Serum triamcinolone levels during intensive, inpatient wet-dressing therapy.

BACKGROUND: Wet dressings combined with topical corticosteroids are beneficial for patients with generalized and refractory dermatosis; however, to our knowledge, serum levels after topical corticosteroid absorption during intensive therapy have not been reported previously. AIM: To examine serum levels of triamcinolone acetonide (TAC) after topical corticosteroid application during intensive wet-dressing therapy. METHODS: We performed a retrospective study of adult patients admitted for inpatient wet-dressing therapy from 7 November 2015 to 24 June 2016. Data were collected on sex, age, body surface area, TAC serum levels, number of wet-dressing changes after 24 and 48 h, and type of wet dressing. RESULTS: In total, 29 patients (14 men, 15 women) were assessed. Median [interquartile range (IQR)] age was 57 years (51.5-67.0 years) and involved body surface area was 1.98 m2 (1.88-2.15) m2 . Before the 24-hour blood draw, patients had received 1-3 dressing changes. Median (IQR) TAC level at 24 h was 0.33 µg/dL (0.20-0.58 µg/dL), with no significant difference noted between the number of dressing changes and TAC serum level. At 48 h, results of a serum TAC test were available for 22 patients with 2-6 dressing changes. Mean (IQR) serum level was 0.30 µg/dL (0.30-0.87 µg/dL). For each additional dressing change, there was an estimated 0.21 µg/dL increase in TAC serum level (95% CI 0.11-0.31; P < 0.001). TAC serum level was not significantly associated with sex, age, body surface area or dressing type. CONCLUSIONS: Intensive, inpatient wet-dressing therapy is associated with detectable TAC serum levels. However, we suspect that topical TAC has a primarily local therapeutic effect on the skin.

Treatment of acne with spironolactone: a retrospective review of 395 adult patients at Mayo Clinic, 2007-2017.

BACKGROUND: Few large studies have assessed spironolactone treatment of adult female acne. OBJECTIVES: To explore the role of spironolactone in the treatment of adult female acne. METHODS: We performed a retrospective case series assessing the efficacy of spironolactone treatment of a cohort of women evaluated at Mayo Clinic in Rochester, Minnesota, from 2007 through 2017. RESULTS: In total, 395 patients (median age, 32 years) received a median spironolactone dose of 100 mg daily. Approximately two-thirds of patients (66.1%) had a complete response; 85.1% had a complete response or a partial response greater than 50%. Median times to initial response and maximum response were 3 and 5 months. Efficacy was observed across all severity subtypes of acne, including those with papulopustular and nodulocystic acne. Patients received long-term treatment with spironolactone (median duration, 13 months) and had few adverse effects. CONCLUSIONS: Spironolactone is a safe and effective treatment of acne for women.

Experience with the dermatology inpatient hospital service for adults: Mayo Clinic, 2000-2010.

BACKGROUND: There is a paucity of medical literature describing the role of dermatology inpatient hospital services for patients with severe dermatologic disease. A diminishing number of US hospitals have a dedicated dermatology inpatient service run by dermatologists. OBJECTIVES: To describe the role of a dermatology-run inpatient service in treatment of severe dermatologic disease from 2000 to 2010 at our institution. METHODS: We studied demographic characteristics, indications for admission and length of stay for the adult (age, >18 years) dermatology inpatient hospital service over the most recent decade. We compared data from the first 5.5 years with the subsequent 5.5 years and with previously published data. RESULTS: A total of 1732 patients had 2216 inpatient admissions to the adult service from 2000 to 2010. The mean (SD) age was 61.3 (17.7) years (age range 18-100 years). Median duration of admission was 3 days interquartile range (IQR), 2-5 days. The most common indications for admission were dermatitis (44.2%), psoriasis (17.4%) and cutaneous T-cell lymphoma (9.2%). We compared admissions from 2000 to mid-2005 (n = 1260) to admissions from mid-2005 to 2010 (n = 956). Statistically significant changes included median length of stay (decreased from 4 days [IQR, 3-6 days] to 3 days [IQR, 2-4 days] P < 0.01), admissions for psoriasis (decreased from 20.7% to 13.0%; P < .01) and admissions for dermatitis (increased from 41.6% to 47.6%; P < .01). CONCLUSION: The number of patients admitted and the median length of stay decreased between the 2 periods. Indications for admission have changed significantly across the two time periods.

