Ageing, also called as senescence, is one of the most complex, intrinsic, biological processes of growing older and resulting into reduced functional ability of the organism. Telomerase, environment, low calorie diets, free radicals, etc., are all believed to affect this ageing process. A number of genetic components of ageing have been identified using model organisms. Genes, mainly the sirtuins, regulate the ageing speed by indirection and controlling organism resistance to damages by exogenous and endogenous stresses. In higher organisms, ageing is likely to be regulated, in part, through the insulin/insulin-like growth factor 1 pathway. Besides this, the induction of apoptosis in stem and progenitor cells, increased p53 activity, and autophagy is also thought to trigger premature organismal ageing. Ageing has also been shown to upregulate expression of inflammatory mediators in mouse adipose tissue. The understanding of pathophysiology of ageing over the past few years has posed tremendous challenges for the development of anti-ageing medicine for targeted therapy. Future research areas must include targeted role of systemic inflammatory markers such as C-reactive protein and interleukin 6 and other biochemical and genetic studies including gene signaling pathways, gene microarray analysis, gene modulation, gene therapy, and development of animal/human models for potential therapeutic measures and evaluations.

Chronic urticaria (CU) is a disturbing allergic condition of the skin. Although frequently benign, it may sometimes be a red flag sign of a serious internal disease. A multitude of etiologies have been implicated in the causation of CU, including physical, infective, vasculitic, psychological and idiopathic. An autoimmune basis of most of the 'idiopathic' forms is now hypothesized. Histamine released from mast cells is the major effector in pathogenesis and it is clinically characterized by wheals that have a tendency to recur. Laboratory investigations aimed at a specific etiology are not always conclusive, though may be suggestive of an underlying condition. A clinical search for associated systemic disease is strongly advocated under appropriate circumstances. The mainstay of treatment remains H1 antihistaminics. These may be combined with complementary pharmacopeia in the form of H2 blockers, doxepin, nifedipine and leukotriene inhibitors. More radical therapy in the form of immunoglobulins, plasmapheresis and cyclophosphamide may be required for recalcitrant cases. Autologous transfusion and alternative remedies like acupuncture have prospects for future. A stepwise management results in favorable outcomes. An update on CU based on our experience with patients at a tertiary care centre is presented.

Use of immunohistochemical technique is increasing in diagnosing various diseases. In many situations it may not be possible to differentiate entities with overlapping clinical and histopathological features. Immunostaining of cellular antigens is immensely helpful in such cases. Immunohistochemistry (IHC) has also been in use for targeted cancer therapy. In this article, the discussion will be restricted to use of IHC in dermatological disorders and use of classical antigens with brief updating of some important newly discovered antigens.

Background: Skin tags (ST) are common benign tumors of the skin but their etiopathogenesis is not well understood. STs arise in sites subjected to trauma. It was proved that mast cells are recruited to sites of skin trauma and increase their tumor necrosis factor-α (TNF-α) content. Aim: STs are linked to obesity and frictional sites, but this has not been studied at the molecular level. We hypothesized that mast cells, TNF-α and its family member, TNF-related apoptosis-inducing ligand (TRAIL) might play a role in the pathogenesis of STs as a response to trauma. Materials and Methods: A study was done on 15 patients with STs. Two STs and a snip of normal skin were obtained in each subject. We counted the mast cells after Toluidine blue staining. Enzyme-linked immunosorbant assay was used to measure TNF-α level while reverse transcriptase polymerase chain reaction was used to evaluate the level of TRAIL mRNA expression. Results: Mast cell count in all STs was significantly higher than that in control (P=0.0355). There was a highly significant increase in the level of TNF-α in all STs as compared to its level in controls (P<0.0001). Expression of TRAIL mRNA was significantly higher in STs as compared to its expression in controls (P<0.0001). Conclusion: Our study suggests that mast cells, TNF-α and TRAIL may play a role in the pathogenesis of STs.

