Cutaneous manifestations of cystic fibrosis.

Cystic fibrosis (CF) is caused by a mutation in the Cystic fibrosis transmembrane conductance regulator (CFTR) gene, and features recurrent sinus and pulmonary infections, steatorrhea, and malnutrition. CF is associated with diverse cutaneous manifestations, including transient reactive papulotranslucent acrokeratoderma of the palms, nutrient deficiency dermatoses, and vasculitis. Rarely these are presenting symptoms of CF, prior to pulmonary or gastrointestinal sequelae. Cutaneous drug eruptions are also highly common in patients with CF (PwCF) given frequent antibiotic exposure. Finally, CFTR modulating therapy, which has revolutionized CF management, is associated with cutaneous side effects ranging from acute urticaria to toxic epidermal necrolysis. Recognition of dermatologic clinical manifestations of CF is important to appropriately care for PwCF. Dermatologists may play a significant role in the diagnosis and management of CF and associated skin complications.

An Ode to Kodachrome: The Color Film That Transformed Dermatologic Medical Education.

Importance: From its debut in 1935 until its discontinuation in 2009, Eastman Kodak Company's Kodachrome color reversal film was a cornerstone for dermatologic teaching innovations that transformed 20th century medical education. This Special Communication examines Kodachrome's contributions to the field of dermatology, as well as its lessons for improving inclusive representation of patients of all skin tones in 21st century dermatologic curricula. Observations: Kodachrome's color quality, its slide transparency format, and its broad commercial availability democratized the creation, sharing, and teaching of visual information about skin disease in the 20th century. Kodachrome's usefulness as a complement to bedside teaching modernized medical school curricula, dermatologic conferences, and the American Board of Dermatology certifying examination, which inspired the Kodachrome-style of didactic that remains central to dermatologic training programs today. However, Kodachrome film was also the product of a prejudiced era when color film technology and photographic best practices were optimized for white skin. These biases are still evident in industry standards, photographic techniques, and the historically unjust representation of skin of color in educational resources. Conclusions and Relevance: Kodachrome film contributed substantially to shaping 20th-century medical education; however, its legacy is a reminder that diverse and inclusive image representation in dermatologic curricula is vital to counteracting implicit biases, correcting assumptions about disease epidemiology, and providing high-quality care for patients of all skin tones. Historical biases that have harmed representations of racial and ethnic minorities in dermatologic curricula are being addressed through improvements in digital photographic technologies, photographic best practices that serve a broader range of skin tones, inclusive skin color representation in contemporary educational resources, and skin-of-color specific curriculum for learners.

Clonal cutaneous and neurosyphilis: A pitfall in pseudolymphoma diagnosis.

Syphilis is a sexually transmitted infectious disease caused by the bacterium Treponema pallidum and can cause a wide variety of cutaneous manifestations, most commonly, a papulosquamous eruption of the trunk and extremities. Treatment with penicillin is curative. We report a case of a 69-year-old man who presented with recent onset of blurry vision and a nonpainful, nonpruritic eruption of pink-to-violaceous dermal nodules on his upper trunk and upper extremities. Biopsies of two separate locations revealed a dense superficial and deep perivascular atypical lymphocytic infiltrate with admixed plasma cells, histiocytes, and eosinophils. Some scattered cells expressed CD30, PD1, BCL-6, and ICOS. T-cell receptor (TCR)-rearrangement showed an identical TCR-gamma clone between both biopsy specimens. The patient was subsequently seen by ophthalmology and diagnosed with acute anterior uveitis. Rapid plasma reagin was reactive and cerebrospinal fluid studies showed findings consistent with a diagnosis of neurosyphilis. A T. pallidum immunostain of the skin biopsies was performed upon re-review, and was diffusely positive for spirochetes at the dermal-epidermal junction and within injured vessels. The patient was treated with penicillin G with near-resolution of his skin lesions. This case highlights the unusual ability of syphilis to mimic a T-cell lymphoma with matching clones across two different biopsy sites.

