Dermato-pathologic clues to diagnosis of adult onset still disease: A case report

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory condition classically consists of high spikes of fever, morbilliform evanescent skin rash, arthritis, neutrophilic leukocytosis, and multiple organ involvement. However, atypical form of skin rashes has been described with few specific clinical and histopathological patterns that can help in making the diagnosis. A 25-year-old female presented with high spikes of fever, severe debilitating arthritis of peripheral joints, and dusky erythematous flagellate rashes over trunk and proximal limbs. Skin punch biopsy from the cutaneous lesion revealed dyskeratotic keratinocytes in upper epidermis and stratum corneum along with acute inflammatory infiltrate in the dermis: a finding that recently has been found to be constantly associated with this specific pattern of rash of AOSD. The presence of necrotic keratinocytes in upper epidermis and a dermal infiltrate of neutrophils along with characteristic clinical scenario may facilitate earlier diagnosis of AOSD.