Background: Vascular proliferation in the papillary dermis is considered to be an important and probably an early feature of psoriasis. Few morphometric studies have attempted to analyze the vascular changes. However, no study was found in the available literature comparing vascular changes between psoriasis and psoriasiform dermatitis. Materials and Methods : Skin biopsies from 25 cases each of psoriasis and psoriasiform lesions were immunohistochemically stained for CD34 (endothelial marker). Microvessel density (MVD), microvessel length density and ratio of microvessel area to papillary dermal area were calculated using image analysis software. Results: Skin biopsies from psoriasis showed higher staining for CD34 on light microscopy. Using morphometric techniques, microvessel length density was significantly higher in psoriasis compared to psoriasiform lesions (P value <0.05). MVD was also higher in psoriasis, though the difference was not significant. The ratio of microvessel area to dermal area was almost similar in both the groups. Conclusion : Our results indicate that vascular tortuousity and dilatation is significant only in psoriatic lesions. These results may assist in automated diagnosis of skin biopsies.

Background: Hidradenitis suppurativa is a chronic skin condition involving the apocrine glandular zones. Affected patients may present with acute abscesses, but the condition often progresses to a chronic state with persistent pain, sepsis, sinus tract, fistula formation, purulent discharge, and dermal scarring. The treatment of patients with severe disease can be difficult and may require complex surgical intervention. Materials and Methods: For this study, we selected 30 patients from the outpatient department. The patients were divided into two groups of 15 patients each. In patients of group I, oral acitretin 0.5 mg/kg body weight was given alone. Oral acitretin was given for a period of 12 weeks, and follow-up of the patients was done every 4 weeks for a period of 6 months. In patients of group II, oral acitretin 0.5 mg/kg was given plus a wide surgical excision was done. Results and Discussion: In our study, the commonest site of involvement of hidradenitis suppurativa was axilla in 83.3% patients, perineum was involved in 13.3% patients, and periumbilical involvement was seen in 3.3% patients. The commonest clinical feature was nodules seen in 90% patients; pain was seen in 60% patients, dermal scarring in 73.3% patients, malodorous discharge in 33.3% patients, abscess in 30% patients, and fistulous tracts were seen in 20% patients. The recurrence rate was low (20%) in group II patients in whom oral acitretin was given plus surgical excision was done as compared with group I (40%) in whom oral acitretin was given alone.

Background: Topical steroids remain the mainstay of treatment in eczema, an inflammatory skin reaction characterized by pruritus, redness, scaling, and clustered oozing papulovesicles. Halometasone is a new potent corticosteroid approved in the Indian market for topical application in the treatment of dermatitis. Aims: To evaluate the efficacy and safety of halometasone in the treatment of acute or chronic noninfected eczematous dermatosis in Indian population. Materials and Methods: A prospective, open, multicentric, phase 3, noncomparative clinical trial conducted at outpatient departments of seven centres. Two hundred endogenous eczema patients meeting study criteria were enrolled. Halometasone 0.05% cream was applied twice daily for 30 days in chronic and 20 days in acute eczema patients. Calculation of eczema area and severity index, and assessment of investigator's global assessment of severity of eczema and severity of pruritus score were done at each visit and compared with baseline. All adverse events (AE) were captured and documented. Laboratory investigations including haematological tests, urinalysis, renal and liver function tests were performed at baseline and at end of treatment. Results: Of the 200 patients enrolled, 180 were chronic and 20 were acute eczema patients. It was found that there was a significant (P<0.001) improvement in all efficacy parameters compared with baseline. The treatment was shown to be successful in 91% patients. AE were reported in 30 patients and there was no serious AE reported. There was no clinically significant difference in laboratory investigations with treatment. Conclusions: Halometasone was shown to be safe and very effective in Indian patients with acute and chronic eczema and the drug was well tolerated.

Background: Tinea corporis is a common superficial dermatophytosis seen in tropical countries. Newer molecules are constantly being introduced for its treatment. Topical clotrimazole is in vogue as the treatment for this condition for a long time. Amorolfine is a comparatively recently introduced drug for topical use in this condition. Aims: To assess the effectivity and safety of amorolfine 0.25% cream in patients with tinea corporis, in comparison to clotrimazole 1% cream. Materials and Methods: Patients presenting with symptoms of tinea corporis were mycologically confirmed for the presence of fungal hyphae. They were randomly divided into two groups: one group received amorolfine and the other received clotrimazole. Treatment duration was for 4 weeks and study duration was for 8 weeks. Evaluation was carried out using the standard clinical parameters on day 1, day 14, day 28 and a follow-up on day 56. Adverse effects were also recorded. Data entry was done in Excel datasheet and analyzed with Epiinfo 2002. Chi-square test and t-test were used according to the type of data. Results: The patients of the two groups were matched at baseline in respect to their demographic profile. Analysis of collected data showed significant improvement in both the groups, suggesting that both the drugs were effective agents in tinea corporis infection. Between-groups comparison of mycological cure rate and clinical improvement showed no significant difference. Conclusion: Amorolfine 0.25% cream is found to be safe and effective, like clotrimazole, when used topically in tinea corporis.

