Dermatopathology, Volume 12, Issue 1 (March 2025) – 8 articles

Hypertrophic lichen planus (HLP) is a chronic inflammatory skin condition defined by verrucous, pruritic, papules and plaques usually affecting the lower limbs. The diagnosis of HLP is primarily clinical. However, due to its feasible generalized presentation and similarities with other hypertrophic cutaneous disorders, histological evaluation is often necessary. Many dermatological conditions that present with a hypertrophic clinical appearance can arise from a histological lichenoid infiltrate (HCLI). Hence, we provide an overview of the clinical, histopathological, and prognostic features of selected HCLI, including HLP, hypertrophic lichenoid dermatitis, hypertrophic lichen sclerosus (HLS), lichen simplex chronicus (LSC), squamous cell carcinoma (SCC), keratoacanthoma (KA), pseudoepitheliomatous hyperplasia (PEH), viral warts, and lupus erythematosus/lichen planus (LE/LP) overlap. Choosing the appropriate procedure and the anatomical site for an incisional biopsy requires thoughtful consideration to ensure sufficient depth and improve diagnostic accuracy by identifying the histological features specific to each hypertrophic condition. Full article

Kikuchi–Fujimoto disease (KFD) is a rare condition characterized by necrotizing lymphadenitis and fever, often associated with immune dysregulation. Histologically, it features necrotic foci with abundant histiocytes and plasmacytoid dendritic cells but notably lacks neutrophils and eosinophils. Recent evidence reveals a notable prevalence among pediatric patients, who may exhibit distinct features compared to adults. We reported the case of an 11-year-old girl presenting with persistent fever, cervical adenopathy, and a malar rash, leading to a diagnosis of KFD following lymph node biopsy, which revealed non-suppurative necrosis and histiocytic infiltration. Empirical treatment with antivirals and antibiotics was ineffective, but corticosteroid therapy achieved symptom remission. A literature review identified 48 relevant studies involving 386 pediatric cases, with histopathological findings consistent with classical descriptions of KFD. Cutaneous involvement was reported in 11.14% of cases, ranging from maculopapular rashes to lupus-like eruptions. Notable complications included the development of systemic lupus erythematous, Sjögren syndrome, and rare instances of hemophagocytic syndrome or central nervous system involvement. Kikuchi–Fujimoto disease should be considered in the differential diagnosis of pediatric patients presenting with fever and lymphadenopathy, taking into account a higher frequency of cutaneous manifestations in pediatric cases. A skin biopsy may be helpful in diagnosing KFD and provide valuable information regarding the potential risk of developing systemic lupus erythematosus in the future. Full article

Based on histologic and genetic patterns, the current World Health Organization (WHO) classification distinguishes six subtypes of lymphomatoid papulosis (Lyp). The aim of our article was to analyze the frequency of histologic and immunohistochemical features of different Lyp subtypes reported in the literature. We used PubMed advanced search builder to systematically review and evaluate English and German literature of Lyp from 1968 to April 2022. We considered only papers in which histopathologic features were mentioned in detail. We identified 48 publications with a total of 518 cases. The diagnoses were based on the diagnostic criteria at the time of publication. In Lyp A and Lyp B a CD8+ phenotype was more often reported than expected (53% and 52%, respectively). A double positive phenotype (CD4+/CD8+) was found in 28% of Lyp E and a double negative (CD4-/CD8-) in 50% of Lyp with 6p25.3 rearrangement. High rates of folliculo- and syringotropism were reported in both Lyp A and B. Surprisingly, strong epidermotropism occurred in 20/38 (53%) cases reported as Lyp B and in 43/64 (67%) of Lyp D cases. The predominating phenotype in Lyp D was CD8+, while TIA-1/granzymeB/perforin expression was reported in 37/46 (80%), and CD56 was expressed in 13/47 (28%) of the investigated cases. The limitation of the data is due to the retrospective approach with diagnostic criteria changing over time and on a case selection in some publications. However, the data indicate that the Lyp subtypes overlap more than assumed. They also show that a prospective study is needed to obtain valid data on the frequency distribution of certain histopathologic criteria. Full article

