Granulomatous fasciitis followed by morphea profunda: Is granulomatous fasciitis part of a spectrum of deep morphea? A case report and review of the literature.

Although eosinophilic fasciitis is known to be part of the deep morphea spectrum, this first report of the coexistence of granulomatous fasciitis and morphea profunda suggests that granulomatous fasciitis may also be a part of the spectrum of deep morphea

Rituximab in the management of juvenile pemphigus foliaceus

BackgroundPemphigus foliaceus (PF) is a blistering disorder most commonly presenting in middle age. As PF is restricted to the superficial epidermis, it is considered more benign than other pemphigus diseases. However, progression to severe disease is not uncommon. Although rituximab’s efficacy has been well-documented in adults with refractory PF, little data is available on its role in adolescents.PurposeWe describe a patient with juvenile PF treated with rituximab and review the literature for similar cases.MethodsPubMed was searched for the terms: antibody, B cells, blistering, CD20, foliaceus, juvenile, pemphigus, rituximab, immunosuppression. As the first reported case of rituximab treated pemphigus was in 2001, only cases from 2001 and after were included. Juvenile PF was defined as disease diagnosis between ages 12-17.ResultsFive cases have been reported. The indication for rituximab in most cases was refractory PF unresponsive to systemic glucocorticoids and non-steroidal adjuvant therapies. All cases demonstrated significant improvement or complete remission and most experienced no adverse events.ConclusionsRituximab appears to be both well tolerated and efficacious for refractory juvenile PF. Therefore, it may be considered for severe cases of PF to avoid side effects associated with conventional glucocorticoid therapy

Rituximab in the management of juvenile pemphigus foliaceus

BackgroundPemphigus foliaceus (PF) is a blistering disorder most commonly presenting in middle age. As PF is restricted to the superficial epidermis, it is considered more benign than other pemphigus diseases. However, progression to severe disease is not uncommon. Although rituximab’s efficacy has been well-documented in adults with refractory PF, little data is available on its role in adolescents.PurposeWe describe a patient with juvenile PF treated with rituximab and review the literature for similar cases.MethodsPubMed was searched for the terms: antibody, B cells, blistering, CD20, foliaceus, juvenile, pemphigus, rituximab, immunosuppression. As the first reported case of rituximab treated pemphigus was in 2001, only cases from 2001 and after were included. Juvenile PF was defined as disease diagnosis between ages 12-17.ResultsFive cases have been reported. The indication for rituximab in most cases was refractory PF unresponsive to systemic glucocorticoids and non-steroidal adjuvant therapies. All cases demonstrated significant improvement or complete remission and most experienced no adverse events.ConclusionsRituximab appears to be both well tolerated and efficacious for refractory juvenile PF. Therefore, it may be considered for severe cases of PF to avoid side effects associated with conventional glucocorticoid therapy

Discoid lupus and human immunodeficiency virus: A retrospective chart review to determine the prevalence and progression of co-occurrence of these conditions at a single Academic Center

Context: Discoid lupus erythematosus (DLE) and human immunodeficiency virus (HIV) are both disorders of the immune system. The pathophysiology of these diseases varies greatly as DLE is characterized by an overactive immune system that attacks normal host cells, whereas HIV is characterized by an exogenous attack on the immune system that depletes it of key cell types. Although the reason is unknown, co-occurrence of DLE and HIV is rare. Aims: The goal of this study is to determine the prevalence of co-occurrence of DLE and HIV and to determine whether patients with both DLE and HIV share any clinical feature. Subjects and Methods: The medical records of all patients seen within a single academic health center over a 20-year period were reviewed to determine the prevalence of cutaneous lupus, HIV, and co-occurrence of these conditions. The charts of patients diagnosed with both conditions were further reviewed to determine similarities between them. Results: Of the 10,719 patients diagnosed with HIV and 182 patients diagnosed with cutaneous lupus, only 2 patients were diagnosed with both conditions. Both of these patients were diagnosed with DLE several years after being diagnosed with HIV. They had an undetectable HIV viral load, normal CD4 T-cell counts, and were on antiretroviral therapy when diagnosed with DLE. Conclusion: These results confirm that co-occurrence of DLE and HIV is rare. Although our study population was small, findings from these patients suggest that in HIV-positive patients, DLE manifestations occur when their HIV disease activity is minimal

Penicillamine-associated cutis laxa and milia en plaque - case report and review of cutaneous changes associated with penicillamine

Penicillamine-induced skin changes are rare and include: hypersensitivity reactions, autoimmune reactions, and cutaneous elastoses. We report a case of a 73-year-old man with cystinuria taking penicillamine for over 50 years who presented with penicillamine-induced cutis laxa and milia en plaque. A brief review of penicillamine induced skin changes, specifically cutis laxa and milia en plaque, is presented

Characteristics of research tracks in dermatology residency programs: a national survey

Pursuing research is encouraged in dermatology residency programs. Some programs offer specific research or investigative tracks. Currently, there is little data on the structure or scope of research tracks in dermatology residency programs. An anonymous online survey was distributed to the Association of Professors of Dermatology listserve in 2016. Program directors of dermatology residency programs in the United States were asked to participate and 38 of the 95 program directors responded. The survey results confirmed that a 2+2 research track, which is two years of clinical training followed by two years of research, was the most common investigator trackmodel and may promote an academic career at the resident’s home institution. Further studies will help determine the most effective research track models to promote long-term outcomes

Non-uremic calciphylaxis in a patient with multiple rheumatologic diseases

Non-uremic calciphylaxis is a rare, life-threatening condition characterized clinically by cutaneous necrosis and histologically by calcium deposition in small vessel walls. The etiology of non-uremic calciphylaxis remains the subject of ongoing speculation and debate. Herein we present a patient with calciphylaxis who had normal kidney function and numerous rheumatologic diseases, namely systemic lupus erythematosus (SLE), Sjogren syndrome (SS), and myasthenia gravis (MG). We review the pathophysiology, possible mechanisms, and management for non-uremic calciphylaxis

A crack in the armor: Wolf isotopic response manifesting as cutaneous lupus

Wolf isotopic response represents the development of skin lesions of one particular morphology occurring at the same site as another morphologically distinct and unrelated skin lesion. Cutaneous lupus erythematosus (CLE) is an autoimmune connective tissue disorder encompassing a wide range of phenotypes that may be associated with systemic involvement. Although CLE is a well-described entity with a broad spectrum, the occurrence of lesions manifesting as an isotopic response is rare. We present a patient with systemic lupus erythematosus who developed CLE in a dermatomal distribution following herpes zoster. When CLE lesions present in a dermatomal distribution, these cases may be difficult to distinguish from recurrent herpes zoster infection in an immunosuppressed patient. Therefore, they pose a diagnostic challenge and require balancing antiviral therapy with immunosuppression to sufficiently maintain adequate control of the autoimmune disease while addressing possible infections. To avoid treatment delay, clinicians should have elevated suspicion for an isotopic response when disparate lesions erupt in areas previously affected by herpes zoster or in cases of persistent eruptions at sites of prior herpes zoster. We discuss this case within the context of Wolf isotopic response and review the literature for similar cases