Generalized Discoid Lupus Erythematosus as the Presenting Sign of Small Cell Lung Carcinoma.

A 46-year-old woman with a 30 pack-year smoking history presented with a worsening eruption on the left cheek that failed to improve with metronidazole gel. The cutaneous eruption spread to most of her face and did not respond to a brief tapering course of prednisone. During the initial evaluation at our institution, approximately 6 weeks after the onset of the cutaneous eruption, the patient had erythematous, crusted plaques on her face and scalp (Figure 1A); they were also present on the V-area of the anterior aspect of the neck and upper region of the chest, the shoulders, and the arms, with isolated lesions on the trunk and legs. Her oral mucosa had erythematous erosions on the hard palate and gingivae. A review of systems revealed pain and burning of her skin lesions, but no muscle weakness or other systemic clinical manifestations. The differential diagnosis included autoimmune connective tissue disease, pemphigus foliaceous, sarcoidosis, lichen planus, phototoxic drug eruption, and eczema herpeticum.

Frequency of cutis laxa-like clinical features and elastolysis in scleromyxedema: a retrospective clinicopathologic study of 19 patients with scleromyxedema.

BACKGROUND: Cutis laxa-like features were observed in a subset of patients with scleromyxedema. Given this observation, clinical and histopathologic features of scleromyxedema were reviewed in correlation with elastic tissue staining. METHODS: We retrospectively reviewed clinical records and histopathologic features from patients with scleromyxedema seen at our institution from 1992 through 2013. We also evaluated available skin biopsies with an elastin stain and assessed whether dermal elastin fibers were diminished in density or were fragmented (or both). RESULTS: Nineteen patients with scleromyxedema and 34 skin biopsies were identified. Alcian blue (mucin) stain was used to grade mucin deposition as weakly positive (24%), positive (44%) and markedly positive (32%). Eight patients (42%) had clinical findings of cutis laxa, which were often observed in conjunction with areas of papular eruption or induration. Elastic tissue fibers were normal in 9 of 34 skin specimens (26%), 18 of 34 specimens (53%) had diminished elastic fiber density and 7 of 34 (21%) had markedly decreased density. The elastic tissue was fragmented in 25 specimens (74%). CONCLUSIONS: A cutis laxa-like clinical presentation and decreased elastic tissue density on skin biopsy were consistent findings. Dermatologists and dermatopathologists should be aware of these previously unreported clinical and histopathologic findings.

Diagnosis of deep cutaneous fungal infections: correlation between skin tissue culture and histopathology.

BACKGROUND: Deep cutaneous fungal infections (DCFIs) are responsible for significant morbidity and mortality, particularly in immunocompromised patients. Although a direct correlation between histopathologic examination and culture is expected, discordant findings may be seen, presenting a unique diagnostic and therapeutic challenge. OBJECTIVES: We sought to determine the correlation between skin tissue cultures and histopathologic examination in patients with DCFI. METHODS: This is a 10-year retrospective review (2003-2012) of patients with a diagnosis of DCFI seen at a single tertiary care institution. Tissue cultures and histopathologic findings were reviewed. RESULTS: In 8 of 33 cases, fungal elements were seen on routine histopathologic sections but skin cultures were negative. Three of 8 of the discordant cases had concurrent positive non-skin tissue cultures that correlated with the pathology interpretation, and 3 of 8 patients in the discordant group died of systemic fungal infection. LIMITATIONS: This was a retrospective study design and a single tertiary care institution experience. CONCLUSIONS: The histopathologic interpretation of skin tissue specimens is critical for rapid and accurate diagnosis of DCFI. Despite the identification of fungal organisms on histopathologic assessment of skin tissue specimens, skin tissue culture may fail to show fungal growth. A diagnosis of a DCFI and initiation of appropriate treatment should always be considered in spite of discordant results.

Cutaneous extramedullary plasmacytoma: clinical, prognostic, and interphase cytogenetic analysis.

Extramedullary plasmacytoma (EMP) of the skin is a rare indolent neoplasm that shares morphological and immunophenotypic features with plasma cell myeloma (PCM), but the molecular features that distinguish these two entities have not been defined. We reviewed the clinical characteristics, course, and molecular abnormalities in 7 cases of cutaneous EMP (cEMP); 2 patients had primary cEMP and 5 had secondary cEMP. Two patients died of progressive extramedullary plasmacytoma, 1 without PCM; 1 patient who had only a hyperdiploid clone, died within 17 months of the diagnosis of cEMP; and 3 died of PCM. One patient, who had cEMP with a hyperdiploid clone and a 13q deletion, was alive 28 months after diagnosis. Our findings raise questions about the relative prognostic value of molecular aberrations observed in cEMP and PCM. The role of fluorescence in situ hybridization testing in predicting disease progression of cEMP remains to be defined.

