RESUMO
Postinfantile giant cell hepatitis (PIGCH), also known as syncytial giant cell hepatitis, continues to be a poorly defined and rare disease presentation in the adult population. Although a common finding in neonates, there is limited literature on the disease process, causes, and treatment success of PIGCH in adults. A strong association between autoimmune disorders and PIGCH, considerably so in the case of autoimmune hepatitis, has been established. However, there have been limited to no reports of PIGCH secondary to rheumatoid arthritis. Our clinical case aims to bring forth a vignette of PIGCH to spotlight this ill-defined disease in the adult population and highlight some of the proposed causes, treatments, and laboratory markers.
RESUMO
Linear porokeratosis is a cutaneous disorder that typically presents in a unilateral linear formation. While the exact cause of linear porokeratosis is unknown, it is thought to be a downstream effect of disrupted cholesterol synthesis and mevalonate accumulation. Our patient is a 61-year-old male with an unusual case presentation of bilateral linear porokeratosis. He had failed numerous standard therapies. Pathologic examination of a skin biopsy was consistent with bilateral linear porokeratosis. Through a PubMed search, there have been limited reported cases of unilateral linear porokeratosis, but there have not been any reported cases of bilateral linear porokeratosis. There are currently limited therapies with satisfactory outcomes for variants of porokeratosis. While there are some studies on the topical application of cholesterol/lovastatin, limited studies have been performed on the linear form. Our study evaluates the efficacy of compounded topical cholesterol 2%/lovastatin 2% ointment on bilateral linear porokeratosis. The patient demonstrated a significant reduction of porokeratotic lesions on the treated arm compared to the untreated arm. Cholesterol/lovastatin is alternative therapy that can be considered in the treatment of linear porokeratosis and other porokeratosis variants.
RESUMO
This is a review of a patient encounter that underscores the common trend of insufficient inclusivity and lack of diversity regarding skin of color representation in teaching materials including textbooks in the medical education setup. A Black woman who was treated with carbamazepine for trigeminal neuralgia after a dental procedure presented with upper airway breathing difficulties and facial pain and swelling. After doubling her dose of carbamazepine as advised by her primary care physician, her symptoms continued to worsen, and she was treated in the emergency department for a presumed allergic reaction of unknown etiology. Two days later, her symptoms progressively worsened. She self-admitted to the emergency department, where she required cardiopulmonary resuscitation. Eventually, the formal diagnosis of carbamazepine-induced Stevens-Johnson syndrome (SJS) was made based on history, clinical presentation, and skin biopsy. The nature of the disease progression in this case prompted our investigation into the lack of representation of skin of color in current medical training resources regarding SJS. Our assessment demonstrates that there is a significant underrepresentation of SJS in skin of color in medical educational resources. Increased inclusivity of skin disorders in patients of color is crucial in training healthcare professionals to recognize life-threatening cutaneous disorders quickly and accurately in such patients.