A 38-year-old Haitian woman was admitted to the hospital with a five-day history of evanescent salmon-colored maculopapular rash, generalized fatigue, and joint stiffness worsening. Associated symptoms included high spiking fevers, nausea, vomiting, and decreased appetite. She denied weight loss, infectious contacts, or recent travel.
Two weeks earlier, she had visited our emergency department (ED) twice. On the first visit, she had a similar salmon-colored rash on her neck, legs, and forearm and was given diphenhydramine. Several days later, she visited the ED because of generalized fatigue and joint stiffness, mainly involving both knees, elbows, and wrists. She was again discharged receiving acetaminophen this time. The patient's joint stiffness, generalized fatigue, and rash persisted, however. Additionally, she developed very high fevers, which now mandated her admission to the hospital.
Her past history was otherwise unremarkable. However, she had also been having heavy menstrual periods, averaging 7 days per cycle for the past several years. She described a craving for cornstarch and clay since her last pregnancy 5 years ago. She denied blood in her stool and had never been tested for parasites while in Haiti. There was no family history of anemia or bleeding disorders.
Her physical examination revealed posterior cervical lymphadenopathy and a maculopapular rash involving the neck. Furthermore, warmth and tenderness of the wrist, elbows, and ankle joints were appreciated. The stool was negative for occult blood.
Pertinent laboratory values on admission were a low hemoglobin (Hgb) (10.3
g/dL), low MCV (67
fl), normal platelet count (272
k/uL), mildly elevated white blood cell count (10.3
k/uL), high RDW (18.3%), and low absolute reticulocyte count (14.2
k/uL). C-reactive protein (139
mg/L) and erythrocyte sedimentation rate (79
mm/hr) were markedly elevated. A comprehensive metabolic panel was normal except for marginally elevated serum aspartate transaminase (72
u/L). Antinuclear antibody (ANA) and rheumatoid factor (RF) were negative. An extensive workup for rheumatologic and infectious processes was done and found to be negative. The serum ferritin level was found to be 26,387μ
g/L (normal 13–150μ
g/L). Serum iron was 49μ
g/dL, total iron binding capacity 215μ
g/dL, and iron saturation 23%.
The patient's high grade fevers with persistent rash led to a diagnosis of adult onset Still's disease by Yamaguchi's criteria [5
], and her marked hyperferritinemia was compatible with the diagnosis. Subsequently, she was given intravenous methylprednisolone with good response. As her fever subsided, the serum ferritin level initially declined steeply within 6 days and then steadily decreased (). Her anemia at that time was attributed to her inflammation. She was discharged with tapering doses of oral prednisone.
At her scheduled visit to a hematologist six months later, there was complete resolution of her inflammatory symptoms. Her laboratory data now showed a serum ferritin of 6.5
ng/mL (). Her Hgb was 9.2
g/dL, MCV 70
fl, serum iron 14
ug/dL, total iron binding capacity 306
ug/dL, and iron saturation 5%. A hemoglobin electrophoresis was normal (Hgb A 97.5%, Hgb A2 2.5%) excluding β
-thalassemia. These laboratory values now indicated obvious iron deficiency anemia, for which she was subsequently treated with ferrous sulfate 325
mg three times a day. When seen 4 months later after starting iron therapy, her blood count and iron studies had returned to normal and her pica resolved ().