Gene H. Kim

Epidermolysis bullosa acquisita: Autoimmunity to anchoring fibril collagen

Epidermolysis bullosa acquisita (EBA) is a rare and acquired autoimmune subepidermal bullous disease of skin and mucosa. EBA includes various distinct clinical manifestations resembling genetic dystrophic epidermolysis bullosa (DEB), Bullous pemphigus, Brunsting–Perry pemphigoid, or cicatricial pemphigoid. These patients have autoantibodies against type VII collagen (C7), an integral component of anchoring fibrils (AFs), which are responsible for attaching the dermis to the epidermis. Destruction or perturbation of the normal functioning AFs clinically results in skin fragility, blisters, erosions, scars, milia, and nail loss, all features reminiscent of genetic dystrophic epidermolysis bullosa. These anti-C7 antibodies are “pathogenic” because when injected into a mouse, the mouse develops an EBA-like blistering disease. Currently, treatment is often unsatisfactory; however, some success has been achieved with colchicine, dapsone, photopheresis, plasmapheresis, infliximab, rituximab, and IVIG.

Successful Treatment of Bullous Pemphigoid With Omalizumab

Bullous pemphigoid (BP) is an acquired, autoimmune, bullous disease that is characterized by autoantibodies against the 230-kDa bullous pemphigoid antigen within basal keratinocytes and the 180-kDa type XVII collagen within the basement membrane zone (BMZ) lying between the epidermis and dermis. In addition to skin blisters, patients with BP often experience pruritus and erythematous urticaria-like skin lesions. IgG is usually the predominant autoantibody in the plasma and skin of patients with BP. Nevertheless, most of these patients also have IgE autoantibodies against type XVII collagen, and these IgE autoantibodies have been shown to be pathogenic. Herein, we report a case of a woman with pruritic BP and very high levels of IgE and eosinophils who was refractory to standard aggressive immunosuppressive regimens for BP but responded rapidly to systemic omalizumab, a biological agent that binds to and nullifies IgE.

Neutrophilic panniculitis developing after treatment of metastatic melanoma with vemurafenib

Vemurafenib is an inhibitor of BRAF and is used to treat patients with metastatic melanoma who carry a V600E BRAF mutation. Recently, four patients have been described in the literature who developed a neutrophilic panniculitis following treatment with a BRAF inhibitor. We present an additional case and review the clinical findings of the cases reported to date.

Hydrophilic polymer microemboli in a patient with a chronic cutaneous ulcer: a case report

Hydrophilic polymer coatings were designed to reduce friction between the catheter and vessel wall and facilitate intravascular manipulations during catheterization and placement of drug‐eluting stents. One newly reported complication of such hydrophilic coatings is the fragmentation and embolization of the polymer, which can lead to ischemia and infarct by blocking the small vasculature. In this report, we present a patient with a non‐healing ulcer on the leg. Biopsy from the ulcer revealed the presence of hydrophilic polymer emboli within the dermal vessels. This is one of the few reports of such a complication involving the skin. Our objective is to emphasize the histopathologic features of this uncommon iatrogenic phenomenon.

Topical Fibronectin Improves Wound Healing of Irradiated Skin

Wound healing is significantly delayed in irradiated skin. To better understand global changes in protein expression after radiation, we utilized a reverse phase protein array (RPPA) to identify significant changes in paired samples of normal and irradiated human skin. Of the 210 proteins studied, fibronectin was the most significantly and consistently downregulated in radiation-damaged skin. Using a murine model, we confirmed that radiation leads to decreased fibronectin expression in the skin as well as delayed wound healing. Topically applied fibronectin was found to significantly improve wound healing in irradiated skin and was associated with decreased inflammatory infiltrate and increased angiogenesis. Fibronectin treatment may be a useful adjunctive modality in the treatment of non-healing radiation wounds.

A 49-year-old man with unilateral, nontender left eyelid swelling

Brandon J. Wong, BA, Bryan K. Hong, MD, Daman Samrao, MD, Gene H. Kim, MD, and Narsing A. Rao, MD Author affiliations: aKeck School of Medicine, University of Southern California, Los Angeles, California; bDoheny Eye Institute, Keck School of Medicine, University of Southern California, Los Angeles, California; cDepartment of Dermatology, Keck School of Medicine, University of Southern California, Los Angeles, California

Metastatic renal cell carcinoma presenting as multiple pyogenic granuloma-like nodules on the penis

RCC: renal cell carcinoma INTRODUCTION Approximately 60,000 new diagnoses of renal cell carcinoma (RCC) are made per year, accounting for 90% to 95% of all malignant neoplasms arising from the kidney with an estimated mortality rate of 20%. Rarely, advanced RCC may exhibit cutaneous metastases, most often to the scalp, chest, arms, and fingers. We discuss an unusual case of cutaneous metastatic RCC involving the penis, presenting as multiple exophytic pyogenic granuloma-like lesions.

Treatment of fibroelastolytic papulosis with fractionated carbon dioxide laser

Abstract Fibroelastolytic papulosis (FEP) is an acquired elastic tissue disorder that presents as white-to-yellow papules and plaques usually occurring on the neck. Although the lesions are often asymptomatic, their appearance may be distressing to patients. FEP has been treated with topical tretinoin in one case report (1). Other reports have not mentioned treatment for this rare disorder (1–6). We present a case of FEP successfully treated with a fractionated carbon dioxide (CO2) laser.

Anti-human milk fat globulin staining of perifollicular xanthomatosis in Fox-Fordyce disease.

A 40-year-old woman presented with numerous ivory-colored papules in the axilla (Figure 6). The asymptomatic papules had developed over the past year and had not been previously treated. Clinically, multiple syringomas or infection by molluscum contagiosum virus was suspected, and a biopsy was performed. The biopsy demonstrated follicular plugging with focal parakeratosis and slight spongiosis of the follicular infundibulum (Figures 1 and 3). In addition, there was an infiltrate of macrophages with foamy cytoplasm surrounding the follicular epithelium (Figure 4). Based upon these findings, a diagnosis of Fox-Fordyce disease was rendered. Fox-Fordyce disease represents an uncommon disorder that primarily affects aprocrine-rich areas of the skin and is characterized by variably pruritic, skinto-yellow-colored papules.1 Involvement of the axilla, as seen in this case, is characteristic. The etiology of Fox-Fordyce disease is thought to be related to follicular plugging, which leads to obstruction of the apocrine duct and secondary follicular spongiosis.2,3 The histopathology of Fox-Fordyce disease can be variable, but the most consistent microscopic findings are dilation and hyperkeratosis of the follicular infundibula.4 Other reported changes include a retention vesicle, spongiosis of follicular epithelium, dyskeratotic cells, vacuolar alteration at the dermoepithelial junction, and formation of cornoid lamella-like structures within infundibula.4,5 However, because these changes can be focal and potentially transient in nature, many of these findings may not be seen in routine sections and Fig. 1. Follicular dilatation and hyperkeratosis are present (40x).