Wetter, David A.

AbstractBackgroundLeukocytoclastic vasculitis (LCV) in children is a complex group of conditions.ObjectivesThis study presents the demographics, clinical features, direct immunofluorescence (DIF) results and suspected aetiologies of 56 biopsy‐confirmed cases of leukocytoclastic vasculitis in children.MethodsRetrospective review of 56 children seen at Mayo Clinic in Rochester, Minnesota, from 1993 to 2013 with clinical features and cutaneous biopsy consistent with LCV.ResultsTwenty‐seven (48%) cases were found to be due to IgA vasculitis (Henoch‐Schonlein purpura). The remaining cases were found to be due to cutaneous small‐vessel vasculitis (n = 19, 34%), urticarial vasculitis (n = 5, 9%), ANCA‐associated vasculitis (n = 4, 7%) and acute haemorrhagic oedema of infancy (n = 1, 2%). IgA vasculitis was found to be associated with abdominal pain (P = 0.008), whereas the non‐IgA vasculitis group was associated with headache (P = 0.052). Children with IgA vasculitis had palpable purpura (P = <0.001), petechia (P = 0.057), vesicles (P = 0.009) and involvement of the buttock (P = 0.004) more frequently than the non‐IgA vasculitis group. On DIF, perivascular IgA was positive in IgA vasculitis compared to non‐IgA vasculitis cases (P = <0.001), the other conjugates were similar between the two groups.ConclusionThe most common subtype of biopsy‐confirmed LCV in children is IgA vasculitis. Clinical features, exam characteristics and DIF results can be helpful in determining the subtype of cutaneous vasculitis in children.

AbstractBackgroundThe epidemiology of cutaneous sarcoidosis is not well‐characterized as only referral‐based studies are available.ObjectivesTo characterize the epidemiology of cutaneous sarcoidosis, with emphasis on annual incidence and clinical characteristics, from 1976 to 2013.MethodsInception cohorts of patients with incident isolated cutaneous sarcoidosis and incident systemic sarcoidosis with cutaneous involvement in 1976–2013 in Olmsted County, Minnesota, United States were identified based on comprehensive individual medical record review. Inclusion in the isolated cutaneous sarcoidosis cohort required physician diagnosis and skin biopsy showing non‐necrotizing granuloma. Inclusion in the systemic sarcoidosis with cutaneous involvement cohort required presence of systemic sarcoidosis and cutaneous lesions. Presence of systemic sarcoidosis was determined by physician diagnosis supported by histopathology of non‐necrotizing granuloma, characteristic radiologic features of intrathoracic sarcoidosis and exclusion of other granulomatous diseases. Cutaneous lesions were defined as either sarcoidosis‐specific or non‐specific.ResultsThere were 62 cases with sarcoidosis‐specific cutaneous lesions (36 cases of sarcoidosis‐specific cutaneous lesions and 26 cases of isolated cutaneous sarcoidosis) which corresponded to an incidence of 1.9 per 100 000 population. The female to male ratio was 2.1 : 1. Plaques, papules and subcutaneous nodules were the most commonly observed cutaneous lesions. There was no significant difference in cutaneous presentation between those who had isolated skin disease and those who had skin disease in association with systemic sarcoidosis. Prognosis of cutaneous sarcoidosis was favourable, as over 90% of patients had a good response to either glucocorticoids, hydroxychloroquine or tetracycline antibiotics. This study has a significant limitation, in that the studied population was predominantly Caucasians who generally have a lower prevalence of skin disease.ConclusionsThe incidence of sarcoidosis‐specific cutaneous lesions was about 1.9 per 100 000 population with female predominance. The cutaneous presentations were similar among those with and without systemic sarcoidosis.