Watersport hands.

Aquagenic syringeal acrokeratoderma is a newly described condition of the palms and soles characterized by hypopigmented papules and plaques, elicited after submersion in water. Symptoms include a burning pain and a tightening sensation in the palms, as well as hyperhidrosis. Initially thought to be rare, its frequent citation in the literature points to a more common entity. It is more often found in young women and has been linked to a number of medications and illnesses, including nonsteroidal anti-inflammatory drugs and cystic fibrosis. It is typically self-limiting, but certain medications such as topical aluminum chloride or salicylic acid ointment have been found to be an effective treatment option. This case details a collegiate-level coxswain who presented to the university athletic training room with a typical presentation of aquagenic syringeal acrokeratoderma. For an aquatic athlete, aquagenic syringeal acrokeratoderma can be a distressing condition that can limit training and athletic participation. As such, the sports medicine physician should be knowledgeable about aquagenic syringeal acrokeratoderma to provide effective counseling and treatment options for the athlete.

Concentration-resistance relationships with Pseudomonas aeruginosa exposed to doripenem and ciprofloxacin in an in vitro model.

OBJECTIVES: To compare the abilities of doripenem and ciprofloxacin to restrict the enrichment of resistant Pseudomonas aeruginosa, multiple antibiotic dosing regimens were simulated in an in vitro model at comparable ratios of the 24 h AUC (AUC24) to the MIC. METHODS: Three clinical isolates of ciprofloxacin-resistant P. aeruginosa (MIC of doripenem 1 mg/L, MIC of ciprofloxacin 4 mg/L) were exposed to thrice-daily doripenem or twice-daily ciprofloxacin for 3 days at AUC24/MIC ratios from 50 to 170 h (doripenem) and from 55 to 180 h (ciprofloxacin). RESULTS: Doripenem- and ciprofloxacin-resistant mutants were enriched at antibiotic concentrations that fell into the mutant selection window for ≥ 45% and ≥ 60% of the dosing interval, respectively. The anti-mutant effects of doripenem and ciprofloxacin expressed by the area under the bacterial mutant concentration-time curve (AUBCM) depended on the AUC24/MIC ratio, and the AUBCM-log AUC24/MIC relationships were antibiotic specific. CONCLUSION: Based on AUC24/MIC relationships with AUBCM, a greater anti-mutant potential was predicted for doripenem compared with ciprofloxacin.

Comparative pharmacodynamics and antimutant potentials of doripenem and imipenem with ciprofloxacin-resistant Pseudomonas aeruginosa in an in vitro model.

To compare the antipseudomonal efficacy of doripenem and imipenem as well as their abilities to restrict the enrichment of resistant Pseudomonas aeruginosa, multiple-dosing regimens of each drug were simulated at comparable values of the cumulative percentages of a 24-h period that the drug concentration exceeds the MIC under steady-state pharmacokinetic conditions (T(>MIC)) and ratios of the 24-hour area under the curve (AUC(24)) to the MIC. Three clinical isolates of ciprofloxacin-resistant P. aeruginosa (MIC of doripenem, 1 µg/ml; MICs of imipenem, 1, 2, and 2 µg/ml) were exposed to thrice-daily doripenem or imipenem for 3 days at AUC(24)/MIC ratios of from 50 to 170 h (doripenem) and from 30 to 140 h (imipenem). The antimicrobial effects for susceptible and resistant subpopulations of bacteria were expressed by the areas between control growth and time-kill curves (I(E)s) and areas under the bacterial mutant concentration curves (AUBC(M)s), respectively. With each antibiotic, the I(E) and AUBC(M) versus log AUC(24)/MIC relationships were bacterial strain independent. At similar AUC(24)/MIC ratios, doripenem was slightly less efficient than imipenem against susceptible and resistant subpopulations of bacteria. However, doripenem appeared to be somewhat more efficient than imipenem at clinically achievable AUC(24)s related to the means of the MICs for the three studied strains and had higher antimutant potentials for two of the three strains.

