Differentiating Pemphigus Foliaceus From Pemphigus Vulgaris in Clinical Practice.

Pemphigus is a skin condition that causes intraepidermal separation of keratinocytes. Multiple types of pemphigus exist, including pemphigus vulgaris and pemphigus foliaceus. These can be differentiated by histopathology, clinical presentation, appearance of lesions, and antibodies, among other factors. It is important to distinguish between the two because of differences in management and prognosis. Here we present a case of pemphigus foliaceus, as well as a discussion of the key differences between pemphigus foliaceus and vulgaris.

A recurrent melanocytic nevus phenomenon in the setting of Hailey-Hailey disease.

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.

Defining the role of CD2 in disease progression and overall survival among patients with completely resected stage-II to -III cutaneous melanoma.

BACKGROUND: Accurate assessment of prognosis remains clinically challenging in stage II to III cutaneous melanoma. Studies have implicated CD2 in immune surveillance, T-cell activation, and antitumor immunity, but its role in melanoma progression warrants further investigation. OBJECTIVE: We sought to investigate the prognostic role of CD2 in primary cutaneous melanoma. METHODS: Patients with American Joint Committee on Cancer stage II and III cutaneous melanoma were identified by retrospective review of dermatopathology databases from 2001 to 2010 at Mount Sinai Medical Center and Geisinger Medical Center. Additional patients were provided by New York University Medical Center based on retrospective review and tissue availability. Immunohistochemistry was performed on tumors from 90 patients with known recurrence status and documented follow-up. RESULTS: Primary tumors from patients who developed recurrent disease had fewer CD2(+) cells (P = .0003). In multivariable analyses including standard clinicopathologic predictors, CD2 was an independent predictor of disease recurrence (P = .008) and overall survival (P = .007). CD2 count correlated with characterization of tumor-infiltrating lymphocytes (P = .0004). Among the intermediate prognosis group of patients with nonbrisk tumor-infiltrating lymphocytes, CD2 count was predictive of disease recurrence (P = .0006) and overall survival (P = .0318). LIMITATIONS: Our retrospective design may have resulted in incomplete representation of patients lacking documented follow-up. CONCLUSIONS: CD2 may be an independent predictor of disease recurrence and overall survival among patients with primary cutaneous melanoma.

Dissection of immune gene networks in primary melanoma tumors critical for antitumor surveillance of patients with stage II-III resectable disease.

Patients with resected stage II-III cutaneous melanomas remain at high risk for metastasis and death. Biomarker development has been limited by the challenge of isolating high-quality RNA for transcriptome-wide profiling from formalin-fixed and paraffin-embedded (FFPE) primary tumor specimens. Using NanoString technology, RNA from 40 stage II-III FFPE primary melanomas was analyzed and a 53-immune-gene panel predictive of non-progression (area under the curve (AUC)=0.920) was defined. The signature predicted disease-specific survival (DSS P<0.001) and recurrence-free survival (RFS P<0.001). CD2, the most differentially expressed gene in the training set, also predicted non-progression (P<0.001). Using publicly available microarray data from 46 primary human melanomas (GSE15605), a coexpression module enriched for the 53-gene panel was then identified using unbiased methods. A Bayesian network of signaling pathways based on this data identified driver genes. Finally, the proposed 53-gene panel was confirmed in an independent test population of 48 patients (AUC=0.787). The gene signature was an independent predictor of non-progression (P<0.001), RFS (P<0.001), and DSS (P=0.024) in the test population. The identified driver genes are potential therapeutic targets, and the 53-gene panel should be tested for clinical application using a larger data set annotated on the basis of prospectively gathered data.

Re-examination of features to distinguish polyarteritis nodosa from superficial thrombophlebitis.

The correct identification of vessel type is crucial in distinguishing cutaneous polyarteritis nodosa from superficial thrombophlebitis. As the treatment and prognosis of these conditions are very different, correct diagnosis is critical, but they have overlapping clinical and histopathologic features, which can sometimes make the distinction difficult. Features commonly used to distinguish an artery from vein include vessel shape and diameter, the presence or absence of an internal elastic lamina, smooth muscle pattern, and the presence or absence of valves. Recently, it has been proposed that the amount and distribution of elastic fibers in the medial muscular layer are the most reliable features to make this distinction. The first part of this study used prosector-identified vessels to determine which of these features is most sensitive and specific for identifying an artery and vein. A total of 19 arteries and 16 veins were dissected from autopsy and amputation specimens. For each specimen, the smooth muscle pattern, elastic fiber pattern, the presence of valves, and the presence or absence of an internal elastic membrane were determined. The quantity of elastic fibers in the muscular wall of each sample was also determined. Although the elastic fiber pattern was the most specific feature in identifying a vein, it suffered from low sensitivity (43.8%). The smooth muscle pattern had the highest combined sensitivity and specificity. In the second part of this study, the histologic features listed above were examined in previously diagnosed cases of superficial thrombophlebitis and arteritis. When inflammation is present within and around the wall of the vessel, all of the studied histologic features become less reliable, and the interobserver reliability of distinguishing arteritis from thrombophlebitis was low. Our findings suggest that no single histopathologic feature is completely reliable and combining the histopathologic features with clinicopathologic correlation is essential for correct vessel identification.