Sweet's Syndrome By A Show Of Hands

Abstract

Recognize an atypical presentation of Sweet’s Syndrome. Skin biopsy. 57 year old Hispanic male with no PMH presents with new onset bilateral hand pain. Five days prior to admission, he noticed black discoloration in his fingernails. He then developed a constant pulsating pain, swelling and pruritic rash in his bilateral hands. He also complained of fevers, sweats and chills. On physical exam, patient had splinter hemorrhages on multiple fingers, marked diffuse tenderness to palpation over bilateral hands, diffuse edema of hands. Skin exam demonstrated maculopapular non-blanching erythematous rash on dorsal aspect of bilateral wrists, painful purpura on palms and fingertips bilaterally. Labs included WBC 12.9 (Neutrophil count 72.9), ESR 82, CRP 146.2. Biopsy of the hand lesion showed dense dermal neutrophilic infiltrate. The above patient had fever, painful/erythematous rash, and neutrophilic infiltration of the skin on biopsy, leukocytosis, and responded to systemic steroids. The patients’ clinical picture, labs, and pathology were consistent with Sweet’s Syndrome. However, this case was unusual as the patient's rash was limited to his hands. Sweet’s Syndrome clinically favors upper body and face but restriction of rash to the hands are rare. NDDH (Neutrophilic dermatosis od the dorsal hands) is a rare variant of Sweet’s Syndrome with localized lesions clinically restricted to the hands and less frequent systemic symptoms. Systemic symptoms such as fever, leukocytosis and increased ESR are observed in 1/3 of the cases. NDDH is a recent disease concept which is evolving as it has been described in only a few case studies.