Acneiform Presentations of Folliculotropic Mycosis Fungoides.

BACKGROUND: Folliculotropic mycosis fungoides (FMF) is a variant of cutaneous T-cell lymphoma that has clinical overlap with a variety of inflammatory follicular unit disorders. However, we describe distinctive presentations of FMF with acneiform features that can be diagnostically challenging, leading to diagnostic delay. OBJECTIVE: To highlight the importance of histopathologic and immunohistochemical evaluation for diagnostic confirmation of presumed inflammatory follicular unit-based disorders that are unusual in presentation or unresponsive to standard therapies. METHODS: A cross-sectional retrospective study of 5 consecutive patients with a histopathologic diagnosis of FMF was conducted. The clinical, histopathologic, immunophenotypic, and molecular genetic features of cases are presented. RESULTS: We describe 5 patients with clinical and histopathologic presentations of FMF masquerading as hidradenitis suppurativa, furunculosis, or acne vulgaris (age range 34-66 years, 4:1 female to male). Clinical morphologies included open and closed comedones, inflammatory pustules, papules and nodules, follicular papules with keratotic plugging, cysts, and scarring involving the face, trunk, and intertriginous areas. All patients failed to respond to standard therapies, including topical and oral antibiotics, topical and oral retinoids, or topical corticosteroids, before receiving the diagnosis of FMF. Lesional skin biopsies showed a perifollicular CD4-positive T-lymphocytic infiltrate with pilotropism, intrafollicular mucin deposition, foreign-body granulomatous inflammation, acute inflammation, and follicular epithelial necrosis. None had concurrent systemic mycosis fungoides. LIMITATIONS: Small retrospective cohort study. CONCLUSION: We present these cases to expand the clinical and histopathologic spectrum of FMF that may strikingly resemble acneiform disorders and to highlight the importance of diagnostic reconsideration with histopathologic evaluation.

Twenty-five practical recommendations in primary care dermoscopy.

Dermoscopy in primary care enhances clinical diagnoses and allows for risk stratifications. We have compiled 25 recommendations from our experience of dermoscopy in a wide range of clinical settings. The aim of this study is to enhance the application of dermoscopy by primary care clinicians. For primary care physicians commencing dermoscopy, we recommend understanding the aims of dermoscopy, having adequate training, purchasing dermoscopes with polarised and unpolarised views, performing regular maintenance on the equipment, seeking consent, applying contact and close non-contact dermoscopy, maintaining sterility, knowing one algorithm well and learning the rules for special regions such as the face, acral regions and nails. For clinicians already applying dermoscopy, we recommend establishing a platform for storing and retrieving clinical and dermoscopic images; shooting as uncompressed files; applying high magnifications and in-camera improvisations; explaining dermoscopic images to patients and their families; applying toggling; applying scopes with small probes for obscured lesions and lesions in body creases; applying far, non-contact dermoscopy; performing skin manipulations before and during dermoscopy; practising selective dermoscopy if experienced enough; and being aware of compound lesions. For clinicians in academic practice for whom dermatology and dermoscopy are special interests, we recommend acquiring the best hardware available with separate setups for clinical photography and dermoscopy; obtaining oral or written consent from patients for taking and publishing recognisable images; applying extremely high magnifications in search of novel dermoscopic features that are clinically important; applying dermoscopy immediately after local anaesthesia; and further augmenting images to incorporate messages beyond words to readers.

Outcomes of dermoscope-guided surgical procedures in primary care: case-control study.

INTRODUCTION No research has been found regarding outcomes of dermoscope-guided surgical procedures in primary care. AIM To establish whether outcomes of dermoscope-guided procedures performed in primary care settings differ from outcomes for similar procedures, performed without the use of a dermoscope. METHODS A retrospective case-control study design was used. All records of dermoscope-guided procedures performed over a 6-month period were retrieved. For each study procedure, the record of the most recent control procedure without dermoscopy guidance performed on a sex-and-age matched patient was retrieved from before we began performing dermoscope-guided procedures. Primary outcomes were: local inflammation and infections within 2 weeks' post procedure; relapse in 6 months; and obvious scars in 6 months. Pain affecting activities of daily living in the first week after the procedure was the secondary outcome. RESULTS Records of 39 dermoscope-guided procedures and 39 control procedures were retrieved. No significant difference in local inflammation and infections in 2 weeks was found; relapse in 6 months after the study procedures was significantly lower for dermoscope-guided than control procedures (risk ratio (RR): 0.22; 95% confidence interval (CI): 0.05-0.95), and there were fewer obvious scars for dermoscope-guided procedures than control procedures (RR: 0.52; 95% CI: 0.32-0.83), with the number of small lesions (<4 mm) leaving scars in study procedures particularly less than that for control procedures (RR: 0.30; 95% CI: 0.13-0.67). There was no difference in the secondary outcome of pain affecting activities of daily living in the first week following the procedure. CONCLUSION In primary care, dermoscope-guided procedures achieved better outcomes than similar procedures without dermoscope guidance. Performing dermoscope-guided procedures in primary care might lower medical costs.

