Eosinophilic fasciitis/generalized morphea overlap.

A 50-year-old woman presented with a three-month history of violaceous, non-tender, indurated plaques on the chest, abdomen, breasts, and proximal portions of the arms and legs. An incisional biopsy specimen showed changes consistent with a diagnosis of inflammatory morphea. Over the course of one year, the patient began to develop signs and symptoms suggestive of a diagnosis of eosinophilic fasciitis, which included the characteristic groove sign on the upper extremities. Although our patient did not exhibit peripheral or histopathologic evidence of eosinophilia, the diagnosis of eosinophilic fasciitis could still be made because the aforementioned phenomena are not required for diagnosis. Multitude treatment regimes have been reported in the literature as single case reports or small patient series. Our patient was maintained on methrotrexate, oral glucocorticoids, and etanercept with improvement of skin lesions and mobility.

Cutaneous piloleiomyomata.

A 49-year-old man presented with an eight-month history of intermittently painful, subcutaneous nodules that were increasing in size, number, and pain intensity. A biopsy specimen showed smooth muscle proliferation, which also stained positive for actin, and was consistent with piloleiomyoma. The patient was placed initially on gabapentin and then nifedipine with very limited success in pain control. The lesions continued to proliferate, and the patient was referred to surgery for excision.

Neurovascular hamartoma.

A 29-year-old man presented with a large, asymptomatic, brown, hyperpigmented, depressed plaque over his left upper back, which included the scapular area, since childhood. Histopathological analyses of the biopsy specimens was consistent with a rare entity known as neurovascular hamartoma. This uncommon lesion has been reported in two publications, either as a possible marker of the malignant rhabdoid tumor or as a hamartomatous tongue lesion in children. Due to its possible association with the aggressive and often fatal rhabdoid tumor, periodic examination of this lesion may be warranted.

Noninvoluting congenital hemangioma.

An 8-year-old boy presented with a lifelong history of a vascular mass overlying his right mandible with central coarse telangiectasias and peripheral pallor. Histopathologic examination showed a proliferation of blood vessels in the dermis. Ultrasound examination identified a mix of high- and low-flow vessels within the lesion. These findings were consistent with a noninvoluting congenital hemangioma, a rare vascular tumor that is fully formed at birth that subsequently grows proportionately with the patient and does not regress.

Palisaded neutrophilic and granulomatous dermatosis.

A 22-year-old woman with mixed connective-tissue disease presented with a 5-month history of recurrent episodes of tender, erythematous papules, nodules, and edematous plaques on the upper extremities and thighs. Cutaneous lesions occurred in the setting of livedo reticularis. A biopsy specimen showed interstitial and perivascular inflammation with lymphocytes, macrophages, neutrophils, nuclear dust, collagen alteration, extravasated erythrocytes, and fibrin within small superficial blood vessels. These changes were consistent with a diagnosis of palisaded neutrophilic and granulomatous dermatosis, which is a rare entity that includes a combination of a neutrophilic infiltrate, abnormal or altered collagen, granuloma formation, and leukocytoclastic debris in the context of an immune-mediated collagen vascular or systemic disease. The underlying mechanism remains poorly understood. Treatment is limited, and resolution of lesions typically occurs within several months to years.

Chemotherapeutic agents and the skin: An update.

UNLABELLED: Chemotherapeutic agents give rise to numerous well described adverse effects that may affect the skin, hair, mucous membranes, or nails. The mucocutaneous effects of longstanding agents have been extensively studied and reviewed. Over the last 2 decades, a number of new molecular entities for the treatment of cancer have been approved by the United States Food and Drug Administration (FDA). This article reviews the cutaneous toxicity patterns of these agents. It also reviews one drug that has not received FDA approval but is in use outside the United States and is important dermatologically. Particular emphasis is placed on the novel signal transduction inhibitors as well as on newer literature pertaining to previously described reactions. LEARNING OBJECTIVES: At the completion of this learning activity, participants should able to list the newer chemotherapeutic agents that possess significant mucocutaneous side effects and describe the range of reactions that are seen with each drug. In addition, they should be able to formulate appropriate management strategies for these reactions.

Hypersensitivity reactions to vaccine components.

Vaccines are responsible for the control of many infectious diseases that were once common in the United States, including polio, measles, diphtheria, pertussis (whooping cough), rubella (German measles), mumps, tetanus, and Haemophilus influenzae type b. National efforts to generate collaboration between federal, state, and local governments and public and private health care providers have resulted in record high levels of vaccination coverage in the United States. The high rate of US vaccinations is paralleled by growing concerns about the safety of their delivery. The variety of substances used in vaccines sometimes causes the development of cutaneous reactions in susceptible adults and children. This article will review adverse cutaneous events consistent with hypersensitivity reactions to the following ingredients in vaccines: aluminum, thimerosal, 2-phenoxyethanol, formaldehyde, and neomycin.

Identification of the F1F0 mitochondrial ATPase as a target for modulating skin pigmentation by screening a tagged triazine library in zebrafish.

A triazine-based combinatorial library of small molecules was screened in zebrafish to identify compounds that produced interesting phenotypes. One compound (of 1536 screened) induced a dramatic increase in the pigmentation of early stage zebrafish embryos. This compound, PPA, was also found to increase pigmentation in cultured mammalian melanocytes. The cellular target was identified as the mitochondrial F1F0-ATP synthase (ATPase) by affinity chromatography. Oligomycin, a small molecule known to inhibit the mitochondrial ATPase, competed with PPA for its cellular target in melanocytes. In addition, PPA was shown to alter the membrane potential of mitochondria, consistent with inhibition of the mitochondrial ATPase. Thus, PPA has been successfully used as a chemical probe in a forward chemical genetic approach to establish a link between the phenotype and the protein. The results attest to the power of screening small molecule libraries in zebrafish as a means of identifying mammalian targets and suggest the mitochondrial ATPase as a target for modulating pigmentation in both melanocytes and melanoma cells.

Identification of compounds that bind mitochondrial F1F0 ATPase by screening a triazine library for correction of albinism.

A triazine-based combinatorial library of small molecules was screened in albino murine melanocytes to identify compounds that induce pigmentation. Six compounds (of 1536 screened) produced at least 3-fold increases in pigmentation. Immunohistochemical studies demonstrated that the compounds conferred correct routing of the mistrafficked enzyme tyrosinase, which is critical to normal melanogenesis. Affinity matrices of the immobilized compounds allowed the cellular target to be identified as the mitochondrial F1F0-ATP synthase. Oligomycin and aurovertin B, small molecules known to inhibit the mitochondrial ATP synthase, were shown to compete with the triazine-based compounds for their cellular target in albino melanocytes and confer similar effects on pigmentation and tyrosinase rerouting. This is the first demonstration of the mitochondrial ATP synthase as a potential therapeutic target for restoring pigmentation in albino melanocytes.

Treatment of irritant and allergic contact dermatitis.

The treatment of contact dermatitis lies principally in the avoidance of the offending agent. In certain circumstances, avoidance protocols are insurmountable, and therapy is rendered to assuage the inflammatory component and its consequent objective and subjective findings. However, the options thereafter vary, as some patients will require continuous symptomatic therapy despite avoidance of the purported offending agent. This manuscript will review established treatment options for contact dermatitis, such as corticosteroids and dietary manipulation, as well as discuss some promising new therapies from the last decade, such as the immunomodulatory and anti-inflammatory agents.