What the rheumatologist needs to know about skin biopsy.

The skin biopsy plays an important and powerful role in helping diagnose rheumatic diseases that present with cutaneous findings. As the skin is a very accessible organ, and the skin biopsy can be performed quickly as an in-office procedure, the skin biopsy is utilized frequently in patients with rheumatic diseases. However, the more challenging aspects of performing the biopsy, such as identifying the type of biopsy to perform, the site (s) to biopsy, the type of media to use, and the interpretation of histopathologic data are nuanced and require considerable thought. In this review, we discuss the common skin findings in rheumatic diseases and the general indications for skin biopsies in these diseases. We then summarize how to perform various skin biopsy techniques and how to select the biopsy technique. Finally, we discuss important rheumatic disease-specific considerations for skin biopsy, including where to biopsy and how to interpret the pathologic reports.

A Retrospective Study of Calcinosis Cutis in Patients With Systemic Lupus Erythematosus.

Calcinosis cutis (CC) is a very rare and poorly characterized finding in systemic lupus erythematosus (SLE).1 In this retrospective study, we present our experience of 10 individuals with SLE who developed CC, describing the epidemiology, diagnosis, and clinical characteristics of this rare entity.

Topical treatments for erythromelalgia.

Erythromelalgia is a rare neurovascular disease that causes episodes of pain, redness, and warmth in the extremities, and can be debilitating. Currently, there is no universally effective treatment for erythromelalgia. As the precise etiology of erythromelalgia remains obscure, presently available treatments are aimed at alleviating erythromelalgia's wide-ranging symptoms. In general, topical therapies for erythromelalgia are preferred for their more limited side effects and for those with contraindications to systemic therapies. This review will summarize the current topical therapies available to treat erythromelalgia and discuss emerging therapies based on our growing understanding of erythromelalgia pathophysiology.

A Retrospective Study of Idiopathic Granulomatous Mastitis Treatment and Outcomes.

Granulomatous mastitis (GM) is a rare, poorly characterized inflammatory disease with a broad differential diagnosis that includes both benign and malignant conditions such as infectious mastitis, sarcoidosis, and inflammatory breast cancer. The etiology of most cases is idiopathic (IGM). In this retrospective study, we present our experience of 31 patients with IGM, underscoring management and treatment outcomes.

A case of Wong-type dermatomyositis treated with rituximab and IVIG.

A 37-year-old woman presented to our rheumatology-dermatology clinic with a rash, muscle weakness and fatigue. She has had prior diagnoses of cutaneous lupus and lichen planus based on skin biopsies. She did not respond to topical steroids, hydroxychloroquine and dapsone. Clinically, she had sharply demarcated photo-distributed erythema over the upper back, chest and upper arms, along with hyperkeratotic follicular papules on bilateral upper arms, shoulders, posterior neck, behind the ears, chest including breasts, abdomen and right buttock. Investigations revealed a high titre ANA, elevated creatinine kinase, aldolase and positive anti-MJ/nuclear matrix protein 2 (NXP-2). A skin biopsy showed findings of connective tissue disease. The diagnosis of Wong-type dermatomyositis was made. She responded to therapy with mycophenolate mofetil, rituximab and IVIG.

Vasculitis: Kids are not just little people.

Cutaneous vasculitis, inflammatory destruction of blood vessels, can present with a wide range of clinical and pathologic findings across a number of heterogeneous conditions. Although some vasculitides are present in both children and adults, some important differences exist in clinical presentation, etiology, management, and prognosis in childhood vasculitis versus adult vasculitis. Cutaneous vasculitis is rare in children, and most childhood vasculitides, of which Henoch-Schönlein purpura is the most common, histologically are small vessel leukocytoclastic vasculitis. In children, infectious etiologies are more common than in adults. Childhood cutaneous vasculitis is most often self-limited with a good prognosis, and treatment is mainly supportive. © 2017 Elsevier Inc. All rights reserved.

Panfollicular carcinoma or trichoblastic carcinoma with panfollicular differentiation?

Herein, we report two cases of a follicular neoplasm with panfollicular differentiation showing architectural and cytologic findings suggestive of a malignancy. Immunohistochemical analysis of ß-catenin expression in the neoplasm showed nuclear and cytoplasmic immunoreactivity, with no reactivity in the transitional and shadow cells, consistent with ß-catenin expression of pilomatrical tumors. Staining for BerEp4 was positive at the periphery of both neoplasms, suggesting germinative differentiation of the neoplastic cells, whereas staining for the follicular stem-cell marker PHLDA-1 (TDAG51) showed strong focal expression in the tumor cells of both cases. Given these findings, these neoplasms show features of both panfollicular neoplasms and basal cell carcinoma with panfollicular/matrical differentiation. These are the first cases of this neoplasm reported to date. More reports are needed to assess their malignant potential.

The role of nutrition in dermatologic diseases: facts and controversies.

Many dermatologic diseases are chronic with no definitive cure. For some diseases, the etiology is not completely understood, with treatment being difficult and associated with side effects. In such cases, patients may try alternative treatments to prevent onset, reduce symptom severity, or prevent reoccurrence of a disease. Dietary modification, through supplementation and exclusion, is an extremely popular treatment modality for patients with dermatologic conditions. It is, therefore, important for dermatologists to be aware of the growing body of literature pertaining to nutrition and skin disease to appropriately inform patients on benefits and harms of specific dietary interventions. We address the role of nutrition in psoriasis, atopic dermatitis, urticaria, and bullous diseases and specific dietary modifications as an adjunct or alternative to conventional therapy.

Nutrition and bullous skin diseases.

Autoimmune and nonautoimmune bullous diseases can both be associated with significant morbidity and mortality. Although our understanding of the pathogenic mechanisms of these diseases has increased tremendously, there is still much to learn about the various factors affecting their onset, course, and therapy. In recent years, increasing information has been published about the effect of vitamins, minerals, and other nutrients on bullous skin diseases. Some factors are believed to be inducers (thiol and phenol-containing foods in pemphigus), whereas others are believed to be protective (antioxidants in cutaneous porphyrias). This contribution reviews the evidence in the literature of the role of various dietary factors in bullous diseases, including the nonautoimmune and the deficiency dermatoses. Additional studies and new investigations are needed to provide a better understanding of the specific associations of dietary factors with bullous diseases and better management for patients affected by these conditions.

Piperazinyl CCR1 antagonists--optimization of human liver microsome stability.

The synthesis, biological activity, and pharmacokinetic profile of CCR1 antagonists are described.