SummaryPneumocystis jiroveci pneumonia is an opportunistic infection associated with substantial rates of mortality in immunosuppressed patients. Prophylaxis recommendations are mostly targeted toward patients with non‐dermatologic diagnoses. This study was conducted to determine when dermatology patients treated with immunosuppressive medications should be offered P. jiroveci pneumonia prophylaxis. We searched the literature from January 1, 1993, to December 31, 2013, using terms relating to P. jiroveci pneumonia and dermatologic diagnoses to analyze the clinical characteristics of previously affected patients. Guidelines for P. jiroveci pneumonia prophylaxis from other medical fields were also analyzed. Of 17 dermatology patients reported to have contracted P. jiroveci pneumonia, eight (47.1%) died of the pneumonia. Risk factors included lack of prophylaxis, systemic corticosteroid therapy, lymphopenia, hypoalbuminemia, low serum CD4 counts, comorbid pulmonary or renal disease, malignancy, and prior organ transplantation. The present conclusions are limited by heterogeneity among the selected studies and limitations in their identification and selection. However, P. jiroveci pneumonia in dermatology patients is associated with a high mortality rate. Based on our analysis, we propose that prophylaxis be considered in dermatology patients in whom treatment with systemic corticosteroids at doses exceeding 20 mg/day or treatment with corticosteroid‐sparing immunosuppressive agents is anticipated for at least 4 weeks, and in patients with additional risk factors for P. jiroveci pneumonia.

AbstractBackgroundPublished case series describe lenalidomide as an effective treatment of refractory cutaneous lupus erythematosus (CLE).ObjectivesThe present study aimed to further characterize lenalidomide use in the treatment of CLE.MethodsA retrospective review of patients treated with lenalidomide for CLE from January 1, 2000, to December 17, 2014, was conducted.ResultsEight of the nine patients (89%) were women. Their median age at initiation of lenalidomide was 62 years (range: 41–86 years). Subtypes of CLE included discoid lupus erythematosus (DLE) (n = 6), lupus panniculitis (n = 2), and subacute CLE (n = 1). Before the initiation of lenalidomide, all patients had been previously treated unsuccessfully or were intolerant to at least one antimalarial and one immunosuppressive agent. With lenalidomide, five patients achieved a complete response (CR), two a partial response, and two had no response (lupus panniculitis). Time to initial response (dose range: 2.5–10.0 mg/d) varied from 2 weeks to 3 months; the median time to CR in five patients was 3 months (range: 3–6 months). The median duration of lenalidomide therapy was 12 months (range: 2–67 months). The median duration of follow‐up was 48 months (range: 20–103 months). Adverse effects included mild leukopenia; one patient had deep vein thrombosis of unclear etiology during a hospitalization. No patients developed or showed progression of systemic LE while receiving lenalidomide.ConclusionsLenalidomide was effective for the treatment of CLE (particularly DLE) but not for the treatment of lupus panniculitis in this series.

AbstractAutoimmune bullous diseases, such as pemphigus, pemphigoid, and dermatitis herpetiformis, are uniquely associated with vulnerability in the mucocutaneous barrier against infection. The management of immunobullous diseases is complex and may at times require immunosuppressive medications. Iatrogenic immunosuppression may increase susceptibility to vaccine‐preventable illnesses. Currently, there are no guidelines to assist the clinician treating patients with immunobullous disease regarding the delivery of various vaccinations. The aim of this review is to provide recommendations for immunization in the unique setting of immunobullous disease. Recommendations are based on careful review of the literature in other conditions requiring iatrogenic immunosuppression, as well as the most recent Centers for Disease Control and Prevention guidelines. Immunization with nonlive vaccines appears to be a safe and effective strategy for preventing infection in the particularly susceptible patient with immunobullous disease. Opportunities for live vaccine administration may become available at lower levels of immune suppression or during clinical remission when immunosuppressive regimens can be reduced. Anticipatory vaccination before the initiation of iatrogenic immunosuppression is ideal. Although immunologic response to vaccination may be suboptimal during immunosuppression, nonlive vaccination is strongly recommended for this patient population.

Frozen section pathology is routinely used for margin assessment of non‐melanoma skin cancer ( NMSC ). Frozen section can also be used for the primary diagnosis of several skin lesions. Limited data exist on the accuracy of frozen section in the diagnosis of NMSC . We performed a retrospective chart review of 300 cases in which frozen section diagnoses were compared with permanent section diagnoses of NMSC . Frozen section and permanent section pathology were concordant 83.3% of the time, with the highest concordance rates noted for basal cell carcinoma (145/153, 95%). Our results show a high level of concordance between frozen section and corresponding permanent section pathology diagnosis for NMSC . The rapidity of frozen section tissue processing and pathology reporting makes this technique useful in dermatologic practice for immediate diagnosis and management of NMSC . Further studies should explore strategies to decrease or eliminate discrepancies between frozen and permanent section diagnosis.

