A 66-year-old white woman presented for an evaluation of her skin. Informed consent was obtained from the patient for being included in the study. Additional informed consent was obtained from all patients for which identifying information is included in this article. The patient’s past medical history was significant for systemic lupus erythematosus and myasthenia gravis. Since 1980, the patient has been treated with hydroxychloroquine at a daily dose of 400 mg.\nThere was diffuse hyperpigmentation. The patient’s forehead, face, neck, v-area of the upper central chest, and upper back were black (Figs. , ), and her right upper chest was blue (Figs. , ). Hyperpigmentation was absent from her oral mucosa and nails.\nFive years earlier, the patient had visited a new clinician on a Friday afternoon. The patient’s clinician misinterpreted her black and blue dyschromia to be trauma-induced and had notified Adult Protective Services. The following Monday morning, a worker from the agency was at her home to investigate the family for elder abuse. Eventually the situation was resolved and the family was cleared of all suspicion of elder abuse.\nDuring her current cutaneous evaluation, a skin biopsy from the area of blue dyschromia on the patient’s right upper chest was performed. Microscopic examination showed both intracellular and extracellular yellow-to-brown granules scattered throughout the reticular dermis. Pigmented granules were seen between the collagen bundles with some accentuation around eccrine glands. The granules stained positively for both Fontana Masson and Perl’s stains.\nCorrelation of the patient’s medical history, clinical presentation, and pathology findings confirmed the diagnosis of hydroxychloroquine-induced hyperpigmentation.
A 45-year-old woman was admitted to our heart center with severe mitral stenosis and left atrial clot. The patient gave a history of shortness of breath on minimal exertion for one week and no cerebral symptoms, including headache, dizziness, transient ischemic attacks, or strokes. She was found to be fully conscious, alert, and oriented with stable vital signs. Blood pressure was 110/80 mmHg, and diastolic murmur was audible over the mitral area. Physical examination revealed intact cranial nerves, motor power, and sensation. There was no evidence of vasculitis such as Osler’s nodes, Jane way pad, palmer erythema, or cyanosis of fingers. Blood investigation showed an erythrocyte sedimentation rate (ESR) of 4 mm with no leukocytosis and a white blood cell (WBC) of 10.000 mm3. The electrolytes and kidney and liver function were within normal limits. Chest X-ray showed prominence of the left cardiac border due to left atrial enlargement. Electrocardiography was indicative of no change, but two–dimensional echocardiography revealed a large bulky non-mobile thrombosis in the left atrium with severe mitral stenosis. The mitral valve area was 0.6 cm2, and the other cardiac valves were normal. Pre- and postoperative laboratory measurements included negative antinuclear antibodies and rheumatoid factor, and elevation of C4 & C3 components of complement; in addition, HBS Antigen (especially in polyarteritis nodosa) was negative.\nThe patient underwent open heart surgery via the ascending aorta and bicaval cannulation. The aorta was cross-clamped, and the heart was arrested with cold cardioplegia by achieving systemic hypothermia at 30 °C on the cardiopulmonary bypass. Intraoperatively, the stenotic mitral valve was found to have a bulky clot attached to the valve and wall of the left atrium. The diseased mitral valve and the clot were excised, and the left atrial cavity was irrigated with copious amounts of normal saline and a valve was thereafter implanted. The left ventricle was then filled completely with saline and blood and fully deaired. The patient was weaned successfully off cardiopulmonary bypass with inotropic support and transferred to the intensive care unit. After 6 hours, blood pressure dropped and central venous pressure elevated and the extremity became cool and cyanotic. Inotropic support was commenced with dobutamine and adrenaline (2 and 1 mic/kg/min, respectively). On the third postoperative day, the inotropic drugs were tapered and blood pressure stabilized. By this point, however, the cyanosis had changed to gangrene and on the 12th postoperative day the acral part of the extremity was amputated and the specimen was sent for histopathological examination. In the histopathological examination, there was only non-specific vasculitis with thrombosis. The patient did not regain full consciousness on the following morning and was found to be quadriplegic with non-voluntary movements of the four limbs and face. The eyes could move only on the vertical plane, with both pupils having become small and fixed. After 72 hours, she managed to respond to questions only by blinking or moving her eyes vertically. Brain MRI revealed multiple infarction of the brainstem. Prolonged ventilatory support was maintained and eventually a tracheostomy was performed to facilitate tracheobronchial suctioning and weaning. Acute renal failure with anemia was managed and the kidneys recovered completely. However, the patient remained in this locked-in state for 4 weeks with no neurological improvement despite all ventilatory, nutritional, and nursing support. She eventually expired from hepatic failure and generalized sepsis.