RESUMO
Scleredema is a rare sclerotic skin disorder that typically develops in conjunction with diabetes, monoclonal gammopathy, or infection and commonly involves the neck, back, trunk, and arms. Scleredema can be categorized into three types according to its cause. The local examination of the lesion is characterized by non-pitting induration that follows a symmetrical spread with sparing of the hands and feet. We present a case of a 19-year-old female patient that presented to the outpatient clinic complaining of facial skin lesions that have been progressive for four years. The skin lesions were found to involve the neck, back, trunk and both arms sparing the hands and there was no systemic involvement of the disease. The patient is non-diabetic but reported frequent attacks of tonsillitis over the past months. Initially, punch biopsy showed no signs of scleredema; however, repeated biopsy at follow-up confirmed the presence of mucin deposits which are suggestive features of scleredema. Due to the similarities with various other diseases, the diagnosis requires clinical and histopathological exclusion which makes the diagnosis quite challenging. It almost always starts in the neck but can present initially in rare cases from the face spreading symmetrically. Close follow-up and continuous monitoring are necessary for systemic organ involvement.
RESUMO
Cutaneous lupus erythematosus (CLE) is a chronic skin disease that significantly impacts quality of life (QOL). This study tested a novel method to measure QOL in CLE using willingness-to-pay (WTP) stated preferences, and aimed to determine which of nine domains of life are most affected by CLE. Twenty-one participants with CLE ranked the domains in order of impact on CLE-related QOL, and then stated how many United States dollars they would be willing to pay for a hypothetical cure for each domain. Eighty-one percent of participants were female; mean age was 42.4 years. Photosensitivity was ranked highest by 71.4% of respondents. Participants were willing to pay the most for a hypothetical cure for CLE specific to photosensitivity (median = $200,000), the least for a cure specific to self-care (median = $0). Participants were willing to pay a median of $1,000,000 for an overall cure for CLE. Limitations include a small sample size for this pilot study and that willingness-to-pay may be influenced by individual perception of money and socioeconomic factors. This study successfully pilot-tested a WTP method and ranking task for health-related QOL. Photosensitivity was the domain of life most affected by CLE, which is a domain unique to our novel tool.