AbstractBackgroundPyoderma gangrenosum (PG) can be associated with systemic diseases, including inflammatory bowel disease, inflammatory arthritis, and hematologic malignancy. Previous literature exploring the rare association between PG and solid organ malignancy is predominantly limited to single case reports.MethodsWe retrospectively reviewed the cases of five patients with PG and solid organ malignancies at our institution between 1996 and 2013.ResultsFor the five patients identified, PG and solid organ malignancy occurred within three months of each other. Mean age at onset of PG was 55.6 years, and four patients were women. Three patients had breast carcinoma (one was recurrent), and two had gastrointestinal carcinoma. PG occurred before (n = 2), after (n = 2), or synchronously with (n = 1) the malignancy. In one patient with a history of PG and quiescent inflammatory bowel disease, recurrent PG developed synchronously with a new diagnosis of metastatic rectosigmoid carcinoma. Three patients had partial or complete response of PG with treatment of PG alone.ConclusionsTo our knowledge, our study is the first to report that the onset of PG can herald a recurrence of a solid organ malignancy and that recurrent PG can be associated with solid organ malignancy. A possible association of solid organ malignancy and PG should be considered in patients with PG of unknown etiology or with a history of malignancy.

AbstractBackgroundIntensive treatment options are lacking for patients with severe skin disease recalcitrant to outpatient therapy. The availability of inpatient dermatology care has almost disappeared in many parts of the United States. One possible solution for this is a day treatment center for patients with severe dermatologic disease recalcitrant to outpatient therapy.MethodsDescriptive study based on retrospective medical record review of all patients attending the day hospital for treatment in 2010. We collected data on patient demographics, distribution of admission diagnosis, treatments used, and length of stay.ResultsA total of 211 patients had 235 admissions. Mean age was 57.7 years (range 3.8–92.1 years). The most common indications for admission were dermatitis (139 admissions [59.2%]), psoriasis (58 [24.7%]), and mycosis fungoides (eight [3.4%]). The main treatment interventions were wet dressings (195 admissions [83.0%]) and Goeckerman treatment (38 [16.2%]). The median number of days of treatment was three (interquartile range, 2–5 days) for wet dressings and 22 days (interquartile range, 21–24 days) for Goeckerman therapy.ConclusionsThe dermatology day treatment center provided intensive skin‐directed therapy and filled a critical practice gap in the management of our patients with severe widespread skin diseases recalcitrant to outpatient therapy. We propose that the dermatology day treatment center is a valuable model that could be considered by both private practitioners and academic centers in the United States as an important adjunct to fill the gap in availability of intensive topical treatments for patients with severe skin disease.

AbstractBackgroundDermatology consultations are frequently requested by inpatient hospital services. As inpatient dermatology services in the USA decline, dermatology hospital consultations are becoming increasingly important.ObjectivesWe aim to describe the spectrum of skin diseases encountered and the health care subspecialties requesting dermatology hospital consultations.MethodsWe performed a retrospective chart review of adult patient (age: ≥18 years) dermatology hospital consultations from January 1 to December 31, 2010. We examined patient demographic characteristics, consultation requesting services, and consultation diagnoses.ResultsAmong dermatology services, 614 patients had 674 separate inpatient dermatology consultations during 2010. Of these patients, 55.9% were male (mean age: 59 years). In total, 205 consultations (30.4%) were requested by the internal medicine subspecialty, 137 (20.3%) by the hematology and oncology subspecialty, and 93 (13.8%) by the surgical subspecialty. The most common conditions seen by the hospital dermatology consulting service were skin infections (n = 125, 18.5%), dermatitis (n = 120, 17.8%), drug eruptions (n = 87, 12.9%), chronic wounds and ulcers (n = 55, 8.1%), cutaneous neoplasms (n = 39, 5.8%), graft‐versus‐host disease (n = 37, 5.5%), ecchymosis, purpura simplex or petechia (n = 26, 3.8%), intertrigo (n = 21, 3.1%), and urticaria (n = 20, 3.0%).ConclusionsThe majority of consultations conducted by the dermatology hospital consulting service were for the management of common skin diseases, such as cutaneous infections, dermatitis, and drug eruptions. Most consultations were requested by the departments of internal medicine, hematology and oncology, and surgical services.