Red tattoo-related mycosis fungoides-like CD8+ pseudolymphoma.

Cutaneous reactions to red tattoo pigment rarely manifest as pseudolymphomatous reactions. We describe an exceedingly rare case of red tattoo-related T-cell predominant pseudolymphoma microscopically mimicking mycosis fungoides. Careful clinicopathological correlation was required to obtain the correct diagnosis and aid in an effective treatment course.

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.

Multiplex matrix network analysis of protein complexes in the human TCR signalosome.

Multiprotein complexes transduce cellular signals through extensive interaction networks, but the ability to analyze these networks in cells from small clinical biopsies is limited. To address this, we applied an adaptable multiplex matrix system to physiologically relevant signaling protein complexes isolated from a cell line or from human patient samples. Focusing on the proximal T cell receptor (TCR) signalosome, we assessed 210 pairs of PiSCES (proteins in shared complexes detected by exposed surface epitopes). Upon stimulation of Jurkat cells with superantigen-loaded antigen-presenting cells, this system produced high-dimensional data that enabled visualization of network activity. A comprehensive analysis platform generated PiSCES biosignatures by applying unsupervised hierarchical clustering, principal component analysis, an adaptive nonparametric with empirical cutoff analysis, and weighted correlation network analysis. We generated PiSCES biosignatures from 4-mm skin punch biopsies from control patients or patients with the autoimmune skin disease alopecia areata. This analysis distinguished disease patients from the controls, detected enhanced basal TCR signaling in the autoimmune patients, and identified a potential signaling network signature that may be indicative of disease. Thus, generation of PiSCES biosignatures represents an approach that can provide information about the activity of protein signaling networks in samples including low-abundance primary cells from clinical biopsies.

Is Gianotti-Crosti Syndrome Associated with Atopy? A Case-Control Study and a Postulation on the Intrinsic Host Factors in Gianotti-Crosti Syndrome.

OBJECTIVES: To investigate whether Gianotti-Crosti syndrome (GCS) in children is associated with atopy. METHODS: The setting was two outpatient clinic. Diagnoses of asthma and atopic dermatitis (AD) were made according to internationally accepted diagnostic criteria. Allergic rhinitis, atopic urticaria, and allergic conjunctivitis were diagnosed clinically. Participants were children with GCS diagnosed over the previous 5 years. For any child with GCS, we extracted the record of the subsequent age and sex pair-matched child seen for problems unrelated to the skin as controls. RESULTS: We retrieved the records of 37 pairs of study and control subjects; 28 (76%) children with GCS and 9 (24%) controls had AD (risk ratio [RR] = 3.11[95% confidence interval {CI} 1.73, 5.73]), 31 (84%) children with GCS and 19 (51%) controls had at least one atopic condition (RR = 1.63 [95% CI 1.13, 2.18]) and 11 (30%) children with GCS and 2 (5%) controls had at least three atopic conditions (RR = 5.50 [95% CI 1.29, 35.35]). CONCLUSION: GCS is significantly associated with AD and the presence of atopic conditions.

Pityriasis Rosea, Gianotti-Crosti Syndrome, Asymmetric Periflexural Exanthem, Papular-Purpuric Gloves and Socks Syndrome, Eruptive Pseudoangiomatosis, and Eruptive Hypomelanosis: Do Their Epidemiological Data Substantiate Infectious Etiologies?