Disease severity and therapy as predictors of cardiovascular risk in psoriasis: a population-based cohort study.

BACKGROUND: Previous studies suggest an increased risk of cardiovascular disease in psoriasis, but the relative contributions of traditional risk factors and markers of disease severity are unclear. We examined the effect of psoriasis disease characteristics on cardiovascular risk after adjusting for traditional cardiovascular risk factors. METHODS: Study populations included (a) case-cohort sample of 771 patients nested within a population-based psoriasis incidence cohort, and (b) cohort of 1905 patients with incident and prevalent psoriasis patients. Both cohorts were followed-up to ascertain disease and treatment characteristics, traditional cardiovascular risk factors and cardiovascular outcomes. Cox proportional hazards regression models were used to identify predictors of cardiovascular outcomes. RESULTS: After adjusting for traditional risk factors, increasing number of psoriasis-affected body sites at disease onset (HR: 1.53 per additional site, 95% CI: 1.20, 1.95) was significantly associated with an increased risk of cardiovascular outcomes. Phototherapy (HR: 3.76, 95% CI: 2.45, 5.77) and systemic therapy (HR: 2.17, 95% CI: 1.50, 3.13) were associated with a higher risk of cardiovascular outcomes in univariate analyses, but these relatively strong associations disappeared after adjusting for cardiovascular risk factors. CONCLUSIONS: Increasing number of psoriasis-affected body sites may be a severity indicator in psoriasis and is associated with an increased cardiovascular risk. Due to low number of patients exposed to systemic therapy, this study had limited power to examine the effect of treatment on cardiovascular risk. Strong associations with phototherapy and systemic therapy suggest that the cardiovascular risk in psoriasis is confined to patients with severe disease.

Naproxen-associated linear IgA bullous dermatosis: case report and review.

Linear IgA bullous dermatosis (LABD) is an acquired autoimmune subepidermal blistering disorder in which linear deposits of IgA are found along the basement membrane. Idiopathic, systemic disorder-related, and drug-induced forms of LABD have been described. Drug-induced LABD occurs in association with drug administration and resolves when the offending agent is discontinued. Other forms of LABD assume a more chronic course. The nonsteroidal anti-inflammatory drugs piroxicam and diclofenac have been previously reported to induce LABD. To our knowledge, this article describes the first documented case of LABD associated with naproxen administration, which resolved after discontinuation of the drug.

Assessment of the accuracy of low-cost store-and-forward teledermatology consultation.

BACKGROUND: Telemedicine has the potential to revolutionize the delivery of dermatologic care to underserved areas. OBJECTIVE: Our purpose was to compare diagnoses from two types of dermatology consultations: telemedicine using store-and-forward (SAF) technology, and traditional face-to-face (FTF) office visits. METHODS: Skin conditions were imaged with a consumer-grade digital camera. A standardized template was used to collect historical data. Information was stored in a secured database for access by 2 or 3 board-certified dermatologists. Results from the FTF visit were used to assess the accuracy of the SAF diagnoses. RESULTS: A total of 106 dermatologic conditions in 92 patients were included. Concordance between FTF and SAF diagnoses was high, ranging from 81% to 89% for all 3 dermatologists. Clinically relevant disagreement occurred in only 4% to 8% of cases. Remaining disagreements did not affect patient care. Diagnostic confidence and image quality affected agreement. When cases of high confidence were analyzed separately, agreement increased to 88% to 100%. This increase was substantiated by means of a chi-square test between the high confidence and low confidence groups, which demonstrated statistical significance (P <. 005) for all dermatologists. Similarly, when cases of above average image quality were considered, agreement increased to 84% to 98%. Again this difference was substantiated by means of a chi-square test between adequate and poor images, with statistical significance for two dermatologists (P <.001). Accuracy was comparable between disease types with the exception of benign neoplasms, which demonstrated agreement of 22% to 46%. CONCLUSION: These data support the use of existing digital technology to construct an accurate SAF teledermatology system. The inexpensive camera and widely available computer equipment make this an extremely affordable system. Furthermore, participating dermatologists appear well aware of system limitations, as reflected in the increased agreement for high confidence cases. Additional investigation of the accuracy of teledermatology for benign neoplasms is warranted.