Background: Nevus of Ota is common condition in Indian patients. The condition is more common in females, with a male-female ratio of 1:4.8. Aim : To evaluate long-term efficacy safety and stability of Q-switched ND:YAG laser in treatment of Nevus of Ota. Design: 6 month follow-up of patients of Nevus of Ota, treated with Q-switched ND:YAG laser Materials and Methods : Twenty-five patients of Nevus of Ota were treated with Q-switched ND:YAG laser for a period of 1 year and 9 months; patient had fitzpatricks skin type 4 and 5; detailed history, clinical examination, ophthalmoscopy, and otoscopy was done in all cases; clinical photographs were taken before and after the completion of treatments. Six-month follow-up was done after the last session. Response to treatment was graded based on physician's global assessment. Result : More than 70% improvement was seen in 15 patients (60%). Eight patients (32%) had moderate and two patients (8%) showed mild improvement.

Background: Burn surgeons use autologous skin graft technique for patients, but a challenge remains for large surface wounds. Recently, a method was described which used a small piece of skin to cover a 70 times greater surface by spraying epidermal cells on injured skin. We designed a comparative study to find the best method to make an epidermal cell suspension. Materials and Methods : Eleven discarded skin samples were sent to our laboratory from Ghotboddin Burn Hospital, Shiraz. Each sample was sliced into four small pieces (1 cm ² ) and each piece was treated with a different chemical including sodium bromide (2N) and (4N), ammonium hydroxide (2N), and trypsin (0.05%) for 20 minutes. The epidermis and dermis were separated using forceps. Trypsin was added to all samples (except the trypsinized sample) to begin the intercellular detachment. Afterward, epidermis was sliced into small pieces followed by filtration and centrifugation. Cells were counted using hemocytometer. Identification of keratinocytes and melanocytes was made through immunocytochemical staining for cytokeratin and melanosome antigens, respectively. Results: There was a significant difference in alive cell counts comparing cells obtained from NaBr (4N) method to other methods. Considering total cell count and alive cell count, NaBr (4N) yielded the most cells. Immunocytochemical staining showed that in all methods, some cells are stained positively for cytokeratin antibody and some for melanosome antibody. Conclusion: Although recent papers had advised trypsin method to make a cell suspension to use for burn patients, we found that NaBr (4N) method yields more alive cells and less toxicity.

Background: Basal cell carcinoma (BCC) and squamous cell carcinoma (SCC), in combination, are referred to as nonmelanoma skin cancers (NMSCs). NMSC is not as extensively studied in the Asian population as it is in the Caucasian population. Aim: This study sought to evaluate the clinical and histopathologic aspects of NMSC from cases of cutaneous malignancies. Materials and Methods: The present study is a descriptive analysis of NMSC specimens seen at Department of Pathology, SSIMS and RC, Davangere. Histologically diagnosed NMSC, i.e. BCC and SCC specimens from January 2005 to December 2009 were analyzed according to site distribution, risk factors and histological variants. Results: Of the various specimens received during the 5year study period, 60 were histologically categorized as skin malignancies, of which 31(51.6%) cases were of NMSC. SCC was the most common NMSC constituting 26 (83.9%) cases and 5 NMSC cases (16.1%) were of BCC. The most common incidence was among the age group 60-80 years (80%) for BCC and 40-60 years (50%) for SCC. Head and neck was the most common site of presentation with predilection for face. Forty-six percent of SCC was histologically categorized as well differentiated, 42.3% as moderately differentiated and 11.5% as poorly differentiated. Most common histological variant of BCC was solid (nodular) type. Conclusion: NMSC often associated with greater morbidity, necessitating increased efforts to assess risk factors in individuals, to encourage periodic self-examination and professional evaluation of skin and to optimize strategies for earlier diagnosis and treatment.

