Dermatopathology, Volume 8, Issue 4 (December 2021) – 11 articles

Early detection of skin cancer is of critical importance to provide five year survival rates that approach 99%. By 2050, one out of five Americans by age 70 will develop some form of skin cancer. This will result in a projected rate of 50 million skin biopsies per year given the current rate of escalation. In addition, the ability to differentiate between pigmented lesions and melanomas has proven a diagnostic challenge. While dermoscopy and visual analysis are useful in identifying many skin lesions, additional non-invasive techniques are needed to assist in the analysis of difficult to diagnose skin tumors. To augment dermoscopy data, we have developed 3D maps based on physical biomarker characteristics of benign and cancerous lesions using vibrational optical coherence tomography (VOCT). 3D images based on quantitative physical data involving changes in cellular and fibrous tissue stiffness along with changes in vascular quality are used to map and evaluate different types of cancers. 3D tumor maps constructed using quantitative VOCT data and OCT images have been used to characterize the differences between melanoma and other lesions. These characteristics can be used to plan the excision of difficult lesions where extensive surgery may be needed to remove the entire tumor in one step. In addition, it is now possible to use dermoscopy and VOCT to non-invasively differentiate between different cancerous lesion types using measurements of the resonant frequency of new cellular and vascular peaks. Quantitative VOCT information along with dermoscopic findings can be collected and analyzed remotely using artificial intelligence to improve cancerous tissue diagnosis. Full article

We present the case of an 83-year-old woman who presented with an advanced cutaneous angiosarcoma of the head. The tumor had first appeared as a small ecchymosis on her forehead 3 months before admission. There was an extremely rapid and dramatic evolution, as evidenced by photographic documentation by her relatives. Unfortunately, the delay in access to the healthcare system due to the COVID-19 pandemic lockdown and the fulminant growth were the main determinants for our patient outcome. Full article

Background: SPARK nevus represents a little-known and characterized entity, with few case series available in the literature. Methods and results: we present a case series of 12 patients (6 F and 6 M) between January 2005 and December 2020 and conduct a review of the current literature. Ten articles were selected on the basis of the adopted inclusion criteria and the PRISMA guidelines. Conclusions: The definition of histopathological and dermoscopic criteria are important to allow for an agreement to be reached among dermopathologists, and for the development of a consensus on higher case studies. To our knowledge, there are not many case series in the literature, and ours is part of the attempt to increase the knowledge of an entity that remains little-known and characterized. Full article

Primary cutaneous gamma-delta T cell lymphomas (PCGDTCLs) are rare and aggressive cutaneous malignancies that have been diagnostically challenging for dermopathologists and clinicians since their first published descriptions in 1991. Since then, the availability of immunostaining for T cell receptors γ and δ in formalin-fixed paraffin-embedded samples has greatly increased our knowledge of the gamma-delta phenotype by showing that it may also be present in the context of indolent entities, such as mycosis fungoides (MFs) and lymphomatoid papulosis, and this has raised questions concerning its diagnostic and prognostic implications. We here describe the histological and clinical differences between the dermo-epidermal and subcutaneous sub-groups of PCGDTCL observed in a cohort of 20 patients attending a single experienced centre, with particular focus on cases with an MF-like presentation, which are still less well defined than those of classic MF. Full article

Mycetoma is an uncommon, chronic infective disease of the skin and subcutaneous tissues, characterized by the triad of tumefaction, draining sinuses, and the presence in the exudate of colonial grains. In cases of long-term disease, the presence of colonial grains together with the host’s derivative material can lead to the formation of real sinuses. Histological analysis is of fundamental importance to allow an accurate etiological diagnosis and to understand if the basic pathogen is an actinomycete (bacterium) or a real fungus (eumycetic mycetomas) and is also fundamental for therapy, which is quite different. Here, we present a case of Mycetoma in an 11-year-old patient who emigrated from Djibouti, Somalia, and showed the essential histopathological features of this rare and forgotten nosographic entity in the industrialized world and briefly discuss the major and most important differential diagnoses. Full article