Clinical consultations using store-and-forward telemedicine technology.

OBJECTIVE: To investigate the potential role of a store-and-forward (SAF) telemedicine system in specialty consultations initiated by primary care physicians. MATERIALS AND METHODS: In this pilot telemedicine study, patients needing consultations in cardiology, dermatology, endocrinology, and orthopedics had both standard face-to-face (FTF) consultations and SAF consultations. RESULTS: Fifteen patients had both FTF and SAF consultations, 4 had echocardiograms transmitted for an SAF consultation only, and 1 had an SAF consultation but no FTF appointment. Of 19 diagnoses made, all were essentially the same in both types of consultations; 14 of 15 FTF consultations and 15 of 19 SAF consultations resulted in additional treatment recommendations. CONCLUSIONS: While it was possible to develop a desktop system for SAF consultations, the equipment was not adequately integrated. Without total digital input, including electronic patient medical records, packaging of information is laborious and impractical. Seamlessly adapting to existing clinical practice is vital. Issues such as increasing work for the physicians or office staff, gathering adequate patient information, and designing a referral process were more difficult than we had anticipated. Patient acceptance was high, but the clinical pilot had very small numbers.

Exaggerated arthropod-bite lesions in patients with chronic lymphocytic leukemia: a clinical, histopathologic, and immunopathologic study of eight patients.

BACKGROUND: Unusual papulovesicular lesions resembling arthropod bites have been described in patients with chronic lymphocytic leukemia (CLL). OBJECTIVE: Our purpose was to describe and characterize further the clinical, histopathologic, and immunopathologic features of these lesions. METHODS: Eight patients were identified retrospectively who had CLL and characteristic skin lesions. Clinical and histologic features were recorded. Skin biopsy specimens were analyzed immunohistochemically for eosinophil granule major basic protein, eosinophil-derived neurotoxin, neutrophil elastase, and mast cell tryptase. RESULTS: The clinical features, including the lesional distribution, suggested arthropod bites, although most patients could not recall having been bitten. Mixed T- and B-cell lymphoid cell infiltrates were present within lesions, along with prominent eosinophil infiltration and eosinophil granule protein deposition. CONCLUSION: Exuberant papulovesicular lesions develop in patients with CLL apparently as an exaggerated response to arthropod bites. Prominent eosinophil infiltration and degranulation within these lesions likely contribute to the severity of symptoms.

Subcorneal pustular dermatosis: a clinical study of ten patients.

Subcorneal pustular dermatosis of Sneddon and Wilkinson (SPD) is a unique clinical and histologic entity. This disorder has been reported in association with monoclonal gammopathy and IgA pemphigus. We report ten additional cases of SPD and determine the frequency of monoclonal gammopathy and IgA pemphigus in patients with SPD and investigate the relationship of this entity with other dermatoses characterized histologically by a subcorneal pustule. The medical records of patients with SPD evaluated at the authors' institution from 1980 through 1995 were reviewed retrospectively. Medical records of twenty patients with pustular psoriasis were reviewed for comparison. Ten patients met the criteria for SPD. Their average age at diagnosis was 66 years. The characteristic flaccid pustules were often generalized and had a tendency to involve the flexural areas. Serum monoclonal gammopathy was present in four patients: three with IgA and one with IgG. On direct immunofluorescence examination, three patients had IgA deposits in the intercellular spaces. None of the patients exhibited both monoclonal gammopathy and IgA deposits. Repeated direct immunofluorescence studies were necessary to detect IgA pemphigus in one patient. SPD is a distinct clinical entity. The findings of IgA deposits intercellularly on immunofluorescence and monoclonal gammopathy may help to further identify this entity.

