Faculty Bibliography

BACKGROUND:Secondary syphilis has a wide spectrum of clinical and histologic manifestations. OBJECTIVE:We sought to determine the frequency of histopathological features characterizing secondary syphilis, and which are most common in specimens displaying few diagnostic findings. METHODS:In a multicenter, retrospective analysis of biopsy-proven secondary syphilis, cases were subcategorized by the number of histologic characteristics present. RESULTS:The 106 cases mostly had 5 to 7 of the features studied. Many features were scarcer in cases with 5 or fewer features, including endothelial swelling (87.7% overall vs 72.4% ≤5 features), plasma cells (69.8% vs 48.3%), and elongated rete ridges (75.5% vs 27.6%). Specimens with 5 or fewer features were more likely to be truncal (61.1% vs 34.4% overall), demonstrate rete ridge effacement (44.8% vs 19.8%), and have pityriasis rosea (33.3% vs 17.2%) or drug eruption (33.3% vs 10.9%) in the clinical differential. An interstitial inflammatory pattern was the most common characteristic of specimens with 5 or fewer features (75.9%). LIMITATIONS/CONCLUSIONS:This was a retrospective review. CONCLUSION/CONCLUSIONS:The independent value of many histologic features of syphilis may be overestimated. Combinations of endothelial swelling, interstitial inflammation, irregular acanthosis, and elongated rete ridges should raise the possibility of syphilis, along with the presence of vacuolar interface dermatitis with a lymphocyte in nearly every vacuole and lymphocytes with visible cytoplasm.

Atypical acquired melanocytic nevi in patients with epidermolysis bullosa (EB) have been referred to as EB nevi and are considered to be a type of recurrent nevus with atypical but distinctive histopathologic findings. Herein, we describe an atypical nevus in a patient with Hailey-Hailey disease with different histopathologic findings from EB nevi because of presumably different pathogenesis. It is important to be aware that the recurrent nevi phenomenon can be seen in acantholytic conditions as well as blistering disorders, given these lesions may clinically resemble melanoma.

Bullous pemphigoid (BP) is an autoimmune blistering disease mediated by autoantibodies targeting BP180 (type XVII collagen). Patient sera and tissues typically have IgG and IgE autoantibodies and elevated eosinophil numbers. Although the pathogenicity of the IgE autoantibodies is established in BP, their contribution to the disease process is not well understood. Our aims were two-fold: 1) To establish the clinical relationships between total and BP180-specific IgE, eosinophilia and other markers of disease activity; and 2) To determine if eosinophils from BP patients express the high affinity IgE receptor, FcεRI, as a potential mechanism of action for IgE in BP. Our analysis of 48 untreated BP patients revealed a correlation between BP180 IgG and both BP180 IgE and peripheral eosinophil count. Additionally, we established a correlation between total IgE concentration and both BP180 IgE levels and eosinophil count. When only sera from patients (n = 16) with total IgE ≥ 400 IU/ml were analyzed, BP180 IgG levels correlated with disease severity, BP230 IgG, total circulating IgE and BP180 IgE. Finally, peripheral eosinophil count correlated more strongly with levels of BP180 IgE then with BP180 IgG. Next, eosinophil FcεRI expression was investigated in the blood and skin using several methods. Peripheral eosinophils from BP patients expressed mRNA for all three chains (α, β and γ) of the FcεRI. Surface expression of the FcεRIα was confirmed on both peripheral and tissue eosinophils from most BP patients by immunostaining. Furthermore, using a proximity ligation assay, interaction of the α- and β-chains of the FcεRI was observed in some biopsy specimens, suggesting tissue expression of the trimeric receptor form in some patients. These studies provide clinical support for the relevance of IgE in BP disease and provide one mechanism of action of these antibodies, via binding to the FcεRI on eosinophils.

Pemphigus is a rare autoimmune blistering disease usually treated with systemic glucocorticoids with adjuvant immunosuppressants or anti-inflammatories. However significant morbidity and mortality is associated with these treatments. This review discusses conventional therapeutic options, as well as new and emerging therapies that may be safer alternatives to broad-based immunosuppression.

Acquired digital fibrokeratoma is a rare benign fibroepithelial tumor that typically presents as a solitary asymptomatic nodule on the finger or toe. Middle-aged adults are most commonly affected. Here we discuss an unusual case of acquired digital fibrokeratoma presenting as a cluster of multiple nodules on the sole of a 15-year-old boy.