Acquired epidermodysplasia verruciformis in a child with the human immunodeficiency virus.

Epidermodysplasia verruciformis (EDV) is a rare genodermatosis characterized by susceptibility to human papilloma virus (HPV) infection. An acquired form of EDV has been described in the setting of immunosuppression, including in patients with the human immunodeficiency virus (HIV). We present the case of an HIV-positive, adopted Haitian boy who presented with EDV. Few cases of chidren with HIV and acquired EDV have been reported and are likely underrecognized.

Update of management of connective tissue diseases: livedoid vasculopathy.

Livedoid vasculopathy (LV) is characterized by painful purple macules and papules that subsequently ulcerate. The lesions heal over weeks to months resulting in smooth, porcelain-white, atrophic plaque-like areas with surrounding telangiectases and hyperpigmentation. The specific cause of LV is still to be determined and it is believed to be multifactorial in nature. Despite numerous available therapeutic agents, there is not a single best efficacious treatment for LV. Most of the available treatment options are based on isolated case reports or case series. In this article, studies on the pathogenesis and therapeutic approaches to LV are reviewed.

Leprosy in a Midwestern Dermatology Clinic: Report of 9 Patients.

OBJECTIVE: To describe the clinical features and epidemiology of leprosy in patients evaluated in a Midwestern dermatology clinic. PATIENTS AND METHODS: We performed a retrospective review of clinical and laboratory data from patients with leprosy who were evaluated in the Department of Dermatology at Mayo Clinic in Rochester, Minnesota, from January 1, 1994, through December 31, 2017. RESULTS: Nine patients, 7 male and 2 female, were identified, ranging in age from 15 to 63 years (mean age, 38 years). Six of the 9 patients (67%) were foreign-born: 3 from Oceania (2 from Micronesia and 1 from Guam), 1 from Southeast Asia (Indonesia), and 2 from Mexico. Three patients were born in the United States. All 9 patients presented with skin lesions (granulomatous histopathologic type), and 8 had neuropathy. Leprosy was multibacillary in 8 patients and paucibacillary in 1. Two patients experienced a type 1 treatment reaction, and 5 had type 2 reactions. Three of the 9 patients had speciation by polymerase chain reaction (Mycobacterium leprae in 2 and Mycobacterium lepromatosis in 1). CONCLUSION: Despite its rarity in the United States, leprosy should be considered in the differential diagnosis when evaluating both foreign- and US-born patients with granulomatous dermatitis and peripheral neuropathy. Because M lepromatosis was not identified until 2008 and requires polymerase chain reaction for diagnosis, the incidence of this species among patients with leprosy diagnosed in earlier years is unknown.

Pneumocystis jiroveci pneumonia in patients treated with systemic immunosuppressive agents for dermatologic conditions: a systematic review with recommendations for prophylaxis.

Pneumocystis jiroveci pneumonia is an opportunistic infection associated with substantial rates of mortality in immunosuppressed patients. Prophylaxis recommendations are mostly targeted toward patients with non-dermatologic diagnoses. This study was conducted to determine when dermatology patients treated with immunosuppressive medications should be offered P. jiroveci pneumonia prophylaxis. We searched the literature from January 1, 1993, to December 31, 2013, using terms relating to P. jiroveci pneumonia and dermatologic diagnoses to analyze the clinical characteristics of previously affected patients. Guidelines for P. jiroveci pneumonia prophylaxis from other medical fields were also analyzed. Of 17 dermatology patients reported to have contracted P. jiroveci pneumonia, eight (47.1%) died of the pneumonia. Risk factors included lack of prophylaxis, systemic corticosteroid therapy, lymphopenia, hypoalbuminemia, low serum CD4 counts, comorbid pulmonary or renal disease, malignancy, and prior organ transplantation. The present conclusions are limited by heterogeneity among the selected studies and limitations in their identification and selection. However, P. jiroveci pneumonia in dermatology patients is associated with a high mortality rate. Based on our analysis, we propose that prophylaxis be considered in dermatology patients in whom treatment with systemic corticosteroids at doses exceeding 20 mg/day or treatment with corticosteroid-sparing immunosuppressive agents is anticipated for at least 4 weeks, and in patients with additional risk factors for P. jiroveci pneumonia.

Nontuberculous Mycobacteria: Skin and Soft Tissue Infections.

Skin and soft tissue infections caused by nontuberculous mycobacteria are increasing in incidence. The nontuberculous mycobacteria are environmental, acid-fast bacilli that cause cutaneous infections primarily after trauma, surgery and cosmetic procedures. Skin findings include abscesses, sporotrichoid nodules or ulcers, but also less distinctive signs. Important species include Mycobacterium marinum and the rapidly growing mycobacterium: M. fortuitum, M. abscessus and M. chelonae. Obtaining tissue for mycobacterial culture and histopathology aids diagnosis. Optimal therapy is not well-established, but is species-dependent and generally dictated by susceptibility studies. Management often includes use of multiple antibiotics for several months and potential use of adjunctive surgery.