Topical psoralen-ultraviolet A therapy for palmoplantar dermatoses: experience with 35 consecutive patients.

OBJECTIVE: To report the effectiveness of topical psoralen-ultraviolet A (PUVA) therapy for palmoplantar dermatoses and to describe our method of administration of this treatment modality. DESIGN: We conducted a retrospective study of 35 patients, 27 to 66 years of age, who received topical hand and foot PUVA therapy. MATERIAL AND METHODS: Ten patients had psoriasis vulgaris, 8 had pustular psoriasis, 5 had dyshidrotic eczema, and 12 had other types of dermatitis. The affected area was soaked in a psoralen solution, 0.0005% methoxsalen (10 mg of methoxsalen in 2 L of warm water), for 30 minutes and then exposed to incremental amounts of ultraviolet A light three times weekly until the dermatosis subsided or cleared. Treatment frequency was then reduced and ultimately discontinued. Time to maximal improvement of the hands and feet was defined as the period from the beginning of PUVA treatment to the point at which the frequency of treatment was reduced from three times weekly to twice weekly. RESULTS: Fourteen patients (40%) had clearing of their disease, and 14 others (40%) had improvement of their conditions. The mean time to clearing was 2.8 months (range, 2 weeks to 7 months), and the mean number of treatments was 27.3 (range, 10 to 55). The mean total dose of ultraviolet A received was 140 J/cm2 (range, 9 to 530). Two patients (6%) had no response to treatment. Mild localized ultraviolet-related erythema developed in 16 patients (46%). Five patients "dropped out" of treatment, and nine other patients discontinued treatment during maintenance therapy. The principal reason cited was inconvenience. CONCLUSION: Topical PUVA therapy is an effective and safe treatment option for recalcitrant dermatoses affecting the palms and soles.

Coexistence of lupus erythematosus and porphyria cutanea tarda in fifteen patients.

BACKGROUND: Lupus erythematosus (LE) and porphyria cutanea tarda (PCT) may have similar clinical presentations, and their coexistence presents special problems in diagnosis and management. OBJECTIVE: The purposes of this study were to describe a patient with discoid LE and PCT and to present a review of 15 patients with coexistent LE (all variants) and PCT. METHODS: The medical records of all patients with coexistent LE and PCT seen between 1976 and 1995 were retrospectively reviewed. RESULTS: Of 6179 cases of LE and 676 cases of porphyria (all variants), there were 15 patients, 6 men and 9 women, with coexistent LE and PCT. The mean age at diagnosis of LE was 42.8 years and of PCT was 48 years. Nine patients had discoid LE; five patients had systemic LE, and one patient had subacute cutaneous LE. The initial diagnosis was LE in eight patients, PCT in five patients, and simultaneous LE and PCT in two patients. Precipitating factors for PCT included alcohol in seven patients, iron overload in one patient, and estrogen in one patient. Treatment of LE with hydroxychloroquine 200 mg daily precipitated PCT in two patients. Patients were treated with phlebotomy or low-dose antimalarials for PCT. Patients with systemic LE received systemic glucocorticoid therapy. Patients with discoid LE and subacute cutaneous LE were treated with topical glucocorticoids. CONCLUSION: The association of LE and PCT poses therapeutic challenges. The preferred treatment for one may exacerbate the other. Use of standard dose antimalarials for LE is inadvisable, and phlebotomy or low-dose antimalarials should be used cautiously in patients with coexistent disease.

Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation.

