Nephrogenic Systemic Fibrosis in the Setting of Transient Renal Insufficiency.

Nephrogenic systemic fibrosis (NSF) is a rare disease that can present in patients with kidney injury who have been exposed to gadolinium-based contrast agents (GBCAs). These patients can easily be misdiagnosed as having another disease because they do not fit the usual presentation of being on hemodialysis. We report a case of NSF occurring in a patient with resolved transient renal insufficiency and no history of chronic renal disease.

Acroangiodermatitis of Mali and Stewart-Bluefarb syndrome.

Acroangiodermatitis (AAD), also known as pseudo-Kaposi sarcoma, is a rare benign vascular proliferation mainly of the extremities. It is characterized by violaceous patches or plaques resembling Kaposi sarcoma. The term pseudo-Kaposi sarcoma encompasses 2 variants of acroangiodermatitis: Mali type and Stewart-Bluefarb syndrome (SBS). Mali-type AAD is more common and is associated with chronic venous hypertension, while SBS is more rare and is associated with arteriovenous malformations and iatrogenic arteriovenous fistulae. We report 2 patients, representing each type of AAD.

Relapsing polychondritis in human immunodeficiency virus.

Relapsing polychondritis (RP) is an inflammatory condition that has been widely accepted as autoimmune in nature and can occur in patients with immune system dysregulation. Although RP has been well documented in patients with other autoimmune conditions, such as vasculitis, its presence in patients with human immunodeficiency virus (HIV) infection has been infrequently described. We describe a case of RP in an HIV-positive patient without other identifiable autoimmune disease.

Necrobiosis lipoidica with superimposed pyoderma vegetans.

Necrobiosis lipoidica (NL) is a granulomatous inflammatory skin disease strongly associated with diabetes mellitus (DM). Red-brown papules expanding into plaques with erythematous indurated borders on the lower extremities are characteristic of NL. Diagnosis is made clinically; however, biopsy of lesions confirms the diagnosis. Untreated NL may ulcerate and lead to further complications, but progression to superimposed pyoderma vegetans (PV) is not a known occurrence.

Inflammatory linear verrucous epidermal nevus responsive to 308-nm excimer laser treatment.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare cutaneous disease that presents as linear psoriasiform plaques with associated prominent pruritus. The lesions commonly pre-sent on the legs with onset during childhood. Inflammatory linear verrucous epidermal nevus typically is refractory to treatment. Therapies range from topical treatments to lasers and surgical options. It is clinically and histopathologically similar to psoriasis, suggesting it may respond to established psoriasis treatments such as the excimer laser. We report the case of an otherwise healthy 20-year-old woman with dry, pruritic, red lesions on the right leg that had been present since infancy. Biopsy revealed psoriasiform hyperplasia with a verruciform surface. Multiple topical treatments including ablative CO2 laser therapy showed no remarkable improvement. The patient was then treated with a UV 308-nm excimer laser and showed noticeable clinical improvement. Because of its clinical and histopathological similarities to psoriasis, we hypothesized that the excimer laser may be useful in the treatment of these lesions.

Eosinophilic pustular folliculitis with underlying mantle cell lymphoma.

Eosinophilic pustular folliculitis (EPF) is a noninfectious condition characterized by folliculocentric papules, pustules, and plaques on the head, trunk, and extremities. Three subtypes of EPF have been described. Histopathology predominantly shows abundant eosinophils concentrated at the follicle, and treatment typically consists of topical corticosteroids or oral indomethacin. We present an unusual case of EPF in a 52-year-old man that preceded the diagnosis of mantle cell lymphoma.

Spontaneous regression of Merkel cell carcinoma.

A 96-year-old woman presented with a rapidly enlarging lesion overlying the suprasternal notch. The lesion originated as a small, erythematous, scaly macule that rapidly increased in size over 8 weeks and became an ulcerated nodule measuring 5 cm in diameter and 4.5 cm in thickness. A 4-mm punch biopsy showed a poorly differentiated tumor with cells that were positive for CAM 5.2 and cytokeratin 20 in a dotlike paranuclear pattern and negative for cytokeratin 5/6, human melanoma black 45, and leukocyte common antigen. Two weeks after the punch biopsy, the lesion noticeably decreased in size, and within 8 weeks of the biopsy the tumor had completely resolved with no further intervention. Regression of Merkel cell carcinoma (MCC) is a very rare event, with as few as 30 cases reported. The mechanism of this phenomenon remains unclear; however, T-cell-mediated immunity and apoptosis appear to play a major role.

Photosensitive atopic dermatitis exacerbated by UVB exposure.