Many clinical and laboratory-based studies have been reported for skin rashes which may be due to viral infections, namely pityriasis rosea (PR), Gianotti-Crosti syndrome (GCS), asymmetric periflexural exanthem/unilateral laterothoracic exanthem (APE/ULE), papular-purpuric gloves and socks syndrome (PPGSS), and eruptive pseudo-angiomatosis (EP). Eruptive hypomelanosis (EH) is a newly discovered paraviral rash. Novel tools are now available to investigate the epidemiology of these rashes. To retrieve epidemiological data of these exanthema and analyze whether such substantiates or refutes infectious etiologies. We searched for articles published over the last 60 years and indexed by PubMed database. We then analyzed them for universality, demography, concurrent patients, temporal and spatial-temporal clustering, mini-epidemics, epidemics, and other clinical and geographical associations. Based on our criteria, we selected 55, 60, 29, 36, 20, and 4 articles for PR, GCS, APE/ULE, PPGSS, EP, and EH respectively. Universality or multiple-continental reports are found for all exanthema except EH. The ages of patients are compatible with infectious causes for PR, GCS, APE/ULE, and EH. Concurrent patients are reported for all. Significant patient clustering is demonstrated for PR and GCS. Mini-epidemics and epidemics have been reported for GCS, EP, and EH. The current epidemiological data supports, to a moderate extent, that PR, GCS, and APE could be caused by infectious agents. Support for PPGSS is marginal. Epidemiological evidences for infectious origins for EP and EH are inadequate. There might be growing epidemiological evidence to substantiate or to refute our findings in the future.

Lenalidomide treatment of cutaneous lupus erythematosus: the Mayo Clinic experience.

BACKGROUND: Published case series describe lenalidomide as an effective treatment of refractory cutaneous lupus erythematosus (CLE). OBJECTIVES: The present study aimed to further characterize lenalidomide use in the treatment of CLE. METHODS: A retrospective review of patients treated with lenalidomide for CLE from January 1, 2000, to December 17, 2014, was conducted. RESULTS: Eight of the nine patients (89%) were women. Their median age at initiation of lenalidomide was 62 years (range: 41-86 years). Subtypes of CLE included discoid lupus erythematosus (DLE) (n = 6), lupus panniculitis (n = 2), and subacute CLE (n = 1). Before the initiation of lenalidomide, all patients had been previously treated unsuccessfully or were intolerant to at least one antimalarial and one immunosuppressive agent. With lenalidomide, five patients achieved a complete response (CR), two a partial response, and two had no response (lupus panniculitis). Time to initial response (dose range: 2.5-10.0 mg/d) varied from 2 weeks to 3 months; the median time to CR in five patients was 3 months (range: 3-6 months). The median duration of lenalidomide therapy was 12 months (range: 2-67 months). The median duration of follow-up was 48 months (range: 20-103 months). Adverse effects included mild leukopenia; one patient had deep vein thrombosis of unclear etiology during a hospitalization. No patients developed or showed progression of systemic LE while receiving lenalidomide. CONCLUSIONS: Lenalidomide was effective for the treatment of CLE (particularly DLE) but not for the treatment of lupus panniculitis in this series.

Doxycycline-Induced Solar Urticaria: A Report of Two Cases.

We report two cases of doxycycline-induced solar urticaria that developed shortly after initiation of therapy with persistence despite discontinuation. Consequently, dermatologists should be aware of the association between doxycycline and solar urticaria and counsel their patients on the potential for this side effect when prescribing doxycycline.

Human herpesviruses 6, 7, and 8 from a dermatologic perspective.

Human herpesviruses (HHVs) have frequently been suspected as etiologic agents or cofactors in cutaneous disease. However, clearly established associations are rare. Investigations into an etiologic association between HHVs and cutaneous disease are complicated by the ubiquity and nearly universal prevalence of some herpesviruses. This article summarizes the associations between cutaneous disease and HHV-6, HHV-7, and HHV-8. In addition to a personal library of references, the PubMed database of biomedical literature was searched using the following Medical Subject Heading terms: HHV-6, HHV-7, and HHV-8, each in conjunction with cutaneous manifestations, virology, epidemiology, dermatopathology, and therapeutics, between 1998 and March 2011. Free-text searches with known or suspected disease associations were added for broader coverage. The results have been summarized to provide a practical review for the physician likely to encounter cutaneous diseases.

Gianotti-Crosti syndrome, pityriasis rosea, asymmetrical periflexural exanthem, unilateral mediothoracic exanthem, eruptive pseudoangiomatosis, and papular-purpuric gloves and socks syndrome: a brief review and arguments for diagnostic criteria.

