Primary immunodeficiency disorders (PIDs) are a group of disorders affecting the capability to fight against infection. These include defects in T cells and B cells affecting cell-mediated and humoral immunity, respectively, combined humoral and cell-mediated immunodeficiency, defects in phagocytosis, complement defects, and defects in cytokine or cytokine signalling pathways which are detrimental for immune function. Depending upon the type and severity, age at onset of symptoms can vary from neonatal period to late childhood. Clinically, this group of disorders can involve any organ system of an individual such as respiratory system, gastrointestinal system, skin and mucous membrane, bone and joints, endocrine organs, and nervous system. Common dermatological manifestations include eczema, warts, molluscum contagiosum, mucocutaneous candidiasis, recurrent nonhealing ulcers, skin abscesses, erythroderma, petechiae, and nail changes. The common skin manifestations of various PIDs include eczema (seen in Wiskott–Aldrich syndrome and autosomal dominant hyper IgE syndrome); erythroderma (in Omen syndrome); viral warts or molluscum contagiosum (in autosomal recessive hyper IgE syndrome); chronic mucocutaneous candidiasis (in hyper IgE syndrome, autoimmune polyendocrinopathy candidiasis ectodermal dysplasia syndrome, Th17 cell defects); recurrent nonhealing ulcers (in leucocyte adhesion defect); skin abscesses (in antibody defects, hyper IgE syndrome, and chronic granulomatous disease); petechial or purpuric spots (in Wiskott–Aldrich syndrome).

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

Treatment of melasma is known to be less satisfactory, often incomplete, and relapse is frequent. Although many treatment options are available, they are either known to be unsafe on long-term use or their long-term safety profile is unknown. Patients often use various drugs, even topical steroid-based preparation without any medical supervision for long period of time, making the skin unsuitable for many of the drugs available. Thus, there has been gross disparity among the treating physician about what drugs and what regimen are best suitable for various categories of melasma patients and in different situations. With this background, numerous newer drugs, mostly combinations of some proprietary molecules or even unknown plant extracts, have flooded the market for the management of melasma. Information on efficacy or safety of these products are almost unknown. Studies on Asian people, especially Indian population, are far less commonly available. Therapeutic guideline for use on Indian patients with melasma is almost missing. Extrapolation of data from Caucasian people for use on Asian people may not be scientifically justifiable because Caucasian and Asian people are known to have inherent difference in their response as well as tolerance to the drugs used for melasma. With this background, we have extensively evaluated, following a strict, scientifically designed protocol, all the available studies on melasma management till May 2016 and prepared this document on level of evidence, grade of recommendation and suggested therapeutic guideline for melasma as per the method proposed by Oxford Centre of Evidence-Based Medicine. Various ethical, social, logical, regional, and economic issues in the context of Indian and similar populations were given due importance while preparing the suggested therapeutic recommendation.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Subungual melanoma (SUM) is an uncommon form of acral melanoma that arises within the nail matrix. The incidence for acral melanomas is similar worldwide, however, the proportion is higher in dark-skinned individuals. The subungual form represents approximately 2% of cutaneous non-sun-induced melanomas in the western world and up to 75% in Africans, 10% in Japanese, and 25% in the Chinese. No specific figures are available from the Indian subcontinent; however, the authors could trace three anecdotal case reports published over the last two decades. A general reluctance to biopsy a nail lesion to confirm the diagnosis may be contributing to the missed diagnosis. We report four cases of SUM of the big toenails seen over a period of 2.5 years. They were three women and one man with an age ranging from the 4^th to 7^th decade and disease duration of 6–18 months. The lesion involved the big toe in all and two patients had liver and lymph node metastasis at the time of presentation. Awareness among dermatologists regarding clinical manifestations, high index of suspicion for acral pigmented lesions, and nail bed biopsy may help in the early diagnosis and management and can prevent mortality.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Introduction and Objective: Platelet-rich plasma (PRP) is an autologous preparation of platelets in concentrated plasma. The platelet is a natural source of different growth factors and cytokines. These growth factors act on stem cells in the bulge area of the follicles and stimulate the development of new follicles, and promote neovascularization. The aim of this study was to investigate the efficacy and safety of PRP injections in androgenetic alopecia (AGA) in men. Patients and Methods: Fifteen male patients (mean age: 39 ± 9.7 years) with AGA grades III–VI were enrolled in the study. Five injections of 2–4 ml PRP (Regenlab PRP Kit-RegenACR®, Le Mont-sur-Lausanne Switzerland) by single spin process were administered every 2 weeks. Standard photographs, trichogram, and measurement of hair density and diameter in an area marked with a tattoo (with digital photographic hair analyzer) were done at baseline and 3 months after the last injection. In addition, patients completed a patient satisfaction questionnaire at each visit on a −2 to +2 score (−2: much worse, −1: slightly worse, 0: without change, +1: slightly better, +2: much better). Results: Thirteen patients completed the study. The number of hairs increased slightly from 149.62 ± 49.56 to 168.46 ± 43.703/cm², however, this increase was not statistically significant (P = 0.24). On the other hand, the thickness of hairs decreased from 0.051 ± 0.105 to 0.045 ± 0.011 mm, which was also not significant (P = 0.37). There was a significant decrease in anagen hairs and increase in telogen hairs, and anagen/telogen ratio decreased significantly from 6.38 ± 4.57 to 2.67 ± 1.87 (P = 0.003). Conclusion: Our study could not show any benefit from PRP injections in the treatment of male AGA. There is a strong need for well-designed, randomized controlled trials with large sample size, proper control group, standard treatment protocols (concerning the amount, number and interval of PRP injections, method of preparation and activation, etc.), and long follow-up periods to evaluate the safety and efficacy of PRP in the treatment of male AGA.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

