINDINGS: Antibiotics, in particular beta-lactams, comprise the majority of causes of SDRIFE. Other drugs which have been implicated include antihypertensives, radiocontrast media, chemotherapeutic agents, and biologics. Histology of lesional skin is variable with predominance of superficial perivascular inflammatory cell infiltrates. Outcomes of allergy tests are variable with positive delayed intradermal tests reported for penicillin V, allopurinol; positive patch tests for erythromycin, mitomycin, nystatin, pseudoephdrine; positive lymphocyte transformation tests for erythromycin; and positive drug provocation tests for clindamycin, cimetidine, corticosteroids, terbinafine, and valacyclovir.

SUMMARY: Diagnosis of SDRIFE is dependent upon recognition of the clinical morphology and distribution of the rash, and its temporal relationship to the use of the suspected drug. Outcomes of in-vivo and in-vitro tests have been inconsistent, and thus may not be useful in the identification of the putative drug.

Recurrent Toxic Erythema vx. Cellulitis

H.P.I. The patient is a 70 yo woman who has had ~ 10 episodes of a cellulitis-like picture of her legs over the past ten years. She has never had fever or constitutional signs. The process can affect one or both lower extremities. I saw her in 2001 for this and then she was referred by the emergency room yesterday with the same picture. It began with a fall two days ago. She hit her hip -- was in pain and took ibuprofen before this episode. She said she does not recall taking this before other episodes.

O/E: Patchy, fiery erythema of left lower extremity. Very mild erythems of right lower leg (mostly around the ankle).

Clinical Photos:





Labs: Biopsy obtained and CBC ordered

Diagnosis: Is this an atypical cellulitis, or toxic erythema secondary to and NSAID?

Discussion: If the patient remains afebrile and wbc is normal, I'd be inclined to just watch and wait.

A Leg to Stand On

Abstract: 85 yo man with cellulitis and lymphedema s/p bypass surgery

HPI: In 2000, this retired professor had CABG surgery with saphenous vein harvesting of the left leg, In 2002, while visiting Vienna, he was hospitalized for cellulitis of the left leg and treated with i.v. antibiotics. In the subsequent years, he has had progressive lymphedema of the left leg with the development of elephiantiasis nostra verrucosa. In the past 6 months he has had four documented episodes of cellulitis which have necessitated antibiotics. Recently, he has developed a generalized dermatitis. His medications include: atenolol, amiodarone, digoxin, diovan, thyrpid, Coumadin, Lovastatin, ASA. He has a possible allergy to penicillin (has not taken it in 40 years). He is otherwise quite healthy and intellectually alert.

O/E: Chronic lymphedema left leg with erythema, scale and some honey-colored crust. The leg in involved from just below the knee to the foot. The left leg is only minimally warmer than the right leg. In addition, he has a wide-spread dermatitis consisting of erythematous scaly patches on legs, arms and abdomen -- sparing the face.

Photos:






Lab: His internist did a skin culture and CBC b efore sending him here (results not back) . He was on Bactrim at the time of the culture.

Diagnosis: Recurrent Cellulitis s/p saphenous veif harvesting wit the development of chronic lymphedema and early elephiantiasis nostras. This was reported in 1982 (see Reference). The wide-spread dermatitis may be an "id" reaction.

Questions:
1) What has been learned about this entity since 1982?
2) Should he be rechallenged with penicillin and kept on long-term antibiotic prophylaxis.
3) What role does "hypersensitivity" to bacterial exotoxins play in the dermatitis.
4) Does anyone have magic for cases such as this?
5) Allergic contact dermatitis will be ruled out by patch testing.

Comment: My plan at the moment is to "clean up" any residual infection, consider prophylactic antibiotics and work on the dermatitis. I will refer him to a lymphedema center, if possible, for evaluation. Aggressive management of his leg swelling will help. He was told not to bathe the leg and I think this sets him up for infection. Your thoughts will be greatly appreciated. This man's life revolves around his leg. He is a keen student, a political scientist and in his later years he must focus exclusively on a leg. Let's give him "a leg to stand on!"

Follow-up: The patient was treated with compression stockings, cephalexin 250 mg qid for two weeks (based on sensitivities), desoximetasone 0.25% cream and lymphatic massage. There was no evidence of tinea pedis. He has improved markedly.
Photos taken June 10, 2008





Reference:

Recurrent cellulitis after saphenous venectomy for coronary bypass surgery.
Baddour LM, Bisno AL.
Ann Intern Med. 1982 Oct;97(4):493-6.

We describe a previously unreported complication of coronary artery bypass
grafting, recurrent cellulitis. Five patients had 20 episodes of acute
cellulitis, each occurring in the lower extremity in which saphenous venectomy
had been done. The cases were striking because the patients presented with high
fever and considerable systemic toxicity. The appearance of the lesions, presence
in one case of obvious associated lymphangitis, and prompt response in three
instances to therapy with penicillin alone all suggest group A streptococcal
infection. In one case, a beta-hemolytic, bacitracin-susceptible Streptococcus
strain was isolated from the lesion. The pathogenesis of this syndrome remains
obscure but, based on our understanding of postsurgical erysipelas, this
cellulitis likely results from the interplay of several factors, including local
compromise of lymphatic drainage, direct bacterial invasion, and acquired
hypersensitivity to streptococcal exotoxins.

Umbilical Erosions

[ See end for final diagnosis]
Abstract: 71 yo woman with three week history of genital, anal and umbilical erosions.
HPI: This healthy 71 yo woman had vaginal prutitus for a few weeks. She saw her gynecologist who prescribed an estrogen cream. It got worse. She was then given clobetasol oint. It did not improve. She tried acyclovir ointment -- not much change. I saw her at this point. I recommended continuing clobetasol ointment, but after a few days getting worse. No new meds. Takes occasional acetoaminophen and diphenylhydramine.
O/E: Periumbilical erosive dermatitis. No frank vesicles. There was only faint erythema of the vulva and anal areas and very slight erosion left groin.



Lab: CBC normal, Chemistries normal. KOH from umbilicus negative. Bacterial culture taken.
Pathology: Biopsies for H&E and perilesional for DIF done Feb. 8, 2008
Diagnosis: I am considering the following:
A vesiculobullous disorder
Fixed drug eruption (but have no likely candidates)
Contact dermatitis unlikely.
HSV a long shot.
Periumbilical cellulitis? B-Strep perianal cellulitis can look similar
What have I missed?
Questions: What are your thoughts? Biopsy and culture should be ready in three days.

The bacterial skin culture grew out Group A Beta Strep. The pathology was consistent with cellulitis. No evidence of an acantholytic process. It is likely that this began with a perianal/vaginal streptococcal cellulitis and spread to the umbilicus. Periumbilical streptococcal cellulitis has not been reported in adults. The patient was started on Pen VK 250 mg qid and mupirocin ointment. Fout days later she was almost completely clear. Unfortunately, the fluorescent correction was not on when picture was taken.