Vork, Diana L.

AbstractAcral localized acquired cutis laxa (ALACL) is a rare variant of acquired cutis laxa, and the clinical appearance is characterized by loose, redundant and wrinkled skin of the distal extremities. By definition, histopathology of affected tissue reveals sparse or fragmented elastic fibers. However, this can be difficult to assess on routine staining, and sometimes requires electron microscopy. The condition has been associated with plasma cell dyscrasias or recurrent inflammatory states. We present a case of a 65‐year‐old man who presented with enlarged and doughy finger pads. Skin biopsy showed diffuse dermal amyloid deposition displacing dermal stroma and reduction of elastic fibers, although these changes were subtle on routine hematoxylin and eosin staining. Mass spectrometry of laser capture microdissected tissue showed AL kappa‐type amyloid and further workup revealed a diagnosis of primary systemic AL‐kappa amyloidosis requiring bone marrow transplantation. This case represents an unusual presentation of acquired cutis laxa and highlights the need for a high index of suspicion when reviewing histopathology of this entity. In addition, the case highlights the importance of investigation into possible systemic associations, such as plasma cell dyscrasias.

AbstractBackground/ObjectivesSkin infection is common in atopic dermatitis (AD), often necessitating treatment with systemic antibiotics. Topical adjunctive therapies such as dilute bleach baths are increasingly recommended, and topical dilute acetic acid (AA) has not been widely studied. The objective of this study was to evaluate whether various topical anti‐infective bathing recommendations were associated with decreased systemic antibiotic exposure in pediatric AD, as well as evaluate topical anti‐infective recommendations over time within our institution.MethodsClinical data were extracted from charts of pediatric patients from 1/1/2000 to 12/31/2005 and 1/1/2009 to 12/31/2014 who visited outpatient dermatology clinics at Mayo Clinic, Rochester, a tertiary referral center. Recommendations for topical anti‐infectives (dilute AA, dilute bleach, other, or none) at any time after patient had clinically proven or suspected AD superinfection were recorded as exposures to topical anti‐infective. Primary outcome was the number of systemic antibiotic courses in a 1‐year period.ResultsOf 1111 patients with AD, 753 met inclusion criteria (303 in 2000‐2005; 450 in 2009‐2014). Of these, 351 (46.6%) had culture‐proven or clinically suspected superinfection. Topical anti‐infective recommendations increased between the time periods (23.3% to 79.2%; P < 0.001) and number of courses of systemic antibiotics decreased (1.9 per year vs 1.5 per year, P = 0.010). Number of systemic antibiotic courses did not differ between those who received bathing recommendations and those who did not, nor between different anti‐infective groups (P = 0.398).ConclusionsPractice behaviors have changed, and topical anti‐infectives are now commonly recommended. Neither dilute AA nor bleach baths were associated with fewer subsequent exposures to systemic antibiotics in the treatment of pediatric AD.

Objective: To report the presentation, diagnosis, management, and convalescence of labyrinthine sequestrum (LS) and summarize all previously published cases. Patient(s): Eleven-year-old female with LS. Intervention(s): Multidisciplinary diagnostic evaluation and treatment. Main Outcome Measures: Imaging and laboratory findings, medical and surgical treatment. Results: We describe a case of LS secondary to medically recalcitrant suppurative otitis media in an 11-year-old female and review all eight previously reported cases. The index patient presented after 6 months of otitis media, profound unilateral hearing loss, with symptoms suggesting meningitis. Temporal bone CT demonstrated marked bony destruction of the left otic capsule. Gadolinium-enhanced MRI showed an enhancing process with evidence of meningitis and subdural empyema. The patient was treated with surgical debridement and culture directed antibiotic therapy. Posttreatment imaging showed resolution of intracranial infection with fibrous bony healing of the otic capsule resembling fibrous dysplasia. Conclusion: LS is a rare form of labyrinthitis characterized by centrifugal destruction of the otic capsule. The current index case highlights the importance of combined medical and surgical treatment and describes for the first time in the literature the fibrous ossification of the otic capsule following disease resolution.