Periorbital Varicella Gangrenosa.

Sir,

Varicella gangrenosa is a rare and potentially fatal complication of varicella infection. It represents a type of necrotizing fasciitis caused by streptococcal infection complicating varicella infection. We report a case of periorbital varicella gangrenosa in a previously healthy child. Periorbital involvement due to varicella gangrenosa in a child has not been earlier reported in the literature.

A 5-year-old healthy male child presented to us with a peri-ocular gangrenous lesion around both eyes and the child was irritable and crying constantly. He gave a history of an attack of varicella 10 days back and was under treatment of a pediatrician, who had prescribed some antibiotics, paracetamol syrup and soothing agent. The patient was assured of spontaneous recovery. But unfortunately after 6 days of varicella onset parents noticed a tender violaceous plaque under the right eye, to be followed by a similar lesion on the other eye next day morning. By evening, the involved area had progressed rapidly to attain a size of 5 cm × 4 cm. Subsequently, central ulceration developed on both the sites for which he was prescribed some antibiotic injections and anti-inflammatory drugs in a local hospital. But as it progressed further without any signs of improvement he was referred to our center.

On examination, he was found to have a swollen and edematous face due to which he was unable to open his eyes. The involved area in the center was covered with a deeply adherent black eschar extending up to upper half of both cheeks. There was a deep groove of dimensions 1 cm × 1 cm between eschar margin and the uninvolved tissue [Figure 1]. The margins were unhealthy and covered with slough. He had multiple marks of healed varicella lesions on the trunk [Figure 2]. Remaining systemic examination was normal.

Figure 1

A deeply adherent black eschar surrounding both the eyes extending upto middle of the cheeks (more in right side). A deep groove seen between the eschar and the normal skin

Figure 2

Multiple hyper pigmented healed varicella lesions seen on chest

The child was of average body built with normal milestones of development. An ophthalmological evaluation was sought, but examination could not be done because eyelids could not be retracted. An empirical therapy of antibiotics drops and artificial tear drops were advised.

The patient was admitted and was empirically put under piperacillin and tazobactum, with daily surgical dressing. All routine investigations like complete blood count, HIV screening, blood culture and pus culture from ulcer base were done. He had hemoglobin level of 9 gm%, white cell count of 9000/cu.mm, platelet count of 2.3 lakhs/cu.mm and his erythrocyte sedimentation rate (ESR) was 22 mm in first hour. Pus culture was positive for Group A –beta hemolytic Streptococcus. From the above clinical findings, history and supportive culture report, a diagnosis of varicella gangrenosa was made.

After clinical stabilization, the patient was referred to plastic Surgery department for further management and intervention.

The credit of original description of varicella gangrenosa goes to Stokes,[1] who first described this condition in 1807. Estimated frequency is only 0.05% to 0.16% of cases.[2] This is a type of necrotizing fasciitis, caused by streptococcal infection complicating varicella infection even in recovery periods. Diagnosis is mainly based on clinical presentation and isolation of the organism. This is a very fatal condition with high mortality and morbidity. The common complications of varicella in children include secondary bacterial infection due to staphylococci and streptococci like impetigo, cellulitis and erysipelas. Serious complications like septicemia, osteomyelitis, pneumonia, encephalitis, and occasionally myelitis and polyradiculitis have been reported. Varicella gangrenosa though very rare has been reported by various authors in the past.[123] Varicella gangrenosa represents one of the many forms of necrotizing fasciitis consequent to subcutaneous streptococcal infection, that can rarely be seen in clinical practice. In the reported cases varicella gangrenosa is usually seen affecting the limbs.[4] Oral and intravenous Acyclovir if given early in the course of varicella infection, and early recognition of complications (even trivial) may reduce the incidence to a great extent.[5] Secondly, early institution of proper antibiotic and surgical debridement is mandated in all cases. This approach may help to reduce the extreme morbidity, mortality and disfigurement incurred by varicella gangrenosa. There have been reports of periocular varicella gangrenosa in adults requiring orbital exenteration.[6] After recovery disfigurement can be managed with prosthesis for aesthetic consideration.

This case teaches us not to be compliant while dealing with patients with varicella and careful follow up and examination till complete recovery (may be upto 3 weeks) is needed to prevent any fatalities. Prompt diagnosis and robust therapy are needed to salvage the patient. This case is reported for its rarity.

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