A 66-year-old male presented with a one-month history of persistent pruritic eruptions distributed mainly on the trunk. A punch biopsy from the left upper abdomen revealed focal acantholytic dyskeratosis with mixed inflammatory infiltrate in the dermis composed of numerous eosinophils. Grover’s disease was diagnosed based on the clinical and histopathological findings. Appropriate treatment was initiated but failed to relieve symptoms of itchiness. A further investigation of the hair follicles under mineral oil preparation revealed an infestation of pediculosis pubis. Subsequent treatment with Ivermectin and permethrin cream led to the complete resolution of his symptoms. This case report highlights an unusual and first documented case of Grover’s disease with a concomitant infestation of pediculosis pubis. To date, no reported cases in the literature have associated Grover’s disease with pubic lice infestation. However, there are three reported cases of concurrent scabies and Grover’s disease in the literature. This rare case underscores the clinical value in further investigating treatable underlying conditions in patients with suspected transient acantholytic dermatosis. Full article

Dedifferentiated melanoma is a particular form of malignant melanoma with a progressive worsening of the patient’s clinical outcome. It is well known that melanoma can assume different histo-morphological patterns, to which specific genetic signatures correspond, sometimes but not always. In this review we address the diagnostic difficulties in correctly recognizing this entity, discuss the major differential diagnoses of interest to the dermatopathologist, and conduct a review of the literature with particular attention and emphasis on the latest molecular discoveries regarding the dedifferentiation/undifferentiation mechanism and more advanced therapeutic approaches. Full article

Our knowledge in vascular anomalies has grown tremendously in the past decade with the identification of key molecular pathways and genetic mutations that drive the development of vascular tumors and vascular malformations. This has led us to better understand the pathogenesis of vascular lesions, refine their diagnosis and update their classification while also exploring the opportunity for a targeted molecular treatment. This paper aims to provide an overview of venous malformations (VM) in childhood. Specific entities include common VMs, cutaneo-mucosal VM, blue rubber bleb nevus syndrome or Bean syndrome, glomuvenous malformation, cerebral cavernous malformation, familial intraosseous vascular malformation and verrucous venous malformation. The clinicopathological features and the molecular basis of each entity are reviewed. Full article

(1) Background: Coronavirus disease 2019 (COVID-19) vaccines are currently employed on a population-wide scale in most countries worldwide. Data about unusual cutaneous adverse drug reactions (ADR) are scant, though. (2) Methods: We retrospectively analyzed moderate to severe vaccine-related ADR in the Department of Dermatology and Allergy of the University Hospital Bonn between May to June 2021 and analyzed related skin biopsies. (3) Results: As a specialized dermatological academic center, we encountered a total of n = 19 clinically and pathologically heterogeneous cutaneous ADR with a female predominance. Delayed cutaneous ADR occurred as late as 30 days after vaccination. The majority of ADR were mild, though a few patients required systemic treatment (antihistamines, glucocorticosteroids). (4) Conclusions: The clinico-pathological spectrum of cutaneous side effects with COVID-19 vaccines is wide; however, the benefits outweigh the risks by far. More dermatopathological studies on cutaneous ADR not limited to COVID-19 vaccines are desirable to enable a better understanding of underlying pathophysiological mechanisms. Full article

Erosive pustular dermatosis of the scalp (EPDS) is an uncommon, pustular, idiopathic disorder typically occurring on the scalp of the elderly, whose diagnosis requires close clinicopathologic correlations. Recently, the primary histopathologic characteristic of EPDS has been identified in some biopsies from hair-bearing scalp lesions as a sterile, vesiculo-pustule involving the infundibulum of hair follicles. To further delineate the clinicopathologic spectrum of the disease, we led a retrospective study of 50 patients (36 males and 14 females) with a diagnosis of EPDS between 2011 and 2021, reviewing clinical and histopathological data. Androgenetic alopecia was present in 32 patients. Triggering factors were present in 21 patients. The vertex was the most common location; one patient also had leg involvement. Two cases were familial. Disease presentation varied markedly from tiny, erosive, scaly lesions to crusted and hemorrhagic plaques, mimicking pustular pyoderma gangrenosum (PPG). Biopsies of patients with severe androgenetic or total baldness produced specimens showing nonspecific pathologic changes (39/50), while in 11 patients with a hair-bearing scalp histopathologic examination, changes were specific. The clinicopathologic similarities between EPDS and PPG suggest that EPDS should be included in the spectrum of autoinflammatory dermatoses. Clinicians could consider the possibility of associated disorders rather than managing EPDS as a sui generis skin disorder. Full article