Contributed by Eumenia Castro, MD, PhD and Rajendra Singh, MD
The patient is a 53-year old male who presented to the emergency room with a five day history of worsening rash, pain and swelling in his right hand that had progressed to include facial and left hand involvement.
The patient related that 5 days ago he had been working outside digging a horseshoe pit. He came inside shortly afterwards and noticed lesions on the base of his right index finger and right thumb. Shortly afterwards, his right index finger began to swell and become red and painful.
By the next day, his entire right hand was swollen, red and painful and he had begun to develop facial swelling and numbness. When asked about associated symptoms, the patient endorsed a two day history of acute onset dysphasia beginning shortly after a possible spider bite, chills and subjective fever. Patient specifically denied any associated shortness of breath, chest pain, nausea, vomiting, diarrhea or constipation.
The patient was seen at an outside institution where he was diagnosed with cellulitis and started on sulfamethoxazole and trimethoprim. Patient stated that since beginning the antibiotics his symptoms worsened. He developed swelling and redness in his left hand and noticed two pustules on the dorsal surface of the left hand. His face remained numb and swollen and he noticed a new rash on both cheeks that crossed the bridge of his nose.
The patient presented to the emergency room and his physical examination revealed temperature 35.9 degrees centigrade, large flaccid bullae and pustules on dorsal surface and lateral finger surfaces of both left and right hands and serosanguinous discharge from lesions of right hand (Figure 1). No erythema, papules or plaques on left or right forearm were observed. Black sharpie markings delineating areas of previous erythema involvement (Figure 2) of the forearm from the hand to the elbow bilaterally were identified.
Pertinent laboratory data included a normal white blood cell count of 9.1X10E+09/L, an elevated erythrocyte sedimentation rate of 46 mm/h and C reactive protein of 4.746 mg/dL. An X-Ray revealed diffuse soft tissue swelling of the right hand. No foreign body or fracture was identified. Wound cultures were negative for microorganisms. A biopsy of the wound was performed. The clinical differential diagnosis included bullous Sweet Syndrome versus other neutrophilic dermatosis.
Examination of multiple sections reveals parakeratosis, serum crust, epidermal hyperplasia (Figure 3, H &E X 2), and dermal edema (Figure 4, H&EX4) with extravasated erythrocytes (Figure 5, HEX4) and a dense interstitial infiltrate composed of neutrophils and few eosinophils (Figures 6 and 7, H& E x20). Neither fungal microorganisms nor basement membrane changes are seen with interpretation of PAS histochemical stain (Figure 8, PAS x20). Gram, GMS, and AFB stains are also negative for microorganisms.