Sporotrichosis is a subcutaneous mycotic infection caused by Sporothrix schenckii that is acquired by traumatic implantation. The diagnosis is established by demonstration of fungal elements on histopathology and culture. Potassium iodide, azole antifungals, and terbinafine are the treatment options available. In this article, we report a 60-year-old female with lymphocutaneous sporotrichosis that responded well to potassium iodide. However, subclinical hypothyroidism (Wolff–Chaikoff effect) was encountered as a side effect of therapy which was managed with thyroxine replacement. Knowledge about the Wolff-Chaikoff effect (WCE) is important for the dermatologist and reinforces the need for screening and monitoring of thyroid stimulating hormone (TSH) in patients where long duration therapy is being planned.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

Background: Across the globe, skin disorders represent a frequent occupational concern for many health professionals including veterinarians and there is a serious impact of skin diseases on their lives and careers. But little is known about the prevalence and distribution of skin diseases (especially occupational) within this important professional group across Asia, especially India. Materials and Methods: The study was a cross-sectional study carried out over a period of one year in which veterinarians and veterinary workers of Kashmir valley were screened for various skin diseases and occupational dermatoses. Results: The study group comprised 910 veterinarians and associated workers working across the valley with the majority being males; 846 workers (93%). The mean age of the group was 38.53 years. Out of these, 267 veterinarians and associated workers (29.3%) were found to have skin lesions. Of the 267 cases, 165 (61.80%) had non-infectious lesions, while the rest had 102 (38.20%) had infectious skin diseases. The main non-infectious lesions included friction-related disorders, eczemas, pigmentary disorders, papulosquamous disorders, and many others; while the infectious lesions were of fungal, bacterial, viral, and parasitic etiology. Fungal infections, eczemas, and melasma were more common in them, indicating an occupational etiology. Conclusion: A huge group of skin diseases was seen in veterinarians and veterinary workers, with some diseases showing an occupational nature. To reduce the burden of skin diseases in this particular group, proper prevention measures need to be instituted at work places by veterinary governing bodies of the state.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

