Systemic autoinflammatory diseases (SAIDs) are rare syndromes characterized by alterations in innate immunity that result in a variety of clinical manifestations that are usually associated with recurrent fevers.1 Thanks to advances in genetic sequencing over the past few years, monogenic causes for some of these autoinflammatory diseases, such as Yao syndrome, have been discovered.2 Previously…
The following report outlines a case of newly diagnosed adult-onset Still’s disease (AOSD) complicated by macrophage activation syndrome (MAS) in a previously healthy and active 32-year-old man who had emigrated from Africa to the U.S. Case A man with no prior medical history presented with acute-onset polyarthritis, fevers and fatigue that began one month previously….
Syphilis, an ancient disease caused by the spirochete Treponema pallidum, has been historically referred to as the great mimicker given its heterogenous presentation. Both systemic lupus erythematosus (SLE) and syphilis can have multi-systemic involveÂment. Both parvovirus B19 and syphilis have been reported to cause histologic features similar to those seen in lupus nephritis. We present…
On a highway traversed by cement trucks and Beetle-Bug auto-rickshaws we travel north from Bangalore, India, for a house call. It is 2007, and the city leaves us grudgingly. Between fields of loose chocolate soil and sprigs of beans poking skyward, the skeletons of homes and businesses rise; armies of workers lay brick from wooden…
A 60-year-old Black woman with a history of stage 3 chronic kidney disease, type 2 diabetes and hypertension presented with a 12-month history of asymmetric polyarthritis of the wrists, metacarpophalangeal (MCP), proximal interphalangeal (PIP), metatarsophalangeal (MTP) and knee joints. The review of systems was unremarkable. She denied oral ulcers, rashes, alopecia, or a history of…
The Case A 56-year-old white woman was evaluated for a one-year history of painless bumps on the dorsal aspect of the proximal interphalangeal (PIP) joints of both hands and suspected flexor tenosynovitis in her palms. On examination, small cystic nodules without erythema or tenderness were present on the dorsal aspect of several PIP joints (see…
Polyarteritis nodosa (PAN) is a necrotizing vasculitis, predominantly involving medium-sized arteries, that causes systemic disease, and, less commonly, cutaneous-limited disease. The population prevalence for PAN ranges from 2 to 33 per million.1-3 Estimates vary due to the increased recognition and classification of other forms of vasculitides over time and variation in the regional prevalence of…
As a dermatologist/internist with a career-long subspecialty interest in the cutaneous manifestations of the rheumatic diseases, I found the case of refractory acute cutaneous lupus by Samantha C. Shapiro, MD, in the June 2022 issue of The Rheumatologist intriguing in several ways, and I felt my perspectives on this case might provide additional educational value…
Scleromyxedema is a primary cutaneous mucinosis characterized by a diffuse and generalized papular skin eruption of mucinous deposits throughout the upper dermis. In addition to dermatologic manifestations, scleromyxedema may involve the cardiopulmonary, gastrointestinal, renal and nervous systems. Dermato-neuro syndrome (DNS) is a rare, severe neurologic complication of scleromyxedema.1,2 The pathogenesis of DNS is unknown, but…
Acute febrile neutrophilic dermatosis, or Sweet syndrome, is an inflammatory disease that classically presents with fever, leukocytosis and tender, erythematous plaques characterized by neutrophilic infiltrates on biopsy. Sweet syndrome has been reported in association with several autoimmune diseases, including inflammatory bowel disease, systemic lupus erythematous, rheumatoid arthritis and sarcoidosis.1 Here, we discuss a case of…