Photosensitive atopic dermatitis (AD) is a rare disease entity that many physicians are not familiar with, thus it often is misdiagnosed. It can be life altering, as patients often strictly avoid the sun and may only leave the house at night. Effective treatments are available, and therefore diagnosis is key to improve quality of life for these patients. We describe a case of photosensitive AD exacerbated by UVB exposure. The diagnosis was made with phototesting, and the patient was able to begin treatment with narrowband UVB (NB-UVB) hardening while on immunosuppression. The literature on photosensitive AD is limited, and this entity typically is not found in the main dermatology textbooks. Our case emphasizes the diagnostic problems and complexity of photosensitive AD. Histopathologic findings are nonspecific. A thorough history and physical examination can provide the necessary clues for further workup. Phototesting should be performed to confirm the diagnosis and evaluate the degree of sensitivity to UV light and the specific wavelength eliciting the cutaneous response. Photoprovocation and photopatch testing also can be useful to confirm the diagnosis.

Lichen Planus With Predominate Plasma Cell Infiltrate: Two Case Reports.

Lichen planus (LP) is a mucocutaneous inflammatory dermatitis of idiopathic origin that can involve the skin, mucous membranes, hair, and nails. LP has an associated set of characteristic histopathologic findings which include hyperkeratosis, vacuolization of the basal layer, Civatte bodies, wedge-shaped hypergranulosis, band-like lymphocytic infiltrate at the dermal epidermal junction, eosinophilic colloid bodies in the papillary dermis, and pigment incontinence. The infiltrate is usually composed of lymphocytes with few histiocytes, mast cells, and macrophages. The presence of plasma cell predominant infiltrate in LP has only been reported in four previous cases and 2 other cases of lichen nitidus. The authors report another 2 cases of LP with predominate plasma cell infiltrate in 2 female patients on the legs. The differential includes a drug-induced lichenoid reaction with predominate plasma cell infiltrate. However, there have been no case reports of that type of reaction. Because plasma cells are seen commonly in certain infectious diseases, malignancy, and macroglobulinemia, it is prudent to rule out those entities. Our patients responded well with a class 1 topical steroid, with improvement of their lower leg lesions within 1 month of treatment.

Pediatric Lichen Sclerosus: A Review of the Literature and Management Recommendations.

Lichen sclerosus is an uncommon, inflammatory disease of the skin and mucosa that can cause significant pruritus, pain, and scarring. There are two peaks of incidence: one in adult females, and the other in young girls. Early recognition and prompt management of the disorder is crucial in preventing long-lasting complications. This article reviews lichen sclerosus in children and provides evidence-based guidance for treatment in the pediatric population.

Merkel cell carcinoma: a review.

Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor of unknown origin that usually presents in the elderly population. A novel polyomavirus has been associated with a large percentage of tumors. Immune response plays an important role in pathogenesis of MCC. This article reviews the history, pathogenesis, presentation, and treatment of MCC. Future treatments also are discussed briefly.

Inflammatory vitiligo versus hypopigmented mycosis fungoides in a 58-year-old Indian female.

Vitiligo, particularly the rarer inflammatory variant, may be difficult to distinguish from hypopigmented mycosis fungoides (MF) clinically. Complicating the distinction is that when biopsies are taken from the periphery of early vitiliginous lesions or from lesions with an inflammatory border (inflammatory vitiligo), a dermal lymphocytic infiltrate, exocytosis, interface dermatitis, and mild spongiosis may be seen, all resembling the findings seen in hypopigmented MF. We present a case demonstrating the difficulty in differentiating between these two diseases and examine some characteristic clinical and histopathological features of each. Often, a conclusive diagnosis cannot be made, necessitating close follow-up of the patient and monitoring for progression of their disease over time.

Cutaneous adverse reaction to infliximab: report of psoriasis developing in 3 patients.

Infliximab is a chimeric immunoglobulin G1kappa monoclonal antibody against tumor necrosis factor alpha (TNF-alpha), a proinflammatory cytokine that participates in both normal immune function and the pathogenesis of many autoimmune disorders. Treatment with infliximab reduces the biologic activities of TNF-alpha and thus is indicated in the treatment of rheumatoid arthritis, Crohn disease, ankylosing spondylitis, psoriatic arthritis, plaque psoriasis, and ulcerative colitis. To our knowledge, there have been 13 case reports of new-onset psoriasis, psoriasiform dermatitis, and palmoplantar pustular psoriasis that developed during treatment with infliximab. We report 3 additional cases of biopsy-proven new-onset psoriasis that developed while the patients underwent treatment with infliximab for inflammatory bowel disease. Although the mechanism for the development of psoriasis in these patients is unclear, several possible explanations are proposed. With increasing use of infliximab and other TNF-alpha inhibitors in clinical practice, more cases of similar reactions to these drugs probably will be reported and are necessary to determine the importance of this eruption.