Graham-Little-Piccardi-Lasseur Syndrome: A Case Report.

Graham-Little Piccardi-Lasseur syndrome is a rare dermatosis that affects the hair follicles throughout the body and often presents with a progressive cicatricial alopecia of the scalp that is unresponsive to medical therapy. While treatment options are limited, prompt recognition through a careful physical exam aided by dermoscopy can facilitate early intervention. Here we present a patient with GLPLS, discuss pertinent morphologic and dermoscopic findings, and review the current literature. J Drugs Dermatol. 2022;22(2):210-216. doi:10.36849/JDD.6926.

Acute Onset Linear Lichen Planus Pigmentosus of the Forehead: A Case Series.

Linear lichen planus pigmentosus (LPP) of the face is a rare variant of lichen planus, with only a few cases published in the literature.1 It is an inflammatory condition with unknown etiology, characterized by blue-gray hyperpigmented macules, and tends to affect sun-exposed areas of the head and neck.1-4 The pathophysiology of linear lichen pigmentosus is poorly understood, though it is postulated to be caused by T-lymphocyte autoimmunity against keratinocytes.5-7 LPP more frequently affects middle age woman and skin phototypes III-VI.1,3 Treatment for linear LPP is difficult and there is no established first-line therapy; however, tacrolimus ointment, topical corticosteroids, and various systemic agents have shown to be effective in improving the appearance.3,8,9 Prior reports have characterized linear LPP that follows the lines of Blashko as more commonly affecting the trunk.1 We present three cases of linear lichen planus pigmentosus (LPP) of the forehead, a unique novel presentation of linear LPP of the face. One of our cases also provides supporting evidence for tacrolimus to be used as a preferred therapy to treat linear LPP of the face; however, more research is needed to support this claim. To our knowledge, this case series is the largest case series of linear lichen planus pigmentosus (LPP) of the forehead to be reported. J Drugs Dermatol. 2023;22(1):94-97. doi:10.36849/JDD.7200.

Clinical and histopathological spectrum of delayed adverse cutaneous reactions following COVID-19 vaccination.

BACKGROUND: As more people become vaccinated against the SARS-CoV-2 virus, reports of delayed cutaneous hypersensitivity reactions are beginning to emerge. METHODS: In this IRB-approved retrospective case series, biopsy specimens of potential cutaneous adverse reactions from the Pfizer-BioNTech or Moderna mRNA vaccine were identified and reviewed. Clinical information was obtained through the requisition form, referring clinician, or medical chart review. RESULTS: Twelve cases were included. Histopathological features from two injection-site reactions showed a mixed-cell infiltrate with eosinophils and a spongiotic dermatitis with eosinophils. Three biopsy specimens came from generalized eruptions that showed interface changes consistent with an exanthematous drug reaction. Three biopsy specimens revealed a predominantly spongiotic pattern, consistent with eczematous dermatitis. Small-vessel vascular injury was seen in two specimens, which were diagnosed as urticarial vasculitis and leukocytoclastic vasculitis, respectively. There were two cases of new-onset bullous pemphigoid supported by histopathological examination and direct immunofluorescence studies. Eosinophils were seen in 10 cases. CONCLUSIONS: Dermatopathologists should be aware of potential cutaneous adverse reactions to mRNA-based COVID-19 vaccines. Histopathological patterns include mixed-cell infiltrates, epidermal spongiosis, and interface changes. Eosinophils are a common finding but are not always present. Direct immunofluorescence studies may be helpful for immune-mediated cutaneous presentations such as vasculitis or bullous pemphigoid.

A case of pleomorphic dermal sarcoma with perineural invasion treated with Mohs micrographic surgery and adjuvant radiation therapy.

Pleomorphic dermal sarcoma (PDS) was recognized in the 2013 World Health Organization Classification of Tumors of Soft Tissue and Bone as a clinical entity with adverse histopathologic features compared to the more superficial and less aggressive atypical fibroxanthoma (AFX). Although the gold standard treatment of AFX is Mohs micrographic surgery (MMS), the optimal treatment for PDS has yet to be determined. We report the case of a 71-year-old man with a PDS with perineural invasion on the scalp, with no recurrence one-year post-treatment with MMS and adjuvant radiation therapy. In contrast to wide local excision, MMS offers complete margin control and tissue preservation, which helps minimize scarring and morbidity. The comparative efficacy of MMS versus wide local excision in the treatment of PDS with and without radiation remains unknown and warrants further investigation.

Histopathologic reaction patterns to differentially cross-linked hyaluronic acid fillers: A retrospective case series.

