Bullous Systemic Lupus Erythematosus Successfully Treated With Intravenous Immunoglobulin and Mycophenolate Mofetil.

Bullous systemic lupus erythematosus (BSLE) is a rare autoimmune blistering disorder of cutaneous lupus erythematosus (CLE) that typically manifests as an acute vesiculobullous eruption in a patient with systemic lupus erythematosus (SLE). Also, it can rarely present as the initial clinical manifestation of SLE. There is no established US Food and Drug Administration (FDA) therapy for BSLE. We report a case of a 71-year-old Hispanic woman with SLE and lupus nephritis classes III and V who presented to the hospital with a worsening rash with painful, ruptured blisters involving the upper arms, chest, and back. Our patient did not respond to topical or systemic steroids but improved rapidly to combination therapy with intravenous immunoglobulin (IVIg) and mycophenolate mofetil (MMF).

Useful cytological confirmation of HPV 13 in lesional mucosa enhances diagnosis of focal epithelial hyperplasia.

An 11-year-old female presented with multiple oral lesions for several months. Histopathological findings suggested focal epithelial hyperplasia (FEH), also known as Heck disease. FEH is strongly associated with Human papillomavirus (HPV), especially genotypes 13 and 32. An oral swab of a mucosal lesion was subsequently obtained for cytology, immunohistochemistry and in situ hybridization. In addition, in situ hybridization and immunohistochemistry were also performed retrospectively on the biopsy specimen for correlation. The cytology specimen showed squamous cells with enlarged, slightly atypical nuclei and rare perinuclear halos. The histology findings included papillomatosis with acanthosis, mild nuclear atypia and focal perinuclear halos. The immunohistochemistry for the consensus HPV L1 capsid protein was found in both the cytology and biopsy specimens indicating that the lesion was HPV-related. High viral copy numbers of HPV 13 were detected by in situ hybridization in both the cytology and histology specimens. Although histologic features of FEH have been well characterized in the literature, to our knowledge, this is the first case to describe in FEH with adjunct immunohistochemistry and in situ hybridization results. Furthermore, these findings assisted in our diagnosis since the patient's clinical presentation was a diagnostic challenge with smooth dome-shaped papules instead of the typically described flat-topped verrucous lesions seen in FEH. In summary, our case reveals that there is a high concordance between the HPV 13 detection in the cytology and histology of FEH, and that performing cytology in addition to histology can be used to optimize diagnostic evaluation towards appropriate patient care.

HPV-57 Verruca Vulgaris Mimicking Epidermodysplasia Verruciformis.

ABSTRACT: Human papilloma virus (HPV) is the causative agent for a variety of cutaneous lesions including verruca vulgaris (VV) and epidermodysplasia verruciformis (EDV). There are more than 200 known genotypes of HPV, and specific HPV types are associated with different clinical manifestations and malignant potentials. Herein, we describe a case of a 43-year-old immunocompetent woman who presented with morphologically distinct lesions that were most consistent with EDV on clinical examination. However, further histopathological and viral analysis confirmed the lesions as HPV-57-positive VV. The risk of malignant transformation, and therefore treatment and surveillance, is dramatically different in VV versus EDV. Therefore, this case highlights the importance of a proper histopathological diagnosis with HPV viral testing when clinical presentations may vary or mimic other diseases.

Atypical Subacute Cutaneous Lupus in a Patient on Apixaban Anticoagulation Therapy: A Case Report and Review of the Literature.

Subacute cutaneous lupus erythematosus (SCLE) is a rare cutaneous lupus erythematosus (CLE) subtype manifesting in middle-aged Caucasians with photo-distributed papulosquamous or annular lesions. Drug-induced SCLE (DI-SCLE) forms present in a similar manner but direct oral anticoagulants are rarely implicated. We report an unusual case of SCLE in a 37-year-old African American patient with a history of unprovoked deep vein thromboses (DVT) who presented with new-onset photoprotected polymorphic lesions two months after the initiation of apixaban anticoagulation therapy. Our case demonstrates the heterogeneous nature of SCLE presentation and highlights the possibility of apixaban as a potential causative agent of DI-SCLE in immunogenetically susceptible individuals. Moreover, we hypothesize on the etiopathogenesis of our patient's atypical presentation.

Langerhans cell histiocytosis: A great imitator.

Langerhans cell histiocytosis (LCH) is an uncommon but serious inflammatory neoplasia that affects many organs, including the skin. Though uncommon, it should remain high on a clinician's differential diagnosis in treatment-resistant cases of conditions, such as seborrheic dermatitis, diaper dermatitis, arthropod bites, and many more. A thorough history nd physical examination for each patient can aid in the diagnosis; however, if clinically suspicious for LCH, a punch biopsy should be performed. Histologic evaluation of LCH is often enough to differentiate it from the many clinical mimickers. Characteristic findings include a histiocytic infiltrate with "coffee bean"-cleaved nuclei, rounded shape, and eosinophilic cytoplasm. Immunohistochemical stains, including CD1a, S100, and CD207 (langerin) are often needed for a definitive diagnosis. Electron microscopy also demonstrates the ultrastructural presence of Birbeck granules, but this is no longer needed due to immunohistochemical staining. Treatment is often necessary for LCH, if systemic involvement exists.

