IgG/IgA pemphigus in a patient with a history of pemphigus vulgaris: An example of epitope spreading?

We report a case of IgG/IgA pemphigus presenting as pemphigus foliaceus following diagnosis and treatment of classic IgG-mediated pemphigus vulgaris. The dual presentation of IgG and IgA positivity on direct immunofluorescence (DIF) constitutes a rare form of pemphigus with a wide variety of clinicopathologic manifestations. The progression of pemphigus vulgaris is commonly because of epitope spreading. However, the patient's disease was partially refractory to rituximab and showed a change in the DIF with dual staining for IgG and IgA. This indicates that class-switching may have occurred with epitope spreading or that there was autoreactive IgA at the onset of disease below the threshold of detection by DIF. Our case indicates that in progressive autoimmune disease refractory to treatment, re-evaluation of the patient for antibody isotypes absent on initial diagnosis may offer key information in better identifying the cause of progression as well as in directing the necessary treatment.

What's the story? An analysis of children's books about atopic dermatitis.

BACKGROUND/OBJECTIVES: Atopic dermatitis is an inflammatory skin condition with significant disease burden. Bibliotherapy, the use of storybooks to understand an illness, has been proven effective in several pediatric disorders but has not been studied in dermatologic disease. The purpose of this study was to assess the availability of storybooks about atopic dermatitis and analyze them based on adherence to American Academy of Dermatology guidelines for the treatment of atopic dermatitis, readability, author qualifications, cost, and availability. METHODS: Selection criteria included that the books were primarily about atopic dermatitis, were illustrated, cost less than $25, and were written in English. Storybooks were identified using an Internet search of the Google, Amazon, and Barnes & Noble websites. Flesch-Kincaid Grade Level was used to calculate reading level. RESULTS: Twenty-three storybooks were identified. The mean cost of the storybooks was $8.2 ± 7.0. Authors included mothers, professional authors, and medical professionals. Mean reading grade level was 4.1 ± 1.5. Of American Academy of Dermatology-recommended treatments, most storybooks mentioned moisturizers or bathing. Fewer than half discussed moisturizing after bathing, topical corticosteroids, wet wrap therapy, oral antihistamines, antimicrobials, systemic agents, or phototherapy. None mentioned topical calcineurin inhibitors or bleach baths. CONCLUSION: Storybooks about atopic dermatitis are available. Of those reviewed, none covered all the American Academy of Dermatology treatment guidelines. Studies have shown that bibliotherapy can be useful for education and behavioral modification for pediatric diseases, and future studies are needed to examine whether comprehensive, accurate storybooks about atopic dermatitis improve clinical outcomes or improve the quality of life of individuals with atopic dermatitis and their caregivers.

Basal Cell Carcinoma Arising in a Breast Augmentation Scar.

We report a case of a 46-year-old female who presented with a persistent lesion on the inferior right breast. The lesion was located within the scar from a breast augmentation procedure 12 years ago. The lesion had been treated as several conditions with no improvement. Biopsy revealed a superficial and nodular basal cell carcinoma, and the lesion was successfully removed with Mohs micrographic surgery. Basal cell carcinoma arising in a surgical scar is exceedingly rare with only 13 reported cases to date. This is the first reported case of basal cell carcinoma arising in a breast augmentation scar. We emphasize the importance of biopsy for suspicious lesions or those refractory to treatment, particularly those lesions that form within a scar. Level of Evidence V This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Vaccination with Melanoma Helper Peptides Induces Antibody Responses Associated with Improved Overall Survival.

PURPOSE: A melanoma vaccine incorporating six peptides designed to induce helper T-cell responses to melanoma antigens has induced Th1-dominant CD4(+) T-cell responses in most patients, and induced durable clinical responses or stable disease in 24% of evaluable patients. The present study tested whether this vaccine also induced antibody (Ab) responses to each peptide, and whether Ab responses were associated with T-cell responses and with clinical outcome. EXPERIMENTAL DESIGN: Serum samples were studied from 35 patients with stage III-IV melanomas vaccinated with 6 melanoma helper peptides (6MHP). IgG Ab responses were measured by ELISA. Associations with immune response and overall survival were assessed by log-rank test and χ(2) analysis of Kaplan-Meier data. RESULTS: Ab responses to 6MHP were detected by week 7 in 77% of patients, and increased to peak 6 weeks after the last vaccine and persisted to 6 months. Ab responses were induced most frequently to longer peptides. Of those with T-cell responses, 82% had early Ab responses. Survival was improved for patients with early Ab response (P = 0.0011) or with early T-cell response (P < 0.006), and was best for those with both Ab and T-cell responses (P = 0.0002). CONCLUSIONS: Vaccination with helper peptides induced both Ab responses and T-cell responses, associated with favorable clinical outcome. Such immune responses may predict favorable clinical outcome to guide combination immunotherapy. Further studies are warranted to understand mechanisms of interaction of these Abs, T-cell responses, and tumor control.

The quality of life impact of acne and rosacea compared to other major medical conditions.

BACKGROUND: Acne and rosacea cause significant negative impact on quality of life. There is limited information comparing the health-related quality of life (HRQL) impact associated with acne and rosacea to other patient populations. PURPOSE: We review available literature to assess the HRQL impact of acne and rosacea and compare them with major medical conditions. METHODS: A PubMed search identified studies that utilized the Short Form 36 (SF-36), the Dermatology Life Quality Index (DLQI), and the willingness-to-pay (WTP) metric to assess the HRQL impact of acne and rosacea. These data were compared to HRQL values for other diseases. RESULTS: The HRQL impact of acne is similar to asthma, epilepsy, diabetes, back pain, arthritis, and coronary heart disease using SF-36 data. DLQI scores for acne ranged from 2 to 17.7 and for rosacea ranged from 4.3 to 17.3; the DLQI scores for psoriasis ranged from 1.7 to 18.2. WTP data identified ranged widely for both acne and rosacea. LIMITATIONS: There was limited broadly generalizable data for acne and rosacea. CONCLUSIONS: Acne and rosacea impact HRQL to a similar degree as other major medical conditions by indirect comparison to psoriasis, a skin condition causing significant disability, and by direct comparison for acne. In the setting of limited health care resources, allocation should be grounded in the evidence that acne and rosacea are not trivial in their effects.

Systemic lupus erythematosus presenting as Stevens-Johnson syndrome/toxic epidermal necrolysis.

Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN)-like lesions in acute cutaneous lupus erythematosus [LE]) are an unusual manifestation of systemic LE. We describe a patient with widespread vesiculobullous lesions diagnosed as SJS/TEN-like acute cutaneous LE as the initial presentation of systemic LE. Stevens-Johnson syndrome/TEN-like LE may be differentiated from other vesiculobullous lesions by factors including a history of recent LE exacerbation, photodistribution of lesions, lack of a precipitating infection or medication exposure, minimal mucosal involvement, a prolonged course, response steroid treatment, and histologic and immunofluorescence findings. It is paramount to identify SJS/TEN-like LE as this condition requires early and aggressive intervention. The optimal treatment approach for SJS/TEN-like LE is unclear, and although some case reports have shown glucocorticoids to be useful, there are also reports of cases in which additional measures, such as intravenous immunoglobulin and plasmapheresis, were required to achieve a response. Our patient's condition was refractory to high-dose corticosteroids and intravenous immunoglobulin but was successfully treated using plasma exchange. As such, this treatment may hold potential for improving the care of other patients with refractory SJS/TEN-like LE.