BACKGROUND: Hyaluronic acid filler reactions have been increasingly observed in recent years. Our study investigates whether the increased number of filler reactions observed since 2014 is associated with a specific histopathologic inflammatory pattern and type of filler. METHODS: The institution's dermatopathology electronic database was retrospectively searched for histopathologic reactions to hyaluronic acid from January 2014 to December 2019. The age, sex, type of filler, procedure, location, and histopathologic patterns were recorded. RESULTS: From 2014 to 2019, there were 15 cases of granulomatous reactions to hyaluronic acid filler. In 10 of these cases, there was a characteristic inflammatory pattern characterized by tightly cuffed palisades of histiocytes with varying numbers of eosinophils. Of the 11 cases in which the type of filler was known, all used Vycross technology, a novel manufacturing process in the production of hyaluronic acid filler. CONCLUSION: A characteristic histopathologic pattern of discrete foci of tightly cuffed palisaded granulomas with eosinophils is associated with fillers manufactured using Vycross technology.

The "bumpy" adolescent nose: Acne associated angiofibroma-like nasal papules.

BACKGROUND/OBJECTIVE: Papular scars are a recently described clinical phenotype of acne scarring characterized by papules occurring on the nose and chin. We have observed a similar presentation of nasal papules among patients seen in our clinic for acne and sought to further characterize the clinical and histopathological characteristics of this entity. METHODS: In this single-site case series, a retrospective review of electronic medical records of patients with nasal papules in association with acne vulgaris between April 2018 and April 2019 was performed. Clinical and histopathologic findings were recorded. RESULTS: We identified 20 patients who presented with a similar clinical phenotype of predominantly skin-colored, dome-shaped papules concentrated on the nose and chin in association with a history of more classic facial acne vulgaris. Papular lesions were seen predominately in adolescent Hispanic males. Concomitant acne on other areas of the face was identified in 18 patients at presentation while two patients had a history of adolescent acne. Biopsies were performed for five patients. Histopathologic examination demonstrated features of fibrosis and dilated thin-walled blood vessels, typical of angiofibromas. CONCLUSION: We present a series of adolescent patients with large, flesh-colored to erythematous papules seen predominantly on the nose. These lesions are histologically indistinguishable from angiofibromas and may represent an under-recognized yet disfiguring sequela of acne that may disproportionately affect adolescents with skin of color.

Neutrophilic panniculitis arising from hematogenous spread of methicillin-resistant Staphylococcus aureus.

Infectious panniculitis from hematogenous spread is uncommon and usually occurs in immunocompromised patients. Dissemination of gram-positive organisms to the subcutaneous tissue is rare with only several reports of disseminated panniculitis caused by Streptococcal species. We report a case of an immunocompetent 2-year-old boy presenting with diffuse neutrophilic panniculitis arising from methicillin-resistant Staphylococcus aureus septicemia. This case represents a highly atypical manifestation of severe MRSA infection and serves as a reminder to consider MRSA as a cause of disseminated neutrophilic panniculitis, particularly in high-risk populations.

Learning Analytics to Enhance Dermatopathology Education Among Dermatology Residents

BACKGROUND: With the advent of digital microscopy, learning analytics can be leveraged to advance teaching of dermatopathology in dermatology residency. OBJECTIVE: To analyze the acquisition and decay of dermatopathology visual recognition skills and areas of diagnostic confusion amongst residents using learning metrics generated by a web-based learning tool. METHODS: This was a prospective, longitudinal study of dermatology residents who studied digital photomicrographs of 18 routine diagnostic entities using an online software platform. Residents at different years of training were given 60 minutes to complete assessments on three occasions (initial test with follow-ups at one and three months). RESULTS: 4,938 responses were analyzed. Accuracy and time to diagnosis improved within each assessment and between the first and second assessments. First year residents showed knowledge decay when tested at three months (67% vs 64%; P=0.002) while third year residents retained knowledge and continued to improve upon their accuracy after three months (83% to 91%, <0.001). Learning analytics highlighted diagnostically challenging cases for residents that contradicted experts' predictions (R=0.48). CONCLUSIONS: The use of learning analytics and interactive digital platforms enhances dermatopathology curriculum development by identifying challenging diagnostic entities, assessing mastery of subject material, and optimizing review schedules. J Drugs Dermatol. 2019;18(12):1231-1236.

Collapsing Glomerulopathy in Lambda Light Chain Amyloidosis: A Report of 2 Cases.

Amyloid nephropathy is an uncommon disease that frequently presents with reduced kidney function and proteinuria and, in developed nations, is most often associated with underlying paraproteinemia. The histologic appearance of glomerular amyloid deposition includes mesangial and capillary wall infiltration by an amorphous eosinophilic material, and features of endo- or extracapillary proliferation are not typically seen. Rare cases of crescentic injury have been reported in a subset of patients with amyloid nephropathy, particularly those with amyloid derived from serum amyloid A protein. Collapsing glomerulopathy, which like crescentic injury is associated with an extracapillary proliferation, has not to our knowledge been reported in the setting of amyloid nephropathy. We report 2 patients presenting with acute kidney injury and nephrotic syndrome found to have amyloid nephropathy with prominent epithelial cell hyperplasia and glomerular collapse on biopsy. This injury is likely multifactorial and related to direct podocyte injury and vascular compromise and expands further the spectrum of paraprotein-associated renal injury.

Evaluation of MITF, SOX10, MART-1, and R21 Immunostaining for the Diagnosis of Residual Melanoma In Situ on Chronically Sun-Damaged Skin.

