Wieland, Carilyn N.

AbstractA 17‐year‐old Caucasian boy presented with progressive left‐sided weakness, transient slurred speech, and skin lesions characterized by 3‐5 mm, pink, asymptomatic papules with white atrophic centers on his central abdomen, back, and lower extremities. Skin biopsy confirmed the diagnosis of malignant atrophic papulosis, a rare vasculopathy that leads to the occlusion of small‐ and medium‐sized arteries. He was treated with cyclophosphamide, eculizumab, treprostinil, pentoxifylline, heparin, and acetylsalicylic acid. Despite the aggressive immunosuppression, humanized monoclonal antibodies, and antiplatelet therapy, he died two months after presentation. We report this case to highlight diagnostic features, as well as to highlight the importance of early diagnosis and treatment.

AbstractBackgroundCertain autoimmune bullous dermatoses are mediated by autoantibodies of the IgG4 subclass. We determined the diagnostic impact of adding IgG4 to our conventional direct immunofluorescence (DIF) panel.MethodsFor all cases submitted to our referral laboratory for DIF over 1 month (n = 630), we performed IgG4 testing and collected consecutive biopsy specimens showing definite or indeterminate linear or cell‐surface deposition of IgG, IgG4, and/or C3. On retrospective blinded review, we classified the pattern and whether the findings were definite, indeterminate, or negative. When present, substantial background staining was recorded.ResultsSeventy DIF specimens met the inclusion criteria. Of 22 (31.4%) specimens equivocal for linear or cell‐surface deposition, 9 (40.9%) had definitive IgG4 findings, either linear (3 of 14 equivocal linear cases; 21.4%) or cell‐surface (6 of 8 equivocal cell‐surface cases; 75.0%). Background deposition was substantial in 14 cases (20.0%) for IgG but in none for C3 or IgG4.ConclusionIgG4 allowed the classification of over 40% of DIF cases that were otherwise equivocal by IgG and C3. IgG4 staining showed lower levels of non‐specific background staining than IgG or C3. IgG4 appears to contribute most value in cases with cell‐surface deposition or with equivocal linear IgG deposition and negative C3 results.

AbstractSpitzoid melanocytic lesions describe a spectrum of pediatric melanocytic proliferations ranging from benign Spitz nevi to malignant spitzoid melanomas typically arising within the first two decades of life. Atypical spitzoid neoplasm (ASN) is a poorly defined category within this spectrum that poses a unique diagnostic challenge due to histologic findings with insufficient atypical characteristics to make the diagnosis of melanoma. This report presents an exceptionally rare case of an ulcerative atypical spitzoid neoplasm mimicking an infantile hemangioma in a two‐month‐old girl treated with pulse dyed laser (PDL) and surgical excision. Our patient ultimately underwent five excisions over a 2‐year period, with successful maintenance of function and dexterity of the affected fingers.

AbstractMucous membrane pemphigoid (MMP) is a rare chronic immunobullous disease that involves the mucous membranes and may result in significant scarring and complications if diagnosis is delayed. MMP typically occurs in elderly patients, with very few cases reported in children. Here, we present a 12‐year‐old female patient with childhood‐onset oral and genital MMP, clinically suspected to be lichen sclerosus, but eventually diagnosed as MMP after multiple supportive biopsies and confirmatory direct immunofluorescence. Although treatment was challenging, the combined use of systemic corticosteroids, dapsone, and mycophenolate mofetil was ultimately successful in achieving disease control.

AbstractA 10‐year‐old Guatemalan girl with past medical history of Epstein‐Barr virus‐associated smooth muscle tumors (EBV‐SMT) and combined immunodeficiency presented for evaluation of painful intraoral lesions. On examination, she was noted to have multiple, white to flesh‐colored, soft, flat‐topped papules, and plaques on the buccal and labial mucosa. Human papillomavirus type 13 was detected on PCR with PGMY primers of previously biopsied buccal tissue, confirming a diagnosis of Heck's disease (multifocal epithelial hyperplasia). We present an immunosuppressed, pediatric patient with two rare, virus‐associated neoplastic disorders that have not been previously reported to occur in the same individual.

AbstractBackgroundLichen planopilaris (LPP) is a scarring alopecia rarely described in men.ObjectiveTo investigate the clinical and histopathologic features of LPP in men.MethodsWe performed a retrospective cohort study of male patients with LPP seen at Mayo Clinic between 1992 and 2016.ResultsNineteen men with biopsy‐confirmed LPP were included. The disease most commonly presented with diffuse (42.1%) or vertex scalp (42.1%) involvement. None of the patients had eyebrow or body hair involvement. Perifollicular erythema (94.7%) and pruritus (57.9%) were the most frequent clinical findings. Androgenetic alopecia (AGA) co‐occurred in 26.3% of patients. Mucosal lichen planus was found in four patients (21.1%). Thyroid disease occurred in three patients (15.8%). Disease improvement (47.3%) occurred with combination topical and systemic therapy, topical clobetasol monotherapy, and minocycline monotherapy.ConclusionsLPP in men has similar clinical and histologic presentations as reported in women. Nonscalp hair loss appears less likely in men with classic LPP than reported in men with frontal fibrosing alopecia, while mucosal lichen planus and thyroid disease appear to be more common in classic LPP. Men with AGA can present with new‐onset concomitant LPP. Limitations included small study size, variable follow‐up, and lack of standardized clinical assessment due the study’s retrospective nature.