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Lupus miliaris disseminatus faciei (LMDF) is a chronic inflammatory dermatosis of unknown aetiology, most often seen in young adults. Although many treatments for LMDF exist, treatment guidelines have not been developed, and response to therapy is generally unpredictable. We present the results of transcriptomic analysis of LMDF lesional skin, which revealed a variety of differentially expressed genes linking LMDF to alterations in innate and adaptive T helper 1 immunity. Immunohistochemical analysis was also performed, identifying similar changes in T-cell immune responses. Given evidence for increased tumour necrosis factor (TNF) pathway activity, our patient, who had previously been refractory to multiple treatments, was initiated on TNF inhibitor therapy with excellent response. This characterization of the LMDF immune response may lead to improved treatment of this condition.

Fluorescence imitating brightfield imaging (FIBI) is a novel alternative microscopy method that can image freshly excised, non-sectioned tissue. We examine its potential utility in dermatopathology by examining readily available specimens embedded in paraffin blocks.Nine skin samples embedded in paraffin blocks were superficially deparaffinized using xylene and ethanol and stained with HE. FIBI captured tissue surface histopathology images using simple microscope optics and a color camera. We then applied deep-learning-based models to improve resemblance to standard HE coloration and contrast. FIBI images were compared with corresponding standard HE slides and concordance was assessed by two dermatopathologists who numerically scored epidermal and dermal structure appearance and overall diagnostic utility.Dermatopathologist scores indicate that FIBI images are at least equivalent to standard HE slides for visualizing structures such as epidermal layers, sweat glands, and nerves.Images acquired with FIBI are comparable to traditional HE-stained slides, suggesting that this rapid, inexpensive, and non-destructive microscopy technique is a conceivable alternative to standard histopathology processes especially for time-sensitive procedures and in settings with limited histopathology resources.

Syphilis is known as the “great imitator” for its varied clinical manifestations. The historically low rate of syphilis in the United States in the early 2000s has been followed by an epidemic of primary and secondary (P&S) infections, with a 71.4% increase between 2014 and 2018 (10.8 vs 6.3 cases per 100,000).1 In 2018, about 85.7% of all reported cases of P&S syphilis were observed in men, most of whom were men who have sex with men.1 Rates of P&S syphilis also increased by 30.4% in women between 2017 and 2018.1 In this case series, we describe 9 patients with condyloma lata (CL) seen in our tertiary academic medical center inpatient dermatology service between May 2015 and December 2018.

Pemphigus vulgaris (PV) is a rare immunobullous disease. Although it classically presents as generalized flaccid blisters affecting the skin and mucosae, atypical cases of PV can be diagnostically challenging. Herein, we report an underrecognized non-blistering manifestation of pemphigus vulgaris, which we call mounded and refractory keratoses (MARK). MARK presents as exuberant scaling plaques on the scalp, often in the skin of color. When MARK features are present, pemphigus vulgaris is prone to misdiagnosis, clinically and histopathologically, leading to delays in appropriate treatment. Specifically, biopsies from these patients may resemble acantholytic dyskeratosis, resulting in initial misdiagnosis. Thus, recognizing this presentation may aid physicians in diagnosing and monitoring the recurrence of pemphigus vulgaris.

Background Appropriate use criteria (AUC) provide patient-centered physician guidance in test selection. An initial set of AUC was reported by the American Society of Dermatopathology (ASDP) in 2018. AUC reflect evidence collected at single timepoints and may be affected by evolving evidence and experience. The objective of this study was to update and expand AUC for selected tests. Methods RAND/UCLA (RAND Corporation [Santa Monica, CA]/University of California Los Angeles) methodology used includes the following: (a) literature review; (b) review of previously rated tests and previously employed clinical scenarios; (c) selection of previously rated tests for new ratings; (d) development of new clinical scenarios; (e) selection of additional tests; (f) three rating rounds with feedback and group discussion after rounds 1 and 2. Results For 220 clinical scenarios comprising lymphoproliferative (light chain clonality), melanocytic (comparative genomic hybridization, fluorescence in situ hybridization, reverse transcription polymerase chain reaction, telomerase reverse transcriptase promoter), vascular disorders (MYC), and inflammatory dermatoses (periodic acid-Schiff, Gomori methenamine silver), consensus by panel raters was reached in 172 of 220 (78%) scenarios, with 103 of 148 (70%) rated "usually appropriate" or "rarely appropriate" and 45 of 148 (30%), "appropriateness uncertain." Limitations The study design only measures appropriateness. Cost, availability, test comparison, and additional clinical considerations are not measured. The possibility that the findings of this study may be influenced by the inherent biases of the dermatopathologists involved in the study cannot be excluded. Conclusions AUC are reported for selected diagnostic tests in clinical scenarios that occur in dermatopathology practice. Adhering to AUC may reduce inappropriate test utilization and improve healthcare delivery.

