Sir,
A 26-year-old man who was being regularly treated for pemphigus vulgaris since two
years presented with complaints of continuous high-grade fever since two days and
multiple fluid-filled lesions that appeared initially over the face, and later over
the chest, back, and limbs. Cutaneous examination showed multiple umbilicated vesicular
lesions (a few of them coalesced) over the face with severe facial edema [Figure 1],
chest [Figure 2], trunk, and all four limbs along with multiple punched out erosive
lesions and a few crusted lesions. Old crusted lesions of pemphigus were present over
the scalp with patchy hair loss. The total leucocyte count was 23,000/mm3, and liver
enzymes were marginally raised. Other investigations were unremarkable. Kaposi varicelliform
eruption (KVE) was suspected considering its acute onset, vesicular lesions, fever,
and facial edema in a known case of pemphigus on immunosuppressants since two years.
Other differentials were flare of underlying pemphigus (as many a times KVE is confused
with the underlying disease), and contact irritant dermatitis which was excluded by
history taking. Blood culture was negative and pus culture from one of the discharging
erosive lesions grew Staphylococcus aureus after 48 h, which was sensitive to the
antibiotics we had started. Tzanck smear showed acantholytic cells. Histopathologic
examination revealed a subcorneal blister [Figure 3] with hemorrhage, vessel damage,
and a mixed inflammatory infiltrate with multinucleated giant cells in the underlying
dermis [Figure 4] suggestive of herpes infection. Immunofluorescence was not done
due to lack of the facility.
Figure 1
Multiple fluid-filled vesicular lesions over face along with multiple punched out
erosive lesions and few crusted lesions with severe facial edema
Figure 2
Clusters of umbilicated vesiculopustules over chest
Figure 3
Subcorneal blister formation: H and E staining and ×40 magnification
Figure 4
Dermis showed hemorrhage with vessel damage along with mixedinflammatory infiltrate:
H and E staining and ×40 magnification
Considering high total counts and clinical condition of the patient, he Was given
intravenous acyclovir (10 mg/kg every 8 hourly), piperacillin-tazobactam (4.5 g 6
hourly) and linezolid (600 mg 12 hourly). and continued for 10 days. The patient continued
to receive oral prednisolone 10 mg once daily and azathioprine 50 mg twice daily for
pemphigus as before. Post treatment, his skin lesions healed with varicelliform scars
[Figure 5].
Figure 5
Post-treatment scarring
KVE is a potentially life-threatening viral infection caused by herpes simplex virus
(HSV) type 1, HSV-2, coxsackievirus A16, or vaccinia virus that occurs over a pre-existing
dermatosis. Multiple skin disorders have been associated with KVE, including pemphigus
foliaceus, Darier's disease, pemphigus vulgaris, pityriasis rubra pilaris, Hailey–Hailey
disease, irritant contact dermatitis, cutaneous T-cell lymphoma, seborrheic dermatitis,
psoriasis, Wiskott–Aldrich syndrome, congenital icthyosiform erythroderma, and Sezary
syndrome.[1
2
3]
KVE usually begins as a sudden eruption of painful, edematous clusters of umbilicated
vesiculopustules on the skin affected by a pre-existing dermatoses and may be accompanied
by a flu-like syndrome. The vesiculopustules progress to painful hemorrhagic, crusted,
punched-out erosions that coalesce to form denuded areas that are prone to secondary
bacterial colonization.[4] The upper body is the most common site of infection, with
a predilection for the head and neck.[1] In some cases, it may progress to fulminating,
life-threatening infection and can have severe sequelae, including herpes keratitis,
disseminated infection with visceral involvement, and death.[1
4] A delay in diagnosis often occurs because the eruption is confused with the underlying
disease. HSV is an ever-present hazard to patients having skin disease with a compromised
barrier function. The virus can remain viable in the environment over the skin surface
for 2 h, on door handles and taps for 2–6 h, over gauze pieces and swabs for 72 h,
and over hospital dust for about 2 weeks.[5] The most clearly delineated risk factor
for KVE is disruption of the epidermal barrier.[1
4] Hence it is necessary to create awareness about this uncommon dermatologic entity
and to stress on the importance of bed-spacing and barrier nursing so as to prevent
secondary bacterial infection. In a retrospective review of 100 KVE patients, Wollenberg
et al. found that a high serum IgE level and early onset of atopic dermatitis were
both risk factors.[3] Defective cytokine secretion and decreased cell-mediated immunity
in skin affected by atopic dermatitis and other diseases also appear to play a role
in the pathogenesis of KVE. Early use of both antiviral drugs and antibiotics is extremely
important; their use should not be delayed pending laboratory tests. When a bacterial
infection is not present, patients should be given a topical antibiotic cream.[6]
Patients with recurrent HSV infections and chronic skin disease predisposing to KVE
should be offered prophylaxis. KVE can be life threatening. This case illustrates
the need for both timely treatment and the use of appropriate laboratory tests to
confirm the diagnosis. Physicians should recognize the early presentation. Effective
treatment is available in the form of antiviral drugs and should not be delayed.