Thomas Konia

Assessment of the ‘no eosinophils' rule: are eosinophils truly absent in pityriasis lichenoides, connective tissue disease, and graft‐vs.‐host disease?

Eosinophils are often present in the inflammatory infiltrate of an interface dermatitis, but the diagnostic specificity of eosinophils in interface dermatitis has not been formally evaluated. We retrospectively identified 97 examples of interface dermatitis with clinically confirmed diagnoses, including lupus erythematosus (LE), lichen planus, pityriasis lichenoides (PL), graft‐vs.‐host disease (GVHD), dermatomyositis (DM) and drug reaction. Diagnoses were clinically confirmed by at least two dermatologists. Slides were reviewed in a blinded fashion by at least two dermatopathologists. The average eosinophil count per 10 ×200 (×20 objective) fields was lowest for PL (0.2), DM (0.3), GVHD (0.4), and LE (0.5) [defined as Group 1] and was higher for lichen planus, drug reactions, erythema multiforme (major and minor) and viral exanthems [defined as Group 2]. Distinction between Group 1 and Group 2 was maximized using an eosinophil count cutoff of 1.1. In conclusion, eosinophils are usually rare to absent in PL, DM, most forms of LE and GVHD. While final interpretation requires a composite assessment of all features, our results suggest that the presence of even a single eosinophil within nine or ten ×20 fields argues against a diagnosis of PL, DM or LE.

ADAM15 Deficiency Attenuates Pulmonary Hyperpermeability and Acute Lung Injury in Lipopolysaccharide-Treated Mice

ADAM15 is a disintegrin and metalloprotease recently implicated in cancer and chronic immune disorders. We have recently characterized ADAM15 as a mediator of endothelial barrier dysfunction. Whether this molecule contributes to acute inflammation has not been evaluated. The purpose of this study was to investigate the role of ADAM15 in mediating pulmonary microvascular leakage during acute inflammatory injury. Immunofluorescent staining and Western blotting revealed that the endothelium was the main source of ADAM15 in lung tissue. In a mouse model of acute lung injury induced by lipopolysaccharide (LPS), upregulation of ADAM15 was observed in association with pulmonary edema and neutrophil infiltration. The LPS-induced inflammatory injury, as demonstrated by bronchoalveolar lavage neutrophil count, lung wet-to-dry weight ratio, and myeloperoxidase activity, was significantly attenuated in Adam15(-/-) mice. Studies with primary cell culture demonstrated abundant ADAM15 expression in endothelial cells (ECs) of mouse lung but not in neutrophils. Deficiency of ADAM15 in ECs had no obvious effect on basal permeability but significantly attenuated hyperpermeability response to LPS as evidenced by albumin flux assay and measurements of transendothelial electrical resistance, respectively. ADAM15 deficiency also reduced neutrophil chemotactic transmigration across endothelial barriers in the presence or absence of formyl-methionyl-leucyl-phenylalanine (fMLP). Rescue expression of ADAM15 in Adam15(-/-) ECs restored neutrophil transendothelial migration. These data indicate that ADAM15 upregulation contributes to inflammatory lung injury by promoting endothelial hyperpermeability and neutrophil transmigration.

Elastin staining patterns in primary cicatricial alopecia.

BACKGROUND Most biopsy specimens of cicatricial (scarring) alopecia can be readily subclassified as lymphocytic versus neutrophilic, but specific diagnosis remains difficult, particularly when a late stage of the disease is sampled. OBJECTIVE We sought to document patterns of scarring highlighted by elastic tissue staining in primary cicatricial alopecia. METHODS We documented Verhoeff elastic van Gieson staining patterns in 58 routinely embedded (vertical) biopsy specimens of cicatricial alopecia. Patterns of fibrosis included perifollicular (wedge-shaped vs broad tree trunk-shaped) and diffuse. The patterns were compared against the diagnosis obtained by independent expert clinical review, including central centrifugal cicatricial alopecia (CCCA), lichen planopilaris, traction alopecia, frontal fibrosing alopecia, discoid lupus erythematosus, and tufted folliculitis. RESULTS Wedge-shaped perifollicular fibrosis was seen in lichen planopilaris but also in CCCA. Broad tree trunk-shaped perifollicular fibrosis was most commonly encountered in CCCA. LIMITATIONS The retrospective nature of the study precluded temporal staging of the disease process. CONCLUSIONS Patterns of fibrosis highlighted by elastin staining in primary cicatricial alopecia appear to be disease specific. Superficial wedge-shaped perifollicular fibrosis is associated with but may not be specific for lichen planopilaris. Broad tree trunk-like perifollicular fibrosis is specific for CCCA but not present in many cases. Elastin staining represents a useful ancillary study for the evaluation of late-stage scarring alopecia in routinely oriented punch biopsy specimens.

Labyrinthine artery aneurysm as an internal auditory canal mass.

We present the first case report of a labyrinthine artery aneurysm masquerading as an internal auditory canal tumor. A 72-year-old woman presented with sudden onset right facial paralysis, facial pain, hearing loss, and vertigo. She demonstrated dense right-sided facial paralysis involving all branches of the facial nerve, left beating horizontal nystagmus, and anacusis of the right ear. Magnetic resonance imaging with contrast demonstrated a 6 × 7 mm peripherally enhancing lesion with lack of central uptake filling the right internal auditory canal. The patient elected to proceed with translabyrinthine approach to the internal auditory canal for definitive resection of the mass as well as to decompress the neural structures of the internal auditory canal in an attempt to recover neural function, particularly of the facial nerve. Intraoperatively, the internal auditory canal mass was resected with minimal difficulty, with intraoperative dissection notable for brisk bleeding at the medial base of the tumor just as the tumor was dissected off its medial fibrous attachments. Final pathology of the resected mass revealed a blood vessel with mucinous degeneration of the medial layer of the vessel wall, with immunohistochemical staining confirming the presence and structure of aneurysmal blood vessel.