Background : Most atopic dermatitis (AD) patients have elevated serum immunoglobulin E (IgE). Impaired folic acid (FA) metabolism was found to reduce the intracellular methyl donor pool, associated with a higher prevalence of atopy. Aim : To assess serum IgE and FA in AD patients and to correlate their levels with the disease severity, and with each other. Materials and Methods : Twenty patients with AD were assessed for serum FA and IgE, compared with 20 age- and sex-matched controls. Patients were classified into three groups (mild, moderate, and severe AD) based on clinical severity according to Nottingham index. In both patients and controls, serum IgE was measured using Enzyme-linked immunosorbent assay technique and serum FA was measured using Microparticle Enzyme Immunoassay technique. Results : Serum FA levels were lower in AD patients compared with controls, but the difference was not statistically significant. FA levels did not show statistically significant difference among disease severity groups and did not correlate with serum IgE levels. On the other hand, serum IgE levels were significantly elevated in AD patients compared with controls, and among AD patients, its levels were significantly elevated in severe AD compared with mild and moderate disease. Conclusion : Serum IgE is useful in assessment of AD severity and activity. FA contribution to AD needs further investigations.

Background: Acanthosis nigricans (AN) is a dermatosis characterized by thickened, hyperpigmented plaques, typically on the intertriginous surfaces and neck. Common in some populations, its prevalence depends on race. Clinicians should recognize AN; it heralds disorders ranging from endocrinologic disturbances to malignancy. In this review, we discuss the pathogenesis of AN and its clinical implications and management. Materials and Methods: We selected 30 patients for the study. Diagnosis of associated disorders was established by history, physical examination, body mass index (BMI), hormone measurements by radioimmunoassays of thyroidnfunction tests, free testosterone, 17 (OH) progesterone, dehydroepiandrosterone sulfate (DHEAS), cortisol, gonadotropins, prolactin, immunoreactive insulin, and C-peptide levels. Results and Discussion: In our study, the flexural involvement (flexures of groins, knees and elbows) was seen in 40% patients, lip involvement was seen in 6.6% patients, and dorsal involvement was seen in 3.3% patients each. Increased serum testosterone levels were seen in 13.3% patients and increased DHEAS levels were seen in 20% patients. Regarding the types of AN, obesity induced AN or pseudo-AN was seen 70% patients, syndromic AN was seen in 23.35% patients and malignant AN was seen in 6.6% patients. The commonest histopathological feature of patients with AN was hyperkeratosis, seen in 100% patients, papillomatosis was seen in 90% patients, dermal infiltrate of lymphocytes and plasma cells was seen in 60% patients, horn pseudocysts were seen in 30% patients, and irregular acanthosis was seen in 26.6% patients.

Background: Cicatricial alopecias have a significant impact on the psychological status, quality of life, and social interaction of those suffering from it. Till date, limited or no data have been available regarding the psychosocial and quality of life aspects of cicatricial alopecias. Aims: To assess the psychosocial impact of cicatricial alopecias. Materials and Methods: Thirty patients fulfilling the criteria for cicatricial alopecia irrespective of their age and sex were included in the study. Psychosocial assessment was carried out in 23 patients who were capable of responding to the questionnaire, using an adopted and suitably modified version of Women's Androgenetic Alopecia Quality of Life Questionnaire. Results : We observed that 73.9% of our patients with cicatricial alopecias had moderate to severe psychosocial impact due to their hair loss. Patients of younger age group and with inactive disease, suffered from greater psychosocial impact of the disease. Patients with slight hair loss also had considerable psychological distress. The chronicity of disease duration did not seem to reduce the psychosocial impact of the disease. Both married and unmarried patients suffered equally from the psychosocial impact of the disease. Conclusion: The management of cicatricial alopecias needs a holistic approach. In addition to laying an emphasis on early diagnosis aided by clinco-pathological correlation, to prevent irreversible hair loss, the psychosocial impact of the disease should also be taken into consideration and addressed by the treating dermatologist.

