D.R.E.S.S. Syndrome  (Drug Reaction with Eosinophilia and Systemic Symptoms)

Condition is a very rare Type IV(b) adverse hypersensitivity reaction  to certain medications, with gradual onset,  on average about 32 days after a new medication. Can occur earlier or later.

Most common medications are anticonvulsants (phenytoin, carbamazepine, phenobarbital, levetiracetam, lamotrigine.  NSAIDS, B-Lactam antibiotics. Vancomycin, Allopurinol, TMP/SMZ.

INCIDENCE: About 1:10,000 of adverse cutaneous drug reactions, with a high mortality rate, if undiagnosed in the range of (3.8%-10%)

The rash is generalized morbilliform, but can be limited to the face, causing facial edema/erythema, mimicking erysipelas/angioedema.

CLINICAL FINDINGS:  Besides the rash, are fever, chills, malaise, pruritus and generalized lymphadenopathy, involving the cervical, axillary and inguinal lymph nodes. Can appear minimally ill or toxic, depending on stage of presentation.

Can cause multi-organ involvement, with liver being the most common target and as well as the kidneys.

Most common cause of death is due to hepatic necrosis with fulminant hepatitis and renal failure.

LABORATORY DATA: Eosinophilia > 6000x10(3)/uL. Thrombocytopenia/Pancytopenia. Leukocytosis Coagulopathy, elevated liver enzymes (ALT x 2 normal). Elevated BUN, proteinuria.

Above laboratory findings are indicators of poor prognosis!

DIAGNOSIS: High clinical suspicion. Should always be on the differential diagnosis of any erythematous rash, in patient on above medications.

DIFFERENTIAL DIAGNOSIS:  Steven-Johnson syndrome. Toxic epidermal necrolysis. Kawasaki disease, Hypereosinophilic syndrome. 

TREATMENT: Discontinue potential causative agent(s). Systemic corticosteroids (Prednisolone IV with gradual tapering. Cyclosporine IV, if no response or steroid contraindicated. Supportive care. Vigilant monitoring for potential multi-organ involvement.

 RS3PE SYNDROME (Remitting Seronegative Symmetrical Synovitis with Pitting Edema).

Clinical syndrome is a rare entity, often mimicking an infectious process.

Patients will present with unilateral or often bilateral abrupt onset of  painful, erythematous swelling 

of upper or lower extremities, associated with palpable pitting edema.

Usually afebrile, but mild temperature elevation can occur (secondary to the inflammatory process).

Generally, recurrent with increasing episodes of flare ups.  Patient often mistakenly treated with

antibiotics, with no significant improvement. Condition, generally abates spontaneously without

any therapy and  next flare up is treated with antibiotics, again. 

Thus, RS3PE can be undiagnosed for many months.

Epidemiology:  More common in patients greater then age 60, male>female, rural setting, rapid onset.

Frequently misdiagnosed as cellulitis, erythrodermatosis, RSD (reflex sympathetic dystrophy)

About 50% likelihood of undiagnosed underlying malignancy (prostate/breast/lung carcinoma).

Laboratory findings:  Elevated inflammatory markers (CRP/Sedimentation rate).

Mild leukocytosis.  Negative RF and Anti-CCP-Ab. 

Treatment: High index of clinical suspicion. 

Hint: Onset of abrupt bilateral cellulitis is extremely unlikely.

Low dose prednisone 10-20 daily, provides rapid improvement.

Remain vigilant for underlying undiagnosed malignancies!

SARS-CoV-2    CLINICAL CLUES...basic findings

Covid-19 related symptoms vary from quite atypical (anecdotal)  to "classic" clinical presentations in the emergency department setting, requiring a high degree of suspicion.