BACKGROUND: Allergic granulomatosis of Churg-Strauss (Churg-Strauss syndrome) is a distinct clinical disease of multisystem vasculitis. OBJECTIVE: We characterize the clinical and histologic features of cutaneous findings in Churg-Strauss syndrome. METHODS: All patients with Churg-Strauss syndrome seen between 1976 and 1995 were retrospectively reviewed. RESULTS: Ninety patients with the diagnosis of Churg-Strauss syndrome were identified; 36 (40%) had cutaneous findings. Five patients (6%) had skin lesions as the initial manifestation. The most frequent cutaneous findings were purpura and petechiae on the lower extremities and cutaneous nodules and papules on the elbows. In 37 biopsy specimens from 29 patients, the most common findings were extravascular necrotizing granuloma (15 specimens) and leukocytoclastic vasculitis (16 specimens). CONCLUSION: Cutaneous lesions in Churg-Strauss syndrome are common. Their characteristic clinical and histologic pattern may help establish the diagnosis.

A new autosomal dominant disorder of pyogenic sterile arthritis, pyoderma gangrenosum, and acne: PAPA syndrome.

OBJECTIVE: To describe a multigenerational family with transmission of an autosomal dominant disorder characterized by pyogenic arthritis, pyoderma gangrenosum, and severe cystic acne. MATERIAL AND METHODS: We present a detailed case report of a 39-year-old man with arthritic changes in several joints, pyoderma gangrenosum, and cystic acne. Several relatives from three generations of his family underwent clinical and genetic investigations. The findings in this kindred are reported. RESULTS: Ten affected family members in three generations manifested variable expression of a pauciarticular, nonaxial, destructive, corticosteroid-responsive arthritis that began in childhood; pyoderma gangrenosum; and severe cystic acne in adolescence and beyond. Other less commonly associated features included adult-onset insulin-dependent diabetes mellitus, proteinuria, abscess formation at the site of parenteral injections, and cytopenias attributable to sulfonamide medications. Laboratory evaluation was nondiagnostic. Genetic studies excluded linkage to the major histocompatibility locus. CONCLUSION: The acronym of PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, and acne) is suggested for this newly recognized pleiotropic autosomal dominant disorder. The nature of the genetic alteration in PAPA syndrome is unknown.

Generalized gravis junctional epidermolysis bullosa: case report, laboratory evaluation, and review of recent advances.

A full-term infant with junctional epidermolysis bullosa (JEB) is described. The distribution and morphologic characteristics of generalized blistering in areas of pressure in conjunction with perioral and perinasal granulation tissue suggested the diagnosis of generalized gravis (Herlitz) JEB. The family history was consistent with autosomal recessive inheritance. Electron microscopy demonstrated a subepidermal cleft arising in the lamina lucida with hemidesmosomal hypoplasia, findings consistent with gravis JEB. Immunofluorescent antigenic mapping localized laminin and type IV collagen exclusively to the blister base and weak reactivity of bullous pemphigold antigen to both the roof and the base. Type VII collagen (LH 7:2 epitope) was detected solely at the base of the cleavage plane, and abnormal staining of laminin 5 (kalinin, GB3, nicein) and 19-DEJ-1 antigen was observed. The patient died of sepsis at age 3 months. DNA extracted from cultured keratinocytes for molecular genetic analysis demonstrated a mutation with the LAMB3 gene encoding the beta 3 chain of laminin 5. We present the clinical and laboratory findings and briefly review recent advances in the diagnosis and management of JEB.

A medical-center-wide, multidisciplinary approach to the problem of natural rubber latex allergy.

Latex is a common cause of occupational allergy in health care workers; latex-sensitized patients are at increased risk of allergic reactions in medical environments. Skin test reagents and latex-specific immunoglobulin E immunoassays were established for diagnosis of latex allergy. Inhibition immunoassays were developed for measuring latex aeroallergens and latex allergens in rubber products. A registry of latex-sensitive employees was established. High-allergen gloves were removed from the medical center inventory; latex aeroallergen levels subsequently declined. Despite an increasing number of gloves used annually, expenditures for gloves in 1994 were lower than in previous years. Latex-sensitive individuals can be identified using skin tests or immunoassays. Latex aeroallergen levels in medical environments can be reduced substantially at lower cost by using powder-free rubber gloves with lower allergen content.