Orf virus causes a self-limited infection in humans that resolves without scarring within 6-12 weeks. However, lesions in the immunocompromised can be progressive and disfiguring. The lesions frequently recur after treatment. To our knowledge, there are eleven published cases of these infections. We propose the name orf progressiva to call attention to this progressive, treatment-resistant entity. We present a 43-year-old male ranch owner with a history of renal transplantation who contracted an orf infection from his lamb. The infection recurred despite attempts at debridement, but achieved near complete resolution after treatment with imiquimod and valacyclovir. The histologic findings of orf progressiva are identical to the early stages of classic orf infection and are characterized by epithelial hyperplasia, intracytoplasmic eosinophilic inclusions, and an edematous, vascular dermis. There is no standard treatment for orf progressiva. Surgical excision has frequently resulted in rapid reoccurrence. Topical therapies such as imiquimod and cidofovir cream in combination with excision have been successful in some cases. Acyclovir or valacyclovir with imiquimod has been reported to be effective. Two patients achieved cure with imiquimod alone. We summarize these cases to prompt recognition of orf progressiva as a distinct clinical entity that requires treatment.

We report a patient with new-onset ulcerated granuloma annulare with concomitant involvement of B-cell leukemia. A granuloma annulare-like eruption with concomitant B cell chronic lymphocytic leukemia involvement in the skin is extremely rare, as only three cases have been previously reported in the literature to our knowledge. Given the rarity of ulceration in conventional granuloma annulare, it is possible this finding may serve as a diagnostic clue for underlying malignancy.

Histologic clues that facilitate rapid diagnosis of morphea at scanning magnification have been described but not well studied. We examined 73 cases of morphea and 42 control cases to determine the sensitivity and specificity of a novel histopathological feature-"the line sign (LS)"-a prominent, straight interface between subcutis and adjacent collagen. The sensitivity of LS was shown to be the most sensitive feature among 4 other existing histopathological features. Its specificity, however, was not the highest among the other features and needs to be evaluated further in future studies to confirm the usefulness of LS as a diagnostic tool for morphea.

Traditional histology relies on processing and physically sectioning either frozen or formalin-fixed paraffin-embedded (FFPE) tissue into thin slices (typically 4-6 μm) prior to staining and viewing on a standard wide-field microscope. Microscopy using ultraviolet (UV) surface excitation (MUSE) represents a novel alternative microscopy method that works with UV excitation using oblique cis-illumination, which can generate high-quality images from the cut surface of fresh or fixed tissue after brief staining, with no requirement for fixation, embedding and histological sectioning of tissue specimens. We examined its potential utility in dermatopathology. Concordance between MUSE images and hematoxylin and eosin (H&E) slides was assessed by the scoring of MUSE images on their suitability for identifying 10 selected epidermal and dermal structures obtained from minimally fixed tissue, including stratum corneum, stratum granulosum, stratum spinosum, stratum basale, nerve, vasculature, collagen and elastin, sweat glands, adipose tissue and inflammatory cells, as well as 4 cases of basal cell carcinoma and 1 case of pseudoxanthoma elasticum deparaffinized out of histology blocks. Our results indicate that MUSE can identify nearly all normal skin structures seen on routine H&E as well as some histopathologic features, and appears promising as a fast, reliable and cost-effective diagnostic approach in dermatopathology.

Multiple myeloma (MM) is an uncommon malignancy that involves proliferation of atypical plasma cells in the bone marrow, generally diagnosed in the seventh decade of life.1 Initial clinical signs and symptoms associated with MM are variable but usually include bone pain, anemia, hypercalcemia, and renal insufficiency. It is estimated that approximately 10% to 15% of patients with MM have concomitant immunoglobulin amyloid light-chain (AL) amyloidosis2, 3; of those patients, 29% to 40% have mucocutaneous disease.4 Here, we report a case of isolated nodules on the bilateral oral commissures as an unusual presenting sign of systemic AL amyloidosis, uncovering a diagnosis of MM.

Background Psoriasis is usually diagnosed clinically, so only non-classic or refractory cases tend to be biopsied. Diagnostic uncertainty persists when dermatopathologists encounter features regarded as non-classic for psoriasis. Objective Define and document classic and non-classic histologic features in skin biopsies from patients with clinically confirmed psoriasis. Methods Minimal clinical diagnostic criteria were informally validated and applied to a consecutive series of biopsies histologically consistent with psoriasis. Clinical confirmation required 2 of the following criteria: (1) classic morphology, (2) classic distribution, (3) nail pitting, and (4) family history, with #1 and/or #2 as 1 criterion in every case Results Fifty-one biopsies from 46 patients were examined. Classic features of psoriasis included hypogranulosis (96%), club-shaped rete ridges (96%), dermal papilla capillary ectasia (90%), Munro microabscess (78%), suprapapillary plate thinning (63%), spongiform pustules (53%), and regular acanthosis (14%). Non-classic features included irregular acanthosis (84%), junctional vacuolar alteration (76%), spongiosis (76%), dermal neutrophils (69%), necrotic keratinocytes (67%), hypergranulosis (65%), neutrophilic spongiosis (61%), dermal eosinophils (49%), compact orthokeratosis (37%), papillary dermal fibrosis (35%), lichenoid infiltrate (25%), plasma cells (16%), and eosinophilic spongiosis (8%). Conclusions Psoriasis exhibits a broader histopathologic spectrum. The presence of some non-classic features does not necessarily exclude the possibility of psoriasis.

