ABSTRACT
Scleromyxedema is a rare and frequently disabling disease characterized by generalized waxy papules, skin induration, and cardinal histological features of dermal fibroblastic proliferation, thickened collagen, and mucin deposition. A monoclonal gammopathy is almost always present with rare progression to multiple myeloma. We describe the case of a 54-year-old man who presented with a rash in the setting of a new medication and histological features suggesting a granulomatous drug reaction. Despite discontinuation of the medication, the rash persisted and a second biopsy confirmed an interstitial granulomatous pattern. Serum protein electrophoresis identified the presence of a biclonal gammopathy leading to a diagnosis of granulomatous scleromyxedema. Review of the medical literature reveals only a handful of well-documented similar cases of this rare variant. It is important for pathologists and clinicians to be familiar with this condition to facilitate timely diagnosis and optimal clinical management of these patients.
Subject(s)
Scleromyxedema/diagnosis , Granuloma/diagnosis , Humans , Male , Middle Aged , Monoclonal Gammopathy of Undetermined Significance/diagnosisABSTRACT
BACKGROUND: Hidradenitis suppurativa (HS) is a chronic inflammatory disease involving intertriginous skin. Previous epidemiologic studies have been limited by small sample size. OBJECTIVE: We sought to describe the prevalence and comorbidities of HS in a large patient care database. METHODS: In this retrospective case-control study, we chart-validated all patients within a hospital database who received at least 1 billing code for HS between 1980 and 2013. Verified cases were matched with controls based on age, gender, and race. Prevalences of a priori selected comorbidities were compared between HS and control groups. RESULTS: A total of 2292 patients at Massachusetts General Hospital received at least 1 code for HS. A total of 1776 cases had a validated diagnosis of HS, yielding a prevalence of 0.08%. In unadjusted analysis, all comorbidities were diagnosed significantly more in HS compared with control including (in rank order of likelihood): smoking, arthropathies, dyslipidemia, polycystic ovarian syndrome, psychiatric disorders, obesity, drug dependence, hypertension, diabetes, thyroid disease, alcohol dependence, and lymphoma (all P < .01). LIMITATIONS: Control subjects were not validated for absence of HS and comorbidity validation was not performed for either group. CONCLUSIONS: Our results highlights the high comorbidity burden of patients with HS compared with matched control subjects.
Subject(s)
Databases, Factual/statistics & numerical data , Hidradenitis Suppurativa/epidemiology , Obesity/epidemiology , Smoking/epidemiology , Adult , Aged , Alcoholism/epidemiology , Case-Control Studies , Comorbidity , Diabetes Mellitus/epidemiology , Dyslipidemias/epidemiology , Female , Humans , Hypertension/epidemiology , Lymphoma/epidemiology , Male , Middle Aged , Prevalence , Retrospective Studies , Substance-Related Disorders/epidemiology , Thyroid Diseases/epidemiologySubject(s)
Ambulatory Care/organization & administration , Primary Health Care/organization & administration , Referral and Consultation/statistics & numerical data , Triage , Adult , Aged , Decision Making , Dermatology/methods , Female , Humans , Male , Middle Aged , Needs Assessment , Time Factors , United States , Waiting ListsABSTRACT
The International Psoriasis Council, a global nonprofit organization dedicated to advancing psoriasis research and treatment, led an initiative to better define the association of various cardiometabolic comorbidities with psoriasis. In November 2013, a workshop was held in Boston, Mass. By assembling a panel of global dermatology, immunology, and cardiovascular experts, the objective was to better define the current status of the science that explains the association of psoriasis with various cardiometabolic-related comorbidities. The International Psoriasis Council has played a historical role in associating psoriasis with various comorbidities by integrating multidisciplinary expertise to advance the scientific and clinical knowledge through publications and clinical trials. This report synthesizes the current understanding of psoriasis with various cardiometabolic risk factors by exploring the potential shared pathogenic mechanisms and genetic connectivity.