Dermatologic Features of ADA2 Deficiency in Cutaneous Polyarteritis Nodosa.

IMPORTANCE: Mutations in the CERC1 gene associated with deficiency in the ADA2 protein (DADA2) have been implicated in the pathogenesis of cutaneous polyarteritis nodosa (cPAN) and early-onset vasculopathy. DADA2 is not only limited to cPAN and vasculopathy but also includes immunodeficiency that affects several cellular compartments, including B cells; however, some patients appear to have a more indolent, skin-limited disease. OBSERVATIONS: In this report, we describe 2 white siblings (female and male) with a history of cPAN with DADA2 as a result of novel compound heterozygous mutations inherited in trans in the CECR1 gene (c.37_39del [p.K13del] and c.1159C>A [p.N328K]). The onset of disease was earlier in the female sibling than the male sibling although both were diagnosed as having cPAN in early childhood. The disease is associated with a more significant immunodeficiency and other systemic symptoms in the female than the male sibling. CONCLUSIONS AND RELEVANCE: These findings suggest a genetic cause of cPAN in some patients. Therefore, DADA2 should be considered in patients with cPAN, specifically in those whose conditions are diagnosed at an early age, regardless of their ethnicity, presence or absence of systemic symptoms, or a family history of the disease.

Indocyanine green angiographic evidence of choroiditis in scleroderma.

PURPOSE: The purpose of this case report was to demonstrate evidence of indocyanine green angiography leakage consistent with choroiditis in a patient with scleroderma. METHODS: In this case report, the patient underwent a variety of tests and examinations, including systemic evaluation, full ocular examination, skin biopsy, indocyanine green, and fluorescein angiography testing. A 52-year-old man had blurred vision centrally in both eyes. Vision was 20/25 and 20/20. Posterior examination showed cotton-wool spots in both eyes. The patient met European League against Rheumatism (EULAR) criteria for scleroderma. RESULTS: Fluorescein angiography confirmed the presence of leakage from the retinal vessels. More importantly, indocyanine green angiography revealed choroidal vessel leakage in both eyes. This provided evidence of choroiditis before vessel obliteration. Previous studies have shown evidence of choriocapillaris obliteration. Choroidal vessel leakage, however, has not been reported in patients with scleroderma. CONCLUSION: The results of this case demonstrate the usefulness of indocyanine green angiography in detecting the presence of choroiditis in scleroderma.

Cutaneous manifestations of immunoglobulin G4-related disease: what dermatologists need to know.

In this review, we summarize the recent literature and use case examples to reach diagnostic criteria for the diagnosis of immunoglobulin G4 (IgG4)-related diseases that may be of relevance to the practicing dermatologist.

Incidence of leukocytoclastic vasculitis, 1996 to 2010: a population-based study in Olmsted County, Minnesota.

OBJECTIVE: To determine the population-based incidence of leukocytoclastic vasculitis (LCV). PATIENTS AND METHODS: This is a retrospective population-based study of all Olmsted County, Minnesota, residents with a skin biopsy-proven diagnosis of LCV from January 1, 1996, through December 31, 2010. RESULTS: A total of 84 patients (mean age at diagnosis, 48.3 years) with newly diagnosed skin biopsy-proven LCV (43 women and 41 men) were identified. The incidence rate (age and sex adjusted to the 2000 US white population) was 4.5 per 100,000 person-years (95% CI, 3.5-5.4). The incidence of LCV increased significantly with age at diagnosis (P<.001) and did not differ between female and male patients. Subtypes of LCV were cutaneous small-vessel vasculitis (CSVV), 38 patients (45%); IgA vasculitis, 25 (30%); urticarial vasculitis, 10 (12%); cryoglobulinemic vasculitis, 3 (4%); and antineutrophil cytoplasmic antibody-associated vasculitis, 8 (10%). LCV was idiopathic in 29 of 38 patients with CSVV (76%) and 24 of 25 patients with IgA vasculitis (96%). Thirty-nine of 84 patients (46%) had systemic involvement, with the renal system most commonly involved (17 of 39 [44%]). Twenty-four of 80 patients (30%) with follow-up data available had recurrent disease. Compared with the Minnesota white population, observed survival in the incident LCV cohort was significantly poorer than expected (P<.001), including the subset of patients with idiopathic CSVV (P=.03). CONCLUSION: The incidence of LCV was higher than that reported in previously published studies. Idiopathic LCV was more common in our population-based cohort than that described previously. Overall survival was significantly poorer (P<.001) and should be explored further in future studies.