Hyperalgesic priming is a model of the transition from acute to chronic pain. Whether a similar mechanism exists for “pruritic priming” of itch is unknown. Here, we tested the hypothesis that itchy skin in a commonly used mouse model of dry skin pruritus develops latent sensitization after resolution. Acetone–ether–water (AEW) treatment induced a dry and itchy skin condition in the mouse cheek that elicited site-directed scratching behavior. After cessation of treatment and the complete resolution of AEW-induced scratching, histaminergic and non-histaminergic pruritogens were administered to the cheek to test for altered site-directed scratching and wiping behavior. Each pruritogen was also tested following the resolution of carrageenan-induced nociceptor hypersensitivity to test for cross-modality priming. Peak AEW-induced scratching occurred 24 h after the final day of treatment, and 5 days were required for scratching levels to return to baseline. Likewise, epidermal thickening was the greatest on the final treatment day and completely returned to baseline after 5 days. After the resolution of itchy cheek skin, acute histamine- and non-histamine-evoked scratching and wiping behaviors were unchanged, nor were scratching and wiping behaviors to acute pruritogens altered after the resolution of carrageenan-induced hypersensitivity. The results indicate that persistent itch due to dry skin likely resolves completely, without producing a latent primed response to subsequent pruritic stimuli. We conclude that the mechanisms regulating hyperalgesic priming are likely distinct from pruritic signaling in the dry and itchy skin model. Full article

Cutaneous silica granulomas are a form of foreign-body granulomatous reactions. They are characterized histopathologically by sarcoidal granulomas in association with silica crystals. Asteroid bodies, a classical histopathological feature of sarcoidosis, have not previously been reported in association with silica granulomas. Herein, we present the case of an 83-year-old man with an asymptomatic papule on the vertex scalp. Histopathology revealed a dermal granulomatous reaction to silica crystals. Asteroid bodies were observed in the cytoplasm of multinucleated giant cells. In the absence of systemic symptoms or laboratory findings suggestive of sarcoidosis, a final diagnosis of silica granuloma with asteroid bodies was made. While they have been observed in several other granulomatous reactions, the present case represents a novel association of asteroid bodies with silica granulomas. Full article

The diagnosis, interpretation, and classification of melanocytic tumors is a very complex topic in the pathology and dermatopathology field that lacks standardization and is still subject to discordance and debate. Here, we review the definitions of dysplastic nevus and superficial atypical melanocytic proliferations and provide an overview of some areas still subject to debate and some attempts of standardization. Furthermore, we describe an algorithmic classification, and provide some examples of clinico-pathological correlation. This step-by-step algorithm has an educational purpose and may automatize the work of dermatopathologists. We hope that through further molecular studies, this fine-grained scheme will prove to be related to the biological behavior of these atypical melanocytic lesions. Full article

We present the case of a 60-year-old immunocompromised man who presented with two pruritic pink–red indurated nodules with overlying scale and focal areas of ulceration on his left dorsal and left medial forearm, which evolved over a 2-month period. The pathology showed numerous fungal hyphae present that were pauci-septate with various branched angles and variable hyphal thickness. Fungal cultures grew Rhizopus species and a universal fungal PCR detected the Rhizopus oryzae complex. Based on the clinicopathologic correlation, the diagnosis of cutaneous mucormycosis was made. Cutaneous mucormycosis is an aggressive fungal infection of the Mucorales family occurring after the inoculation of fungal spores in disrupted skin. It usually presents as a necrotic eschar but can also present as cellulitis that evolves into a necrotic ulcer. A prompt diagnosis is critical for the effective management of cutaneous mucormycosis. The treatment includes an immediate systemic treatment with amphotericin B and a surgical debridement of the necrotic regions. Given the wide range of presenting symptoms, clinical suspicion for this emergent condition must remain high in immunocompromised and diabetic patients. Full article

Cutaneous squamomelanocytic tumor (SMT) is a very rare cutaneous malignancy, composed of a dual phenotypic population of both malignant melanocytes and keratinocytes, intimately intermingled together. Herein, we report a new case of a SMT occurring in an 82-year-old man, located on the scalp. Histopathology revealed a mixed population consisting of squamous cell carcinoma and melanoma within the same lesion, also confirmed using immunohistochemical staining for high molecular-weight cytokeratins (HMWCKs) and Melan-A. Moreover, to the best of our knowledge, for the first time, we tested SMT for the preferentially expressed antigen in melanoma (PRAME), which revealed a strong and diffuse positivity in the melanocytic component. These tumors need to be distinguished by more frequent collision tumors and colonization. Furthermore, we provide a comprehensive review of the literature, focusing on clinical and histopathological aspects, biological behavior and still-debated, but fascinating histogenesis of this elusive entity. Full article