Several exanthems including Gianotti-Crosti syndrome, pityriasis rosea, asymmetrical periflexural exanthem, eruptive pseudoangiomatosis, and papular-purpuric gloves and socks syndrome are suspected to be caused by viruses. These viruses are potentially dangerous. Gianotti-Crosti syndrome is related to hepatitis B virus infection which is the commonest cause of hepatocellular carcinoma, and Epstein-Barr virus infection which is related to nasopharyngeal carcinoma. Pityriasis rosea has been suspected to be related to human herpesvirus 7 and 8 infections, with the significance of the former still largely unknown, and the latter being a known cause of Kaposi's sarcoma. Papular-purpuric gloves and socks syndrome is significantly associated with human B19 erythrovirus infection which can lead to aplastic anemia in individuals with congenital hemoglobinopathies, and when transmitted to pregnant women, can cause spontaneous abortions and congenital anomalies. With viral DNA sequence detection technologies, false positive results are common. We can no longer apply Koch's postulates to establish cause-effect relationships. Biological properties of some viruses including lifelong latent infection, asymptomatic shedding, and endogenous reactivation render virological results on various body tissues difficult to interpret. We might not be able to confirm or refute viral causes for these rashes in the near future. Owing to the relatively small number of patients, virological and epidemiology studies, and treatment trials usually recruit few study and control subjects. This leads to low statistical powers and thus results have little clinical significance. Moreover, studies with few patients are less likely to be accepted by mainstream dermatology journals, leading to publication bias. Aggregation of data by meta-analyses on many studies each with a small number of patients can theoretically elevate the power of the results. Techniques are also in place to compensate for publication bias. However, these are not currently feasible owing to different inclusion and exclusion criteria in clinical studies and treatment trials. The diagnoses of these rashes are based on clinical assessment. Investigations only serve to exclude important differential diagnoses. A wide spectrum of clinical features is seen, and clinical features can vary across different populations. The terminologies used to define these rashes are confusing, and even more so are the atypical forms and variants. Previously reported virological and epidemiological results for these rashes are conflicting in many aspects. The cause of such incongruence is unknown, but low homogeneity during diagnosis and subject recruitment might be one of the factors leading to these incongruent results. The establishment and proper validation of diagnostic criteria will facilitate clinical diagnosis, hasten recruitment into clinical studies, and allow results of different studies to be directly compared with each another. Meta-analyses and systematic reviews would be more valid. Diagnostic criteria also streamline clinical audits and surveillance of these diseases from community perspectives. However, over-dependence on diagnostic criteria in the face of conflicting clinical features is a potential pitfall. Clinical acumen and the experience of the clinicians cannot be replaced by diagnostic criteria. Diagnostic criteria should be validated and re-validated in response to the ever-changing manifestations of these intriguing rashes. We advocate the establishment and validation of diagnostic criteria of these rashes. We also encourage the ongoing conduction of studies with a small number of patients. However, for a wider purpose, these studies should recruit homogenous patient groups with a view towards future data aggregation.

Becker nevus with an underlying desmoid tumor: a case report and review including Mayo Clinic's experience.

BACKGROUND: Becker nevus is a nevoid melanosis, referred to as Becker nevus syndrome when it is associated with other anomalies. Our objectives were to report the occurrence of a Becker nevus with an underlying desmoid soft-tissue tumor; to review Mayo Clinic's experience with Becker nevi, concentrating on Becker nevi associated with bone, vascular, neural, and other soft-tissue abnormalities; to inform physicians of the Becker nevus syndrome; and finally to alert clinicians to evaluate a Becker nevus with its associations in mind. OBSERVATIONS: A 46-year-old woman had a Becker nevus with an underlying desmoid-type fibromatosis (desmoid tumor) presenting clinically as a "painful dimple" within the nevus. Review of medical records for 1997 through 2006 at Mayo Clinic, Rochester, Minnesota, yielded 52 patients with Becker nevi, 12 of whom had an associated bone, vascular, neural, congenital, or other soft-tissue abnormality, ranging from liposarcoma to an accessory areola. CONCLUSIONS: We add to the literature a unique case of desmoid-type fibromatosis immediately beneath a Becker melanosis, which presented as a painful dimple. We hope to raise awareness that a Becker nevus may be associated with other abnormalities, including an infiltrative soft-tissue tumor. We also emphasize the importance of follow-up, including inspection of not only the surface but also the deep tissues underlying the Becker nevus.