Imiquimod cream 5% for the treatment of arsenic-induced cutaneous neoplasms.

Long-term exposure to arsenic has been linked to the development of numerous cutaneous neoplasms including arsenical keratoses, basal cell carcinomas (BCCs), and squamous cell carcinomas (SCCs). We report a patient with a remote history of psoriasis treated with arsenic who subsequently developed more than 40 nonmelanoma skin cancers as well as arsenical keratoses. This patient had a remarkable response to imiquimod cream 5% applied once daily to affected areas for 6 weeks with complete resolution of all cutaneous neoplasms and no evidence of recurrence in more than 3 years of clinical surveillance.

Cystic fibrosis presenting with dermatitis.

BACKGROUND: Patients with cystic fibrosis classically present with evidence of pulmonary disease, exocrine pancreatic insufficiency, and high sweat chloride concentrations. Dermatitis as an initial manifestation of the disease is uncommon and has been attributed to multiple nutritional deficiencies. OBSERVATION: We describe the case of a 3-month-old female infant with cystic fibrosis presenting with dermatitis in the setting of protein-energy malnutrition. A review of the laboratory study results in this case and others showed that a deficiency in zinc, essential fatty acids, and protein likely contributes to the development of the rash seen in cystic fibrosis. CONCLUSIONS: Given the frequent delay in diagnosis, as well as the increased morbidity and mortality associated with protein-energy malnutrition in these patients, it is important to consider cystic fibrosis as a possible diagnosis in any infant presenting with a rash and other signs of malnutrition. The relative contribution of specific nutritional deficiencies and the degree to which they influence and interact with each other in producing the dermatitis remain unclear, although they may all affect a common underlying metabolic pathway.

Metastatic cutaneous breast carcinoma: A case report and review of the literature.

Cutaneous metastatic carcinoma, also known as carcinoma erysipeloides, is an unusual clinical finding. In women, it is most frequently found in association with breast cancer rather than other visceral malignancies. Breast cancer patients can present with cutaneous manifestations of breast disease at the time of their initial diagnosis; however, cutaneous metastases more often present well after the initial diagnosis and treatment of the breast disease. Prompt recognition of skin metastases by the plastic surgeon, especially during the delayed setting of reconstructive surgery following mastectomy, allows for earlier intervention in treating the systemic spread of the disease. A clinical case of carcinoma erysipeloides presenting in this delayed setting is outlined in the present report, along with a review of the literature.

Successful outcome utilizing hypothermia after cardiac arrest in pregnancy: a case report.

BACKGROUND: To date, pregnancy has been considered a contraindication to the use of therapeutic hypothermia after cardiac arrest. CASE: We present the case of a 35-yr-old woman, 13 wks pregnant, who had a witnessed out-of-hospital ventricular fibrillation cardiac arrest. She was resuscitated by prehospital personnel yet remained comatose at arrival to the hospital. Therapeutic cooling (33 degrees C) was initiated for 24 hrs, and she was discharged home with mild neurologic deficit (Cerebral Performance Category 2) on hospital day 6. The infant was delivered via cesarean section at 39 wks' gestation. Apgar scores were 8 and 9, and neurodevelopmental testing was appropriate for age at birth and at 2 months. CONCLUSION: This is the first case of therapeutic hypothermia applied to postarrest care of a pregnant woman followed by a successful delivery. This therapy should be considered in pregnant patients with cardiac arrest.

Selection of linezolid-resistant Enterococcus faecium in an in vitro dynamic model: protective effect of doxycycline.

