Hydroxychloroquine (HCQ) is a commonly used medication for its immunosuppressive and dermatologic effects. It has known ocular side effects, which include retinopathy, corneal deposits, and choroidal thinning. Herein, we report the first known case of HCQ-induced hyperpigmentation of the sclera. A 75-year-old female presented after 10 months of gradual progression of painless blue-gray discoloration of the bilateral sclera, fingernails, and lower extremities secondary to oral HCQ therapy. Cessation of the drug led to a partial reversal of the hyperpigmentation at 5 months, further supporting HCQ as the causative agent. Hyperpigmentation reactions can be distressing to patients and lead to decreased medication adherence; given the widespread use of HCQ, it is important to increase awareness of this potential drug reaction.

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Nevus of Ota or congenital oculodermal melanosis (ODM) is characterized by brown or blue/gray asymptomatic brown or blue/gray flat lesions of the skin, mucosae, episcleral/sclera, and uvea, which are located near the trigeminal nerve\'s ophthalmic and mandibular branches. The main ophthalmic complications are glaucoma and predisposition to uveal melanoma. \"trichilemmal cyst\" is also known as \"wen\" \"pilar cyst\" or \" isthmus catagen cyst\". It occurs in the scalp and mimics sebaceous cysts clinically. The swelling appears smooth in outline and is filled with cytokeratin. An unusual case of a 32-year-old male with both trichilemmal cyst and nevus of Ota, a 27-year-old female, and a 47-year-old male with nevus of Ota is discussed here.

Primary adrenal insufficiency (PAI), also known as Addison\'s disease (AD), is a condition resulting from adrenal gland diseases that lead to glucocorticoid and/or mineralocorticoid deficiency, in addition to androgen deficiency in females. Here, we report a 40-year-old male indoor worker with an insignificant past medical history who presented to the dermatology clinic with a history of asymptomatic, slowly progressive skin hyperpigmentation for the past three years. It was associated with fatigue and weight loss. Skin examination revealed diffuse, non-scaly hyperpigmented patches on his face, dorsae of the hands, and palms. Early morning cortisol and adrenocorticotropic hormone (ACTH) serum levels were 1.00 µg/dl (5.0-19.4 µg/dl) and 2000 pg/mL (7.2-63.3 pg/mL), respectively. Based on the above clinical and laboratory findings, a diagnosis of AD was made. The patient was started on the following medications for 14 days: hydrocortisone 20 mg in divided doses (15 mg am/5 mg pm) and fludrocortisone 0.1 mg once daily (od). On the second visit, the patient\'s symptoms (both the cutaneous hyperpigmentation and fatigue) significantly improved, but he was complaining of edema in both upper and lower limbs, so the dose of fludrocortisone was reduced to 0.05 mg od.

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Perioperative stroke is a potentially devastating complication in patients undergoing noncardiac surgery. The most consistent risk factor associated with the condition is a history of a prior stroke. Cerebral oximetry is a simple, non-invasive, and continuous monitoring device that uses near-infrared spectroscopy (NIRS) to monitor cerebral oxygenation. However, like other monitoring devices, cerebral oximetry has certain limitations, and it must be interpreted cautiously and by taking into account all available clinical information related to the patient. We present a case of a 62-year-old Caucasian woman with a past medical history of a transient ischemic attack (TIA), who had been advised to undergo a right pneumectomy by video-assisted thoracoscopic surgery for treating chronic infection of bronchiectasis. Before administering any drug and while the patient was still alert, we monitored NIRS, and the values recorded were 15 on the left side and 26 on the right side. Despite being Caucasian, she had a darker brownish skin color due to chronic clofazimine use, which is known to cause skin pigmentation. Skin pigmentation is known to attenuate the transmission of near-infrared (NIR) light, potentially affecting the estimation of cerebral oxygen saturation. Thus, our patient suffered from clofazimine-induced skin pigmentation, which may have interfered with the NIR light transmission, which explains the extremely low values observed. Regional intracerebral oxygen saturation should be interpreted in the context of all available clinical information since NIRS transmission can be influenced by several factors and skin pigment has been found to independently influence regional intracerebral oxygen saturation. Apart from race or high serum bilirubin concentration, we should also consider other causes of skin pigmentation alterations, such as pharmacological therapy.

Whipple\'s disease (WD) is caused by Tropheryma whipplei, frequently found in lamina propria\'s macrophages in the small intestine. It is a rare and chronic systemic infection, and the principal clinical manifestations are diarrhea, weight loss, abdominal pain, and arthralgia. The diagnosis is difficult mainly because of its rarity and should be considered in patients with arthralgias, diarrhea, abdominal pain, and weight loss after more common conditions have been excluded. The laboratory diagnosis is established by a duodenal biopsy. The treatment involves 14 days of intravenous antibiotics with good penetration in the cerebrospinal fluid (i.e., ceftriaxone) and one-year treatment with oral co-trimoxazole. Early diagnosis and proper treatment are crucial because it improves the prognosis. We report the case of a 58-year-old female with skin hyperpigmentation, loss of appetite and weight (16% of body weight in three months), nausea, upper abdominal pain, and diarrhea. Esophagogastroduodenoscopy and colonoscopy were performed to obtain biopsy samples, which, together with laboratory tests and microbiological studies, led to a diagnosis of Whipple\'s disease.

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We report a case of an eight-year-old boy with mucopolysaccharidosis (MPS) II with atypical skin lesions of hyperpigmented streaks along Blaschko\'s lines. This case presented with mild symptoms of MPS such as hepatosplenomegaly, joint stiffness, and quite mild bone deformity, which was the reason for the delay in diagnosis until the age of seven years. However, he showed an intellectual disability that did not meet the diagnostic criteria for an attenuated form of MPS II. Iduronate 2-sulfatase activity was reduced. Clinical exome sequencing of DNA from peripheral blood revealed a novel pathogenic missense variant (NM_000202.8(IDS_v001):c.703C>A, p.(Pro235Thr)) in the IDS gene, which was confirmed in the mother with a heterozygous state. His brownish skin lesions differed from the Mongolian blue spots or \"pebbling\" of the skin that are observed in MPS II.

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