Catastrophic antiphospholipid syndrome (CAPS) is a rare but life-threatening disease entity characterized by widespread vascular thromboses causing multiorgan failure in the setting of antiphospholipid antibody positivity.1 CAPS is the most severe subset of antiphospholipid syndrome (APS), an autoimmune clotting disorder in which the body attacks its own phospholipids. CAPS can present with a variety of pseudovasculitic skin findings. We report on a CAPS patient with tumid lupus erythematosus (LE)-like lesions.

We report a patient with new-onset ulcerated granuloma annulare with concomitant involvement of B-cell leukemia. A granuloma annulare-like eruption with concomitant B cell chronic lymphocytic leukemia involvement in the skin is extremely rare, as only three cases have been previously reported in the literature to our knowledge. Given the rarity of ulceration in conventional granuloma annulare, it is possible this finding may serve as a diagnostic clue for underlying malignancy.

Porokeratosis ptychotropica is a rare and commonly misdiagnosed subtype of porokeratosis involving the body folds. We present a 53-year-old man with systemic mastocytosis who presented with a pruritic, verrucous plaque in the gluteal fold that showed multiple cornoid lamellae on histopathologic evaluation, diagnostic of porokeratosis ptychotropica. Various treatments have been reported, including topical corticosteroids, retinoids, vitamin D analogs, calcineurin inhibitors, imiquimod, phototherapy, cryotherapy, or ablative laser therapy, but recurrences are common.

Background Dietary interventions are implicated in the development of atopic dermatitis, psoriasis, and acne. Objective To investigate the effect of diet and the bile acid (BA) receptors, such as TGR5 (Takeda G protein receptor 5) and S1PR2 (sphingosine-1-phosphate receptor 2) in the development of dermatitis. Methods C57BL/6 mice were fed a control diet (CD) or Western diet (WD) since weaning until they were 10 months old followed by analyzing histology, gene expression, and BA profiling. Results Mice developed dermatitis as they aged and the incidence was higher in females than males. Additionally, WD intake substantially increased the incidence of dermatitis. Cutaneous antimicrobial peptide genesS100A8, S100A9, and Defb4 were reduced in WD-fed mice, but increased when mice developed skin lesions. In addition, Tgr5 and TGR5-regulated Dio2 and Nos3 were reduced in WD intake but induced in dermatitic lesions. Trpa1 and Trpv1, which mediate itch, were also increased in dermatitic lesions. The expression of S1pr2 and genes encoding sphingosine kinases, S1P phosphatases, binding protein, and transporter were all reduced by WD intake but elevated in dermatitic lesions. Furthermore, dermatitis development increased total cutaneous BA with an altered profile, which may change TGR5 and S1PR2 activity. Moreover, supplementation with BA sequestrant cholestyramine reduced epidermal thickening as well as cutaneous inflammatory cytokines. Conclusion In summary, activation of TGR5 and S1PR2, which regulate itch, keratinocyte proliferation, metabolism, and inflammation, may contribute to WD-exacerbated dermatitis with Th2 and Th17 features. In addition, elevated total BA play a significant role in inducing dermatitis and cutaneous inflammation.

Background Acantholytic squamous cell carcinoma (aSCC) is regarded as a high-risk variant of cutaneous squamous cell carcinoma (SCC). Acantholytic actinic keratosis (aAK) has been regarded as a precursor risk factor for aSCC. However, supporting evidence is limited. Objective We sought to document clinical features, histologic features, management, and outcomes in a series of aSCC cases. Methods Definitions of aSCC, aAK, and aSCC arising in association with aAK were applied to a consecutive series of aSCC cases. Clinical characteristics and outcomes were obtained from electronic medical records. Results Of 115 aSCC cases (103 patients, mean age 71.8 years), actinic keratosis was present in 23% (27/115) but only 7.8% (9/115) exhibited associated aAK. Ten cases (10/115, 9%) fulfilled strict histologic criteria for follicular SCC as previously defined, but 50 of 115 (43%) of our aSCC cases exhibited predominant involvement of follicular epithelium rather than epidermis. Clinical outcome (median follow-up, 36 months) was available in 106 of 115 (92%). One patient experienced regional extension (parotid), and 1 patient experienced a local recurrence (nose). No disease-related metastases or deaths were documented. Limitations This was a single-institution retrospective study from the United States. Conclusions The presence of acantholysis in cutaneous SCC does not specifically confer aggressive behavior, a finding that may inform clinical practice guidelines.