Dermatitis herpetiformis.

Dermatitis herpetiformis (DH) is an autoimmune, pleiomorphic, papulovesicular disorder associated with celiac disease and gluten sensitivity. DH is characterized by subepidermal bullae on hematoxylin and eosin staining and granular immunoglobulin A deposits in the dermal papillae using the direct immunofluorescence method. Antibodies to tissue transglutaminase and epidermal transgulatminase can be measured serologically, although biopsy is still required for definitive diagnosis of DH. Gluten free diet (GFD) is the first-line therapeutic approach that can alleviate both cutaneous and intestinal manifestations of this condition, while dapsone and sulfones target the skin eruption only. Combined therapy with GFD and dapsone is an initial treatment of choice to control the cutaneous manifestations of DH. This article will provide a comprehensive review of DH, including its epidemiology, clinical and pathological findings, diagnostic evaluation, and management.

Herpes simplex virus-induced plasmacytic atypia.

The clinical and histopathological features of cutaneous herpes simplex virus (HSV) infection have been well described. Genital herpetic infections are largely induced by HSV type 2, but 30% of cases can be caused by HSV type 1. Immunocompromised patients are known to exhibit atypical patterns of clinical presentation with variable lesion morphology and anatomic location. A subset of patients may show morphology such as nodules or verrucous lesions. Analogously, some biopsy specimens may show unusual microscopical features, such as a lack of keratinocyte cytopathology, lymphocyte infiltration or vasculopathic changes that are expected irrespective of the patient's immune status. We present the case of a patient carrying a previous diagnosis of pemphigus vulgaris, status posttreatment with methotrexate and prednisone, who developed a perineal ulcer exhibiting significant numbers of plasma cells, many of which were cytologically atypical. This morphology was suggestive of a hematopoietic malignancy. Immunoperoxidase staining for HSV decorated a focal collection of keratinocytes that lacked appreciable viral changes expected of HSV infection.

Primary cutaneous aspergillosis in a patient with a solid organ transplant: case report and review of the literature.

GOAL: To understand primary cutaneous aspergillosis to better manage patients with the condition. OBJECTIVES: Upon completion of this activity, dermatologists and general practitioners should be able to: 1. Describe risk factors for Aspergillus infection. 2. Identify diagnostic methods for primary cutaneous aspergillosis. 3. Discuss treatment options for primary cutaneous aspergillosis. Primary cutaneous aspergillosis is an uncommon disease in immunocompetent individuals that often affects immunosuppressed hosts. We present the first reported case of primary cutaneous aspergillosis in a solid organ transplant recipient caused by Aspergillus niger. Fruiting bodies were isolated from a necrotic ulcer arising in a surgical wound. Debridement alone failed to resolve the infection, emphasizing the need for early antifungal treatment combined with surgical management of this infection.

Unilateral facial angiofibromas without other evidence of tuberous sclerosis: case report and review of the literature.

Bilateral facial angiofibromas are common cutaneous manifestations of tuberous sclerosis, an autosomal-dominant disease characterized by hamartomas of multiple organs. Papules in patients with tuberous sclerosis typically appear between 4 and 10 years of age, becoming more extensive during puberty before stabilizing. We present a 28-year-old man with unilateral facial angiofibromas, which may represent a segmental form of tuberous sclerosis.

Amantadine-induced livedo reticularis: a report of two cases.

In the last several years, amantadine has been increasingly prescribed for akinesia in Parkinson's disease and to combat fatigue associated with multiple sclerosis. Amantadine is a well-known medication involved in drug-induced livedo reticularis (LR), yet remains under-reported in the English literature. We describe 2 patients with amantadine livedo reticularis: one patient with multiple sclerosis had, previous to this eruption, tolerated 4 years of amantadine therapy without adverse reactions and another patient with Parkinson's disease who developed LR 1 month after starting amantadine.

The effectiveness of tumor necrosis factor alpha antibody (infliximab) in treating recalcitrant psoriasis: a report of 2 cases.

BACKGROUND: Psoriasis is being recognized as an autoimmune disease in which immunocyte-derived cytokines are thought to drive the development of the altered keratinocyte phenotype. Although the role of tumor necrosis factor alpha (TNF-alpha) in psoriasis is not completely understood, it may underlie many of the key steps that lead to induction and maintenance of the disease. Infliximab is an immunoglobulin monoclonal antibody that binds and inactivates TNF-alpha and has been successfully used in the management of TNF-alpha-mediated diseases, such as Crohn disease and rheumatoid arthritis. OBSERVATIONS: Two patients with recalcitrant psoriasis that was unresponsive to multiple skin-directed and systemic therapies were treated with a single infusion of infliximab. The treatments resulted in rapid and complete clearing of psoriatic erythroderma and resolution of symptoms of arthritis in one case and complete clearing of widespread psoriatic plaques and improvement of symptoms of arthritis and inflammatory bowel disease in the other. The single treatments with infliximab were well tolerated with no immediate or long-term adverse effects noted. CONCLUSION: A single infusion of infliximab at 5 to 10 mg/kg resulted in the rapid and complete clearing of recalcitrant psoriatic plaques and erythroderma with a disease-free interval of 3 to 4 months in these 2 patients and improved the symptoms of psoriatic arthritis.

Familial ulcerative pyoderma gangrenosum: a report of 2 kindred.

Pyoderma gangrenosum is a rare, chronic ulcerative skin disease. It is a diagnosis of exclusion, after ruling out other causes of cutaneous ulceration. The etiology of pyoderma gangrenosum is poorly understood but is likely multifactorial. We describe 2 families affected by ulcerative pyoderma gangrenosum. This familial clustering suggests a possible genetic role in the development of pyoderma gangrenosum in some cases.