BACKGROUND: Melanocytic immunostains can assist in margin evaluation of melanoma in situ (MIS) excisions; however, their accuracy and reliability relative to hematoxylin & eosin (H&E) is yet to be determined. OBJECTIVE: The objective of this study was to evaluate the sensitivity, specificity, and concordance of 4 melanocyte-specific immunostains for diagnosing MIS occurring on chronically sun-damaged skin. MATERIALS AND METHODS: Serial permanent sections from representative areas of negative margin and residual tumor were stained using H&E, MITF, MART-1, SOX10, and R21 and examined in a blinded fashion. The study set included 100 digital microscopy images from 10 cases of MIS excisions from the face. Two board-certified dermatopathologists, 4 fellowship-trained Mohs surgeons, 2 Mohs fellows, and 2 dermatology residents independently reviewed the 100 images. RESULTS: The average melanocyte density was 11 versus 28 melanocytes per 0.5 mm for chronically sun-damaged skin versus residual MIS on H&E, respectively. Statistically significantly higher melanocyte densities were observed using MITF, MART-1, and SOX10 on negative margins. The sensitivity and interobserver concordance was highest using MITF and SOX10. The intraobserver agreement on 4 duplicate images was 85%. CONCLUSION: In conclusion, the nuclear immunostains (MITF and SOX10) show the most promise for improving the diagnosis of MIS in chronically sun-damaged skin.

Margin Assessment for Punch and Shave Biopsies of Dysplastic Nevi.

INTRODUCTION: Biopsies of atypical melanocytic nevi are among the most commonly performed procedures by dermatologists. Margin assessment is often used to guide re-excision, but can be a point of confusion as negative margins reported in the planes of sections examined do not always reflect complete removal of a lesion. This study investigates the rates of false negative margins after both punch and shave biopsies. METHODS: We performed a retrospective analysis of 50 consecutive punch and shave biopsy specimens (1) diagnosed as DN, and (2) reported as having clear margins in the planes of section examined. Identified specimen blocks were then sectioned through to examine true margin involvement. RESULTS: Of the 50 specimens identified, 20% (n = 10) were found to have positive margins upon additional sectioning. We found no difference between the groups with respect to biopsy technique, type of nevus, degree of atypia, or gender. CONCLUSION: This study observed false negative peripheral margin status in a sizeable proportion of biopsy specimens, which did not vary significantly based on biopsy technique or pathologic characteristics. This finding reflects a limitation of standard tissue processing, in which a limited proportion of the true margin is evaluated, and may be of note to many dermatologists who base their decision to re-excise on the reporting of margin involvement. J Drugs Dermatol. 2018;17(7):810-812.

Neutrophilic urticarial dermatosis as a presenting feature of systemic juvenile idiopathic arthritis.

This report describes a case of chronic neutrophilic urticarial dermatosis as a presenting feature of systemic juvenile idiopathic arthritis. When encountered in children, neutrophilic urticarial dermatosis should raise suspicion of autoimmune or autoinflammatory disease.

Folliculotropic mycosis fungoides.

Folliculotropic mycosis fungoides (MF) is a distinct subset of cutaneous T cell lymphoma (CTCL). The disease is typically marked by an aggressive course and is often recalcitrant to skin-direct therapy. We report a case of an 83-year-old woman with folliculotropic MF characterized by erythematous, scaly plaques on the forehead along with poliosis and alopecia of the right medial eyebrow.

Lichen planus pigmentosus.

Lichen planus pigmentosus (LPP) is an uncommon variant of lichen planus of unclear etiology that predominantly affects patients of skin types III to VI. We report a case of LPP of two years duration in a 67-year-old man involving upper extremities, chest, abdomen, and upper back.

Necrobiosis lipoidica.

We present a case of necrobiosis lipoidica (NL) of the right abdomen in a 75-year-old man. A skin biopsy performed showed a layered infiltrate of mono and multinucleated histiocytes palisaded around degenerated collagen bundles. Laboratory workup was unremarkable. The patient was treated with topical corticosteroids with cessation of progression of his disease, although the eruption did not resolve. There are a number of treatments for NL reported in the literature, all with varying efficacy. Although NL lesions are usually asymptomatic, patients with NL must be monitored closely for signs of ulceration or malignant transformation, in which case more aggressive treatment options may be warranted.

Generalized essential telangiectasia.

The pathophysiology of generalized essential telangiectasia is not well understood. Generalized essential telangiectasia is an uncommon disorder in which widespread telangiectasias of unknown cause develop without associated systemic or antecedent dermatologic disease. We report a case of generalized essential telangiectasia in an otherwise healthy 49-year-old man.

Lymphomatoid papulosis.

Lymphomatoid papulosis is often regarded as a low-grade variant of cutaneous T cell lymphoma (CTCL). Given the excellent long-term prognosis, recent consensus guidelines indicate that patients can be monitored off therapy. We report a case of a 67-year-old man who presented with lymphomatoid papulosis, with necrotic papules that have been intermittently present for over forty years.