Ecthyma gangrenosum in a case of acute lymphoblastic lymphoma

Description

A 14 year-old-girl, a recently diagnosed case of pre-B acute lymphoblastic leukaemia (on modified BFM-90 protocol, week 2), presented with septic shock (heart rate, 124/min; blood pressure, 70/40 mm Hg) and swelling on the left side of face with necrotic ulcer, local induration and tenderness (figure 1A) of 2 days duration suggestive of ecthyma gangrenosum. Cultures from skin scraping, biopsy and peripheral blood revealed Pseudomonas aeruginosa. Evaluation for mucormycosis (fungal culture and histology of skin/nasal scrapings) was negative. She was promptly treated with empirical antibiotics and antifungals, later tailored per sensitivity (intravenous imipenem, intravenous amikacin). Her situation initially worsened during the first 48 h (figure 1B) but subsequently recovered in 2 weeks (figure 1C, D).

Figure 1.

(A) Ecthyma gangrenosum with swelling of the left side of the face with two ulcers of 5×5 mm along the left border of the nose and another measuring 1×2 mm below the left eye, with necrosis, erythematous margins on day 1. (B) Progression of the lesions by day 3 as the swelling progressed over the neck and right side of the face. (C) Improvement of patient's lesions after the antibiotic therapy on day 6. (D) Patient's current status at the end of sensitivity guided antibiotic therapy on day 16 of admission.

Ecthyma gangrenosum is a rapidly progressive infection of axilla, groin, buttocks or groin; however, involvement of the face is rare.¹ It is often seen in immunocompromised individuals (leukaemia, lymphoma, postchemotherapy, severe burns, severe malnutrition, etc) rarely affecting immunocompetent individuals.² It is characterised by initial erythematous nodule or haemorrhagic bullae which rapidly progresses to form necrotic ulcer which can have a characteristic eschar in the centre surrounded by erythema as seen in our patient. It is most commonly caused by P. aeruginosa, however, it can also be caused by other bacteria (Aeromonas, Escherichia coli, Haemophilus influenzae) as well fungi (Candida, Fusarium, Zygomycetes).³ Prompt diagnosis and management with empirical antimicrobials (targeting Pseudomonas, eg, cephalosporins, carbapenems, fluoroquinolones), broad-spectrum antifungal (like amphotericin) and surgical debridement where feasible are keys to success. Once septicaemia or organ failure ensues, the mortality rises exponentially.⁴

Learning points.

• Ecthyma gangrenosum is a life-threatening infectious disorder.

• Prompt diagnosis and differentiation from mucormycosis can have implications in therapy and long-term prognosis.