A Helpful Clue to Calciphylaxis: Subcutaneous Pseudoxanthoma Elasticum-like Changes.

Calciphylaxis, otherwise known as calcific uremic arteriolopathy, is an aggressive disease characterized by painful, ischemic skin lesions with histologic findings of microvascular calcification involving the fat. It is most commonly seen in patients with end-stage renal disease who are on dialysis. Early diagnosis is pivotal for optimal management. However, calciphylaxis can be challenging to diagnose, as many diseases can bear a clinical resemblance to calciphylaxis. Skin biopsies are often necessitated for diagnosis, but unfortunately, one is commonly challenged with a suboptimal amount of subcutaneous fat that does not always show the classic findings of microvascular calcification. However, when calciphylaxis is clinically suspected, the microscopic detection of subcutaneous pseudoxanthoma elasticum (PXE)-like changes, although not unequivocally diagnostic of calciphylaxis, can be a helpful clue in the diagnosis. We report a case of a 49-year-old woman who was diagnosed with uremic calciphylaxis on the basis of the clinical scenario, laboratory testing, and microscopic presence of subcutaneous PXE-like changes that was highlighted with the elastic and von Kossa stains. This case demonstrates the importance of recognizing subcutaneous PXE-like changes on suboptimal skin biopsies that lack microvascular calcification, especially in those patients who are clinically suspect for calciphylaxis as to prevent delay in diagnosis and treatment.

A rare case of acral amelanotic melanoma, nodular type.

Amelanotic melanoma is a rare melanoma subtype, and it is even more rare when it occurs at an acral site. We here present a case of a nodular amelanotic acral melanoma (NAAM) occurring on the heel of an 83-year old female. It presented as an ulcerated pink nodular growth on the heel, which clinically mimicked other nodular non-pigmented lesions, causing a delay in diagnosis until it was biopsied. This case is a demonstration of the critical importance to include NAAM in the differential diagnosis of nodular non-pigmented skin lesions as to avoid delay in diagnosis and disease progression, in which early detection can provide the most modifiable prognostic factor.

The great impostor: Lues maligna in an HIV-infected male.

Lues maligna is a rare severe cutaneous manifestation of secondary syphilis. It is also known as malignant syphilis and ulceronodular syphilis. We report a case of a 58-year-old HIV-infected male who presented with diffuse, pruritic, non-tender, maculo-papular skin lesions, ulcerated nodules and plaques surrounded by an erythematous base. The disseminated skin lesions were at various stages and were located on his back, chest, arms and testicles. Patient had been receiving antiretroviral therapy. Laboratory studies had demonstrated CD4 lymphocyte count of 463 cells/mm3 and an undetectable HIV viral load. Workup revealed a rapid plasma reagin of 1:256 dilutions and the skin biopsy findings were compatible with syphilis. The skin lesions resolved with intramuscular penicillin. We herein describe a rare case of lues maligna in an HIV-infected patient with a preserved immune function and viral suppression. Such skin lesions can mimic fungal or mycobacterial infections and can pose a diagnostic challenge. Even in the modern era, syphilis remains the great impostor. Clinicians must be able to recognize this condition based on clinical characteristics and risk factors to diagnose and treat this condition promptly.

Tumid lupus: An unexpected diagnosis for the otolaryngologist.

Tumid lupus is an uncommon variant of lupus erythematosus. Patients present with subcutaneous lesions. Ophthalmic literature reports disease manifestation as orbital inflammation. Autoimmune serology is often negative. Without a high index of suspicion, the diagnosis is easily overlooked delaying treatment. Tumid lupus is not significantly discussed in the Otolaryngologic literature. Here we present a Case Report of a male who initially presented to Ophthalmology with unilateral orbital complaint of eyelid puffiness. Orbital biopsy and subsequent biopsy of his submental skin lesions ultimately led to this unexpected diagnosis. We discuss the method to diagnose tumid lupus including representative histopathologic findings.

Pemphigus vegetans with coexistent herpes simplex infection and deep venous thrombosis of the lower extremities.

An elderly patient presented with a 4-month history of eroded hypertrophic condylomatous plaques with areas of vesiculation on the groin and lower extremities bilaterally, associated with swelling. Biopsy and immunofluorescence studies confirmed the diagnosis of pemphigus vegetans (PVeg). Further clinical evaluation revealed deep venous thrombosis of the lower extremities and septic shock from secondarily infected pemphigus lesions. Fluid obtained from vesicles was positive for herpes simplex virus (HSV) via PCR-based testing. The patient was therapeutically anticoagulated, treated with high-dose corticosteroid therapy, broad-spectrum intravenous antibiotics and acyclovir. This case represents a constellation of diagnoses not previously described. Although pemphigus vulgaris has been linked to thromboembolic events and has been associated with HSV, these associations have not been previously reported in PVeg. The coexistence of these diseases should encourage vigilance in the clinical work up of a patient with PVeg.