The following are a short list of symptoms--presentations to consider:

Classic presentations are: 1. Dyspnea with hypoxia (pulse oximetry < 92% on room air)

                                             2. Tachypnea-tachycardia

                                             3. Fever, chills, night sweats

                                             4. Cough

                                             5. Myalgias

Atypical presentations:     1. Profound malaise-physical exhaustion

                                             2. Lack of appetite

                                             3. Insomnia

                                             4. Dysgeusia (altered-impaired sense of taste)

                                             5. Anosmia-hyposmia  (altered sense of smell)

                                             6. Nausea-diarrhea

                                             7. Delirium

                                             8. Embolic-thrombotic events in younger pationts

                                             9. Headache-vertigo

Laboratory data:               1. CBC with differential--lymphopenia

                                             2. Elevated ALT/AST

                                             3. Elevated LDH

                                             4. Elevated CRP

                                             5. Elevated serum Ferritin

                                             6. ABG--hypoxemia

Chest x-ray:                        1. Bilateral basilar infiltares to ARDS finding

CT scan chest:                    1, Ground glass appearance

              

"INTERNATIONAL" Infectious diseases....

In clinical practice and frequently on board exams, it is important to consider infectious diseases
that occur in certain "international", geographic regions, as these subtle hints will help with diagnosis
and in correctly answering the question. Examples:



1.     Patient from Egypt/Northern Africa develops hematuria. What organism is the likely cause?


2.     Patient from Mexico (Central/South America) develops a seizure.
        What is the likely cause?


3.    Patient from Scandinavia, develops B12 deficiency.
       Likely etiology?


4.    Patient returning from a vacation in the Caribbean, develops sudden headache,
       retroorbital pain, myalgias, arthralgias and high fever.
       What is the causative factor?



5.    Patient returning from a wild boar hunting trip develops gradual onset of
       myalgias, ,periorbital edema, persistent fever,malaise and photophobia.
       What organism is responsible?


========================================================================

Answers:

 1.  The diagnosis is SCHISTOSOMIASIS, specifically, S. Haematobium, that is prevalent
      in the fresh waters of African continent, Middle East and India.
      It is a Trematode (Fluke), a flat worms that infect mainly the GU tract, causing bladder
      ulceration, hematuria, that can frequently result in anemia and squamous  cell carcinoma
      of the bladder. Infertility is a frequent consequence.


2.   Diagnosis is NEUROCYSTICERCOSIS. It is a pork tapeworm, Taenia Solium.
      It can only occur from consuming undercooked pork. When the agent invades the
      CNS, the first symptoms is usually a seizure as usually it is asymptomatic.
      However, can have vague headaches, focal neurologic deficits and hydrocephalus.


3.   Diagnosis is DIPHYLLOBOTHRIASIS. The organism is a freshwater fish tapeworm.
      It is prevalent in Northern, cooler climates, worldwide, and especially common in
      Finland, Norway, Sweden.   Consumption of raw fish causes, the D. Latum to invade
      the small intestine, especially the terminal ileum, causing B12 deficiency, from
      continued feeding.



4.    Diagnosis: Acute DENGUE FEVER.  It is a disease transmitted by mosquitos, especially
       common in tropical regions. Caribbean, Puerto Rico, etc.  Caused by a flavivirus.



5.    Diagnosis:  Acute TRICHINOSIS.    This is another infectious disease, common in
       countries that consume pork, wild boar or even bear meat. The classic nematode is
       T. Spiralis. 



Will soon continue.
      

WHAT YOU EAT.......IS WHAT YOU GET.

When patients present to the emergency department with GI symptoms, vague muscle aches/pains and malaise, with or without fever, it is good to consider the FOOD(S) they have consumed, as the
cause of their symptoms.

Often, we "diagnose" them with "viral gastroenteritis", etc. but the medical literature is replete with
conditions associated with a non-viral agents.  The boards also favor these topics!

These following list of associations is partial but may help with your next diagnosis!


CONSUMPTION:                                                   AGENT/ILLNESS:



  1.  RAW MILK ...................................................Campylobacter Jejuni


  2.  SALMON/SQUID.........................................  Anisakiasis


  3.  PORK (undercooked)......................................Trichinosis
                                                                                Neurocysticercosis


  4.  TUNA/MACKAREL/MAHI-MAHI...............Scombroid



  5.  RAW EGGS/CHICKEN..................................Salmonella



  6.  GROUPER/SNAPPER....................................Ciguatera



  7.  RAW OYSTER................................................Vibrio Vulnificus



  8.  BEEF (undercooked)........................................E.Coli (O:157:H7)



  9,  SHELLFISH.....................................................Vibrio Parahemolyticus



10.  WATER (unsanitary)........................................Giardia Lamblia



11.  RASPERRIES...................................................Cyclospora



12. FRIED RICE......................................................Bacillus Cereus




Thanks for viewing. We'll continue soon.