Localized cold urticaria to the face in a pediatric patient: a case report and literature review.

We present a curious case of localized cold urticaria restricted to the face in a 10-year-old girl. Testing for the condition using an ice cube was positive only in the facial area. After 2 years, the patient continues to experience localized urticaria only on her face on cold exposure. A review of the available published medical literature on cold urticaria was performed using Ovid and PubMed databases. The literature search was not limited to the English language. Only three other cases of cold urticaria localized to the face were identified. Upon review of the published reports on cold urticaria and discussion of classification and diagnostic testing, we conclude that cold urticaria clearly is a rare and poorly understood entity.

Osteosarcoma of the skin.

BACKGROUND: Osteosarcoma is a common malignancy, although skin metastasis is rare. We sought to review the incidence, epidemiology, risk factors, and prognosis of osteosarcoma with skin involvement. METHODS: We conducted a retrospective chart review that covered 30 years and involved clinic patients younger than 18 years who had cutaneous metastases of osteosarcoma. The main outcome measure was histologic documentation of both primary tumor and metastatic lesion in the skin. RESULTS: Two patients were found to have osteosarcoma with skin involvement. No unifying factors were identified. CONCLUSIONS: Although osteosarcoma is a common malignancy with frequent metastases, involvement of the skin is rare. Further studies to identify risk factors and subsequent prognosis are necessary. Nevertheless, unidentifiable skin lesions in a patient with a history of osteosarcoma should be investigated to rule out tumor metastasis, regardless of cancer status.

Effective use of topical amitriptyline hydrochloride 2.5% and ketamine hydrochloride 0.5% for analgesia in refractory proctodynia.

Idiopathic proctodynia, an enigmatic pain syndrome affecting the perianal region, can be persistent, relatively refractory to treatment, and associated with considerable psychological distress. The authors present the case of a patient with a long history of severe proctodynia that had been resistant to a range of topical and systemic treatments. With the use of topical amitriptyline hydrochloride 2.5% and ketamine hydrochloride 0.5% cream, the patient's pain resolved rapidly, leading to a substantially improved quality of life.

Early cutaneous gene transcription changes in adult atopic dermatitis and potential clinical implications.

Atopic dermatitis (AD) is a common pruritic dermatitis with macroscopically non-lesional skin that is often abnormal. Therefore, we used high-density oligonucleotide arrays to identify cutaneous gene transcription changes associated with early AD inflammation as potential disease control targets. Skin biopsy specimens analysed included normal skin from five healthy non-atopic adults and both minimally lesional skin and nearby or contralateral non-lesional skin from six adult AD patients. Data were analysed on an individual gene basis and to identify biologically relevant gene networks. Transcription levels of selected genes were also analysed by quantitative PCR. Differential transcription occurring early in AD skin was indicated for (i) individual genes such as C-C chemokine ligand (CCL)18, CCL13, and interferon-alpha2 (IFNalpha2), (ii) genes associated with peroxisome proliferator-activated receptor (PPAR)alpha- and PPARgamma-regulated transcription, and possibly for (iii) immunoglobulin J-chain and heavy chain isotype transcripts. These data suggest that local changes in immunoglobulin-associated transcription may favour IgE over secretory immunoglobulin (multimeric IgM and IgA) expression in AD skin. Decreased PPAR activity appears common to both AD and psoriasis, and reduced cutaneous IFNalpha2 transcription also appears characteristic of AD. Identification of these genes and pathways will direct future research towards controlling AD.

Temporal case clustering in pityriasis rosea: a regression analysis on 1379 patients in Minnesota, kuwait, and diyarbakir, Turkey.

OBJECTIVE: To detect temporal case clustering in patients with pityriasis rosea in different geographic locations. DESIGN: Regression analysis of dates on which 1379 patients were diagnosed as having pityriasis rosea in 3 different geographic locations. The control data consisted of dates of diagnosis of patients with psoriasis in the same settings. SETTING: Dermatology clinics in Kuwait, Minnesota, and Diyarbakir, Turkey.Patients Patients with pityriasis rosea and psoriasis seeking care in the clinics. RESULTS: Three significant positive clusters (P = .005, P =.001, and P =.01, respectively) and 1 significant negative cluster (P<.001) were detected in these series of patients. No cluster was detected in 2 corresponding series of patients with psoriasis in Kuwait and Turkey. CONCLUSION: Temporal case clustering exists in pityriasis rosea.