A life-threatening, rapidly-spreading herpes

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Angie is a close friend of a family friend who was referred to me for recurrent eczematous lesions.  The patient on the day of consult had erosions (natuklap na balat) and excoriations (skin lesions that result from repeated urge to pick at one’s own skin) noted over the forehead and cheeks, extending over to the neck area and slightly over the chest.  She has had this kind of rash several times already and each time, she was given topical and oral steroids, moisturizing lotions, and antihistamines.  There was a time her eyes were badly swollen and her whole face bright red, hot, itchy with a burning sensation.  When she came to me though, it was not really bad so I suggested for her to undergo a test to help us find out what she really had.  But while waiting for the results of the tests, her problem deteriorated so fast that in two days, she looked as if she burnt her face and her eyelids were so engorged she could hardly open them.  Upon examination, noted was the presence of punch-out ulcers with local spread over the frontal, periorbital (around eyes), perioral (around lips) areas and cheeks, exuding a serous discharge.  Severe periorbital swelling and eye discharge were also noted.  Before this, she would always be told that she had eczema and then treated the same way.  The rash would disappear, but only to come back after several weeks.  It would usually start with clusters of tiny fluid-filled bumps that would suddenly give out a very light yellow discharge.  According to Angie, it has only been a year since her problem started to appear.  I finally got the result of the test, which says she is positive for herpes virus infection.

Angie’s case is a very difficult one because the rash seems to be an ordinary kind of eczema (eczema is a general term for a recurring inflammatory condition of the skin characterized by redness, itching, and oozing vesicular lesions which become scaly, crusted, or hardened; it encompasses many skin problems so it is not a definite diagnosis).  What Angie really has is not atopy but what we call Kaposi’s Varicelliform eruption (KVE) secondary to a viral infection, usually herpes simplex.  These infections, which are otherwise mild and localized, present in a florid and disseminated manner on the background of such dermatoses as skin asthma. Angie also has atopy (skin asthma), the most common allergic skin disease in the general population.  Atopic patients are prone to opportunistic infection because their skin barrier is compromised and their immune response may be suppressed.  Simply said, atopy is a chronic inflammatory skin disease complicated by recurrent bacterial and viral infections, that when left untreated, can lead to significant complications.  Infections include staphylococcus aureus (bacteria), eczema vaccinatum (not seen anymore these days from smallpox vaccination), eczema coxsackium (from Coxsackie virus) and KVE (from herpes).   In Angie’s case, KVE can be severe because it can progress to a disseminated infection and death if untreated.  The sudden onset of a painful vesicular rash in a patient with a preexisting skin condition should alert one to this possibility of KVE in patients with atopy.  This may be accompanied by fever, enlarged lymph nodes, and malaise (a condition of general bodily weakness or discomfort, often marking the onset of a disease).   Patients with more severe atopy, history of food allergies, history of pulmonary asthma are more susceptible to developing KVE.  Mutations in filaggrin (a protein that is vital for skin cells to mature properly into the tough, flat corneocytes that form the outermost protective layer of our skin) have clearly been associated with atopic dermatitis and recent studies have indicated that these mutations may confer increased susceptibility to KVE.  KVE is not confined to skin asthma. Patients with disrupted epidermal barrier such as contact dermatitis, seborrheic dermatitis, icthyosis vulgaris, pemphigus foliaceus, Darier disease, lupus, psoriasis, patients who recently underwent dermabrasion, burns, skin graft, and laser treatments etc.

Just like other herpes simplex virus infections, KVE can recur.  Oftentimes, the underlying viral cause is misdiagnosed as a secondary bacterial infection that can also occur at the same time, masking the real culprit.  This confusion can lead to a delay in treatment with an antiviral drug.  Misdiagnosis can lead to eye problems  (the likes of herpetic keratitis) and death.  In an immunocompromised patient, the mortality rate is reported to be as high as six to 10 percent and even 50 percent.   Systemic viremia (a medical condition where viruses enter the bloodstream and hence have access to the rest of the body) with multiple organ involvement is the major cause of morbidity and mortality. 

Clinicians should be aware that timely diagnosis and treatment of KVE are very important to avoid severe complications.  Giving corticosteroid therapy for exacerbation of what is thought to be just plain and simple atopy of the skin can result in progression of ocular herpes simplex infection or inevitable demise.  But just in case this happens, intravenous and topical antiviral should be instituted immediately to prevent blindness or death.

KVE is usually managed on an outpatient basis.  Admitting patients is reserved for those with high fever, poor oral intake and evidence of another infection, a topical ophthalmic antiviral may be added for prevention of keratitis in patient with eye involvement, superimposed impetigo (bacterial infection) should be treated immediately with antibacterial antibiotics.  Before the availability of an effective antiviral treatment, mortality associated  with KVE was as high as 75 percent.  Mortality is now low.  Although the average duration of illness is 15 days, cases lasting as long as six weeks have been reported.  Recurrent episodes tend to be milder and are not associated with systemic symptoms.  Vigilance on the part of dermatologists in diagnosing such a sinister and baffling disease is important.

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For questions or inquiries, call 09174976261, 09998834802 or 263-4094;  email  gc_beltran@yahoo.com.

 

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