 
                                                                         

UNCOMMON CAUSES OF CHEST PAIN....

In the daily practice of emergency medicine, we all come across the common causes of chest pain, such as Angina, Acute MI, pericarditis, Pleurisy, PE and an occasional Thoracic Dissection.

But often, even after extensive work-up, the diagnosis remains elusive...thus, making the diagnosis of ATYPICAL CHEST PAIN or UNDETERMINED CHEST PAIN common.


The list below is partial, but including them in your differentials, will certainly help close the gap!


UNCOMMON CAUSES OF CHEST PAIN:


   1. DRESSLER'S SYNDROME
      
       CLUES: Recent myocardial infarction. Normal cardiac enzymes. Fever. Elevated ESR.
                      Benign of pericarditis like findings on EKG.

 
   2. ACUTE PNEUMOMEDIASTINUM

       CLUES: Main symptom is severe chest pain, due to rupture of alveolar blebs with air
                      escaping into the mediastinum. Can palpate subcutaneous crepitations,
                      especially at the suprasternal notch/anterior neck. No dyspnea as opposed to
                      an acute pneumothorax.


   3. BOERHAAVE'S SYNDROME

       CLUES: Repeated emesis with sudden onset of intense chest pain, causing the tear in
                      the esophageal wall. CXR will show pneumonia-like findings. An increases
                      serum amylase and WBC is also key.  Condition can be especially common
                      in post head/neck radiation patient, esophageal cancer patient or in  patients
                      with previous damage of esophagus due to corrosive ingestion.


  4. ACUTE VARICELLA ZOSTER

      CLUES: Generally elderly patient presenting to ED with sharp stabbing pain, without
                     an obvious cause, involving the specific dermatome.  Can be challenging to
                     diagnose, as pain always occurs 2-3 days before the rash.


  5. CORKSCREW/NUTRCRACKER ESOPHAGUS

      CLUES: Due to an esophageal motility disorder, causing severe intense episodic chest
                     pains, especially after consuming carbonated beverages. The esophagus is
                     spasmodic and hypercontracting. Difficult to diagnose, as patients repeatedly
                     present to ED's with chest pain. Diagnosis: Barium swallow and best confirmation
                     study is Manometric Study of Esophagus.


 6. ACUTE CHEST SYNDROME

     CLUES: This is a complication of Sickle Cell Anemia affecting a subset of patients.
                    Carries a high mortality, if not treated early. Pathophysiology involves occlusion
                    of pulmonary microvasculature by deformed (sickled) RBC.'s causing infarction.


 7. KAWASAKI DISEASE

     CLUES: It is a form of vasculitis involving small-medium coronary arteries, causing blood
                    vessel aneurysms, that lead rupture, causing myocardial infarction.
                   This is the most common acquired coronary vascular disorder in children
                   Death can be sudden an unpredictable.





Thanks for your time. Will continue soon.
                  

COMMON TICK BORNE ILLNESSES....Differential diagnosis.

Tick borne illnesses are commonly seen in emergency departments.
As we all know, many times the patient has no awareness of being bitten by a tick and can present
with multiple vague complaints, which we'll present on the next posting.

However, most importantly it is always good practice to keep a list of alternate diagnosis
besides LYME'S, when considering this illness in the first place. Most  have very similar presentations and ALL have one thing in common....TICKS.


ALTERNATES TO CONSIDER:



1. BABESIOSIS (due to Babesia Microti).

2. ROCKY MOUNTAIN SPOTTED FEVER (due to Rickettsia Ricketti).

3. Q FEVER (due to Coxiella Burnetti).

4. TULAREMIA (due to Francisella Tularensis)

5. EHRLICHIOSIS  2 TYPES:
              
            * HUMAN MONOCYTOTROPIC EHRLICHIOSIS (HME)
               ( due to Ehrlichia chaffeensis)

            * HUMAN GRANULOCYTOTROPIC ANAPLASMOSIS (HGA)
               ( due to Anaplasma phagocytophilum)