Background: Melasma is a characteristic pattern of facial hyperpigmentation, occurring primarily on the forehead, cheeks, and chin, in a mask-like distribution. The pathogenesis of melasma is not fully understood. Vitamin D plays a role in skin pigmentation. It exerts its effect through vitamin D receptor (VDR), which is expressed in variable cells including normal melanocytes. Aim and Objective: The aim of the current work was to investigate if VDR gene polymorphism (TaqI) confers susceptibility to melasma in Egyptian patients. Materials and Methods: A total of 45 female patients with melasma were recruited and 50 healthy subjects that were matched on age, sex, body mass index, and skin phototype, were included as a control group.TaqI polymorphism was investigated using restriction fragment length polymorphism polymerase chain reaction (RFLP PCR). Results: Presence of (t) allele and (tt) genotype was significantly associated with melasma cases compared with control group (P < 0.001 for both). No significant association was found between (tt) genotype or (t) allele and clinical data of the studied cases. Conclusion: TaqI polymorphism is associated with melasma. Further, large-scale studies are recommended to underscore and validate the current findings. It is also necessary for future studies to extend the research to other populations and ethnicities. Investigating other VDR gene polymorphisms in melasma is also warranted. Since melasma is a multifactorial disease, gene–gene and gene–environment interactions should be considered in future genetic-epidemiologic researches to apply more comprehensive insight into the role of VDR gene in its pathogenesis.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Background: Mean platelet volume (MPV) is an indicator of platelet activation and aggregation. MPV has been found to be related with the inflammation of certain disorders in recent studies. Aims: We sought to investigate whether MPV could be an indicator of inflammation in patients with Behçet disease (BD) and recurrent apthous stomatitis (RAS). Materials and Methods: Our study was designed as a retrospective case-control study and data was retrieved from our institutional database. We randomly generated a total of three study groups from our clinical archive. All controls were sex and age-matched and randomly selected by computer. Eighty-five patients with BD, 82 patients with RAS, and 721 healthy controls were included for the study design. We compared mean MPV values in the patient groups and controls. Results: We found no difference in the mean MPV value between BD group and BD-control group. The mean MPV value was significantly higher in patients with RAS than that in the RAS-control group (9.11 ± 1.01 fL vs. 8.76 ± 1.15 fL, P = 0.045). There was no difference in mean MPV level between BD and RAS group. Conclusion: The association between MPV and inflammatory skin diseases such as BD and RAS should be investigated prospectively in case-control studies. Limitations: Retrospective study design.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

Inflammatory myofibroblastic tumor (IMT) is a peculiar low-grade neoplasm of spindle cell fibroblasts and myofibroblasts in an inflammatory background. The lung is the most common site of involvement. Here, we report a case of paraneoplastic pemphigus (PNP)-associated with an extensive retroperitoneal IMT. The patient had a favourable response following treatment with a low dose of systemic steroid, mycophenolate mofetil, and intravenous immunoglobulin (IVIG). He subsequently underwent surgery for resection of the tumor with nephrectomy and five courses of IVIG were administered after surgery due to a minor relapse. He was in remission in her last follow-up visit 16 months after surgery. The occurrence of PNP with IMT is notable; early detection and treatment are crucial for this tumor-associated autoimmune disease.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

We report a case of mutilating keratoderma with alopecia and keratoses follicularis spinulosa decalvans (KFSD), which was initially diagnosed as ectodermal dysplasia and Olmsted syndrome but was revisited as a case of X-linked Olmsted (XLO) syndrome. We focus on this uncommon entity (XLO) to highlight the differentials of alopecia with palmoplantar keratoderma.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

Background: The evidence for efficacy of psychoeducational interventions in psoriasis is not well researched. Objective: The objective of this study was to assess the benefits of a group, multi-professional, psychoeducational training in psoriasis. Materials and Methods: Adults with moderate-to-severe psoriasis were randomized into psychoeducational intervention plus treatment (intervention arm [IA]) and treatment as usual alone (control arm [CA]) groups using random number tables. The primary efficacy measures were mean changes in Psoriasis Area Severity Index (PASI) and Dermatology Life Quality Index (DLQI) at 6months from the end of intervention. The secondary efficacy measures were the mean changes in the WHO-5 well-being index (WHO-5) and the subsets of the patient health questionnaire (PHQ), namely, PHQ-9, generalized anxiety disorder (GAD)-7, andPHQ-15. Datawere analyzed by intention-to-treat analysis. Results: One-hundred and three participants were randomized into IA (n = 54) and CA (n = 49). The prevalence of psychiatric disorders in the entire population was 26.2% and was similar in both groups.Following intervention, there was statistically significant improvement in the mean scores in PASI, DLQI, and WHO-5 in the IA unlike that seen in the CA. There was statistically significant improvement in PHQ-9 scores in IA and CA. The scores on PHQ-15 and GAD-7 did not show any change. Intergroup comparisons showed that PASI was reduced significantly in IA as compared to CA. More participants in CA dropped out of the study than in the IA. Limitations: Modest sample size and lack of blinding of the participants and the administrators were the limitations of this study. Conclusion: Group psychoeducational intervention resulted in overall improvement in both clinical and psychological outcome measures in psoriasis patients.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]  [PubMed]