Background: Although psoriasis generally does not affect survival, it certainly has a number of major negative effects on patients, demonstrable by a significant detriment to quality of life. Aims: We have done a study with the objective of assessing the clinical variables adversely affecting quality of life in patients diagnosed with psoriasis. Materials and Methods: This study is a cluster analysis of 50 consecutive consenting patients with psoriasis, of both sexes, aged over 18 years, attending the dermatology outpatient department of a tertiary care center. We measured the clinical severity using psoriasis area severity index and health-related quality of life using psoriasis disability index (PDI). Statistical analysis was performed using unpaired independent student's t-test, analysis of variance (and Scheffe's post hoc test as appropriate) and Pearson's correlation coefficients. Results: There was a significant correlation between the physician-rated severity of psoriasis and the extent of impact of psoriasis on physical disability as measured by the PDI. We have identified that a younger age at onset of disease and self-reported stress exacerbators suffer greater disability in most aspects of quality of life. Conclusions: On the basis of this study, we would recommend that psoriasis patients especially with severe disease require a more holistic treatment approach that encompasses both medical and psychological measures.

Background: Psoriasis is a chronic skin disease that affects 1 to 3% of the population in most industrialized countries. It is commonly associated with a variety of psychological problems including low self-esteem, depression, suicidal thoughts, and sexual dysfunction. Materials and Methods : We have performed a pilot study in which we have tried to assess the impact on sexual dysfunction in patients with psoriasis who have started treatment with biological therapy using validated indexes in Spanish: International Index of Erectile Function for men and female sexual function index in women. Results : Considering the men and women from our study, an improvement in FSFI by an average of 9.5 and 6.3 points is observed, respectively. Conclusion: We considered our series as a first step for a more detailed approach to the study of sexual function in patients with psoriasis.

The increasing recognition of occupational origin of airborne contact dermatitis has brought the focus on the variety of irritants, which can present with this typical morphological picture. At the same time, airborne allergic contact dermatitis secondary to plant antigens, especially to Compositae family, continues to be rampant in many parts of the world, especially in the Indian subcontinent. The recognition of the contactant may be difficult to ascertain and the treatment may be even more difficult. The present review focuses on the epidemiological, clinical and therapeutic issues in airborne contact dermatitis.

Occupational and recreational plant exposure on the skin is fairly common. Plant products and extracts are commonly used and found extensively in the environment. Adverse reactions to plants and their products are also fairly common. However, making the diagnosis of contact dermatitis from plants and plant extracts is not always simple and straightforward. Phytodermatitis refers to inflammation of the skin caused by a plant. The clinical patterns may be allergic phytodermatitis, photophytodermatitis, irritant contact dermatitis, pharmacological injury, and mechanical injury. In this article, we will focus mainly on allergy contact dermatitis from plants or allergic phytodermatitis occurring in Asia.

Airborne-contact dermatitis (ABCD) represents a unique type of contact dermatitis originating from dust, sprays, pollens or volatile chemicals by airborne fumes or particles without directly touching the allergen. ABCD in Indian patients has been attributed exclusively by pollens of the plants like Parthenium hysterophorus, etc., but in recent years the above scenario has been changing rapidly in urban and semiurban perspective especially in developing countries. ABCD has been reported worldwide due to various type of nonplant allergens and their clinical feature are sometimes distinctive. Preventive aspect has been attempted by introduction of different chemicals of less allergic potential.

Eyebrow threading is a practice of shaping the eyebrows. Many dermatological complications have been briefly mentioned in various publications. There are scant data regarding the appearance of molluscum in the line of eyebrows after a session of threading. We report a series of eight patients both males (3) and females (5) who had lesions of molluscum in the eyebrow region after threading. The earlier reported cases are only among the females. The present study is highlighting the appearance of molluscum in the region of eyebrow after a session of threading from beauty salon. So, to the best of our knowledge, this is the first report of its kind describing same pathology in males. The rational of reporting this case series is to create awareness among the dermatologists as well as in general population about potential hazards of threading.

Churg-Strauss syndrome (CSS) is a rare granulomatous necrotizing small vessel vasculitis characterized by the presence of asthma, sinusitis, and hypereosinophilia. The cause of this allergic angiitis and granulomatosis is unknown. Other common manifestations are pulmonary infiltrates, skin, gastrointestinal, and cardiovascular involvement. No data have been reported regarding the role of immune complexes or cell mediated mechanisms in this disease, although autoimmunity is evident with the presence hypergammaglobulinemia, increased levels of IgE and Antineutrophil cytoplasmic antibody (positive in 40%). We report the case of a 27-year-old lady presenting with painful swelling of predominantly lower limbs with extensive vesicles and ecchymotic patches and fever shortly after stopping systemic steroids taken for a prolonged duration (2002--2010). The aim of this case report is to point to the possibility of CSS in patients presenting with extensive skin lesions masquerading as Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Syndrome (SJS/TENS).