OBJECTIVES: To relate the enrichment of linezolid-resistant Enterococcus faecium with linezolid pharmacokinetics, the pharmacodynamics of linezolid and its ability to prevent the selection of resistant mutants were studied in an in vitro model that simulates antibiotic concentrations in and out of the mutant selection window (MSW), i.e. the concentration range from the MIC to the mutant prevention concentration (MPC). METHODS: A clinical isolate of E. faecium (MIC 1.8 mg/L and MPC 7 mg/L) at a starting inoculum of 8 log cfu/mL was exposed to twice-daily linezolid, alone and in combination with once-daily doxycycline (MIC 0.2 mg/L and MPC 3.4 mg/L), for 3 consecutive days in a hollow-fibre two-compartment model. RESULTS: The ratios of 24 h area under the curve (AUC24) to MIC of linezolid were estimated at 70, 100 and 230 h and those of doxycycline were estimated at 230 and 720 h. At the two lower AUC24/MIC ratios of linezolid given alone, E. faecium resistant to 2 x MIC-16 x MIC and 2 x MIC-8 x MIC of linezolid, respectively, were selectively enriched with a concomitant slight loss in susceptibility. Neither growth on linezolid-containing media nor changes in susceptibility occurred at the high AUC24/MIC ratio. A similar protective effect was observed with the minimal AUC24/MIC ratio of linezolid (70 h) combined with doxycycline at an AUC24/MIC of 230 h. CONCLUSIONS: This study suggests that selection of linezolid-resistant enterococci can be predicted from the MSW concept and can be prevented by linezolid given in combination with doxycycline, each at suboptimal AUC24/MIC ratios.

Immunoglobulin A pemphigus involving the perianal skin and oral mucosa: an unusual presentation.

Immunoglobulin A (IgA) pemphigus is a rare autoimmune blistering disease characterized by epidermal acantholysis and neutrophilic infiltrates, as well as intraepidermal IgA deposits. We report an unusual case of IgA pemphigus involving anal/ perianal skin and oral mucosa that demonstrated a successful response to dapsone treatment.

Alteration of marrow cell gene expression, protein production, and engraftment into lung by lung-derived microvesicles: a novel mechanism for phenotype modulation.

Numerous animal studies have demonstrated that adult marrow-derived cells can contribute to the cellular component of the lung. Lung injury is a major variable in this process; however, the mechanism remains unknown. We hypothesize that injured lung is capable of inducing epigenetic modifications of marrow cells, influencing them to assume phenotypic characteristics of lung cells. We report that under certain conditions, radiation-injured lung induced expression of pulmonary epithelial cell-specific genes and prosurfactant B protein in cocultured whole bone marrow cells separated by a cell-impermeable membrane. Lung-conditioned media had a similar effect on cocultured whole bone marrow cells and was found to contain pulmonary epithelial cell-specific RNA-filled microvesicles that entered whole bone marrow cells in culture. Also, whole bone marrow cells cocultured with lung had a greater propensity to produce type II pneumocytes after transplantation into irradiated mice. These findings demonstrate alterations of marrow cell phenotype by lung-derived microvesicles and suggest a novel mechanism for marrow cell-directed repair of injured tissue.

Acquired hypertrichosis lanuginosa as a presenting sign of metastatic prostate cancer with rapid resolution after treatment.

BACKGROUND: Acquired hypertrichosis lanuginosa (AHL) is a rare cutaneous disorder that involves spontaneous growth of lanugo-type hair in association with overt or occult malignant neoplasms. Bronchopulmonary and gastrointestinal malignancies are most commonly associated. CASE PRESENTATION: We report the occurrence of AHL associated with metastatic prostate cancer and its abrupt resolution after bilateral orchiectomy. To our knowledge, this is the first reported case of an association with prostate cancer. LIMITATIONS: The case presented represents a single patient; therefore, the findings and results reported may not be applicable to all patient populations. CONCLUSION: A variety of cutaneous findings are considered warning indicators of underlying neoplastic disease. Physician awareness of such signs can prompt timely and potentially life-saving investigations and interventions. AHL is regarded as such a sign. Physician awareness of the addition of prostate cancer to the growing list of AHL-associated malignancies provides rationale for appropriate testing and referral.