An elderly man from the region of Ladakh presented with recurrent episodes of lower respiratory tract infection, rapidly progressive Acanthosis nigricans, Acanthosis palmaris and plantar keratoderma. Detailed investigations revealed underlying metastatic transitional cell carcinoma of the bladder. This case is being reported for its rarity in the literature.

Cancer metastases represent the most devastating aspect of malignancy, since the mortality of cancer patients is mainly related to the metastatic behavior of the primary neoplasm. Skin metastases are usually late events in the course of tumor progression. Excluding melanoma, the most common tumor to metastasize to the skin is breast cancer. Patients who develop cutaneous metastases rarely present with a zosteriform distribution. Herein, we present a 60-year-old female, an undiagnosed case of breast cancer, with zosteriform metastases along her right T2-T3 dermatome.

Primary cutaneous leiomyosarcoma of the skin is a rare soft tissue neoplasm, accounting for about 2-3% of all superficial soft tissue sarcomas. It arises between the ages of 50 and 70 years, and shows a greater predilection for the lower extremities. Clinically, it presents with solitary, well-circumscribed nodule and, microscopically, consists of fascicles of spindle-shaped cells with "cigar-shaped" nuclei. Local recurrence is known in this tumor. We document a case of primary cutaneous leiomyosarcoma in a 77-year-old man and discuss the histological features and immunohistochemical profile of this uncommon neoplasm.

Juvenile hyaline fibromatosis is a rare, autosomal-recessive disease characterized by papular and nodular skin lesions, gingival hyperplasia, joint contractures and bone involvement in variable degrees. It is a connective tissue disorder with aberrant synthesis of glycosaminoglycans by fibroblasts. We report a 5-year-old female born of first-degree consanguineous marriage who presented with multiple, recurrent, painless, variable-sized nodules. Fine needle aspiration cytology smears and the subsequent histopathological examination from the nodules showed benign spindle cells in a Periodic acid Schiff-positive myxoid background. The disease has a relentlessly progressive course, with most patients surviving only up to the 4 ^th decade. As of now, there is no specific treatment for this disorder. Genetic counseling is essential to explain to parents about a 25% chance of having a diseased baby in any pregnancy. With the gene being mapped recently, techniques for antenatal diagnosis are likely to be established.

Neonatal lupus erythematosus (NLE) is an autoimmune disease affecting the fetus as a result of transplacental transfer of anti-Ro autoantibodies. Typically, it presents in the first few months of life with an annular form of subacute cutaneous lupus erythematosus. We report an unusual case of NLE presenting at birth with scaly erythematous telangiectatic patches and macules with skin atrophy involving the face, head, and upper trunk. Thrombocytopenia was discovered on laboratory investigations. Histopathology of skin biopsy was consistent with subacute cutaneous lupus. The mother was clinically free of disease and had no family history of autoimmune disease. Serology (extra-nuclear antigens) was positive in both the baby and the mother. This is a rare presentation of a rare disease.

An 8-year-old male patient who had been diagnosed as acute lymphoblastic leukemia (ALL) 4 years ago and received 120 cures of chemotherapy presented at our clinic complaining of spots on his body 3 months after the end of chemotherapy. Anamnesis of the patient revealed that the lesions started 3 months after the last cure of chemotherapy on the abdomen and spread throughout the body. It was learnt that the number of spots increased very rapidly in a period of 2 months, and exceeded 100. A dermatological examination of the patient showed 142 hyperpigmented macules, the largest of which was 1 cm x 1 cm and the smallest was 0.2 cm x 0.2 cm in size, diffusely scattered to the skin and oral mucosa. When the biopsy material taken from the lesions was histopathologically examined, nest structures composed of nevus cells with oval nuclei and eosinophilic cytoplasm starting from the dermoepidermal junction and extending toward the lower epidermis were observed and found to be consistent with the compound nevus. We present this rare case who was diagnosed as "eruptive compound nevus" on the basis of clinical signs, ALL diagnosis, chemotherapy history and histopathologic evaluation.

Papillon-Lefevre syndrome (PLS) is a rare disease characterized by skin lesions, which includes palmar-plantar hyperkeratosis and hyperhidrosis with severe periodontal destruction involving both the primary and the permanent dentitions. It is transmitted as an autosomal-recessive condition, and consanguinity of parents is evident in about one-third of the cases. This paper describes a 13-year-old male patient who presented to the department of pedodontics, with rapidly progressing periodontitis. A general physical examination revealed scaling on the hands and feet, which had been medically diagnosed as PLS. The incidence of this rare entity is increasing in the recent times, which is associated with irreparable periodontal destruction at an early age, with not so prominent skin lesions in some cases. In such instances, the dentist has a more important role in diagnosing, treatment planning and preservation of the periodontal tissues and, at the same time, referring for the treatment of the skin lesions. This paper emphasizes the combined effort of the two specialities in order to maintain skin as well as dental conditions in health by early intervention and a synergistic treatment approach.

We report a case of 15-year-old girl who was diagnosed as a case of pituitary macroadenoma on computed tomography and magnetic resonance imaging (MRI) scans for her complains of diminished vision in the left eye and headache. On investigation she was found to have optic atrophy of left eye and primary hypothyroidism. She was started on thyroxin therapy in October 2008 and planned for transcranial excision of the tumor. Just before the date for planned surgery she developed mildly itchy vesico-bullous rash and the surgery was postponed. On histopathology and immunoflourescence studies it was confirmed to be the rash of dermatitis herpetiformis (DH) and treatment was started with corticosteroids in the last week of December 2008 and dapsone was added from mid January 2009. The lesions responded dramatically and within 6 weeks of starting treatment, she had become asymptomatic except for persisting diminution of vision. Repeat MRI scans in mid-February 2009 revealed total regression of pituitary mass. The case is being reported for interesting association of primary hypothyroidism, pituitary mass mimicking macroadenoma and DH; its successful outcome with medical management in the form of total regression of pituitary mass, thereby avoiding an unnecessary surgery.

We are reporting a very unusual case of unilateral lower limb gangrene in 12 days neonate, who was brought to us with bluish discoloration of left foot with gangrene of toes. A negative sepsis screen, normal Doppler study, normal magnetic resonance angiography and absence of evidence of hypercoagulable state prompted us to make the diagnosis of idiopathic unilateral lower limb gangrene in this newborn. Because of the rarity we are reporting this case with vide review of literature.

A 2-month-old infant presented to our emergency department with fever, altered consciousness, and focal seizures of acute onset. He had vesicular skin lesions over the right preauricular region. CT brain showed a large hypodense lesion involving the left temporo-parietal region, left basal ganglia and left thalamus. MRI brain revealed bilateral multifocal corticomedullary lesions suggestive of encephalitis. CSF-PCR was positive for herpes simplex virus (HSV) type I. He was treated with standard dose intravenous acyclovir for 15 days along with a trial of pulse methylprednisolone, but was readmitted within a week with features of an early relapse. The infant survived but developed significant neurological sequelae. Although treatment of HSV is available, the neurological outcome is guarded even with adequate antiviral therapy. Adjunct corticosteroid therapy did not appear to attenuate the neurological sequelae.

Tumor necrosis factor-alpha (TNF-α) inhibitors, such as etanercept, infliximab, and adalimumab, bind to TNF-α and thereby act as anti-inflammatory agents. This group of drugs has been approved for the treatment of rheumatoid arthritis, psoriatic arthritis, moderate to severe plaque psoriasis, ankylosing spodylitis, Crohn disease, and juvenile idiopathic arthritis. We describe a 56-year-old woman who developed an erythematous pruritic rash on both arms-diagnosed as granuloma annulare by skin biopsy-approximately 22 months after initiating adalimumab for treatment of rheumatoid arthritis. On stopping adalimumab there was total clearance of the skin lesions, but a similar rash developed again when her treatment was switched to another anti-TNF agent (etanercept). This clinical observation supports a link between TNF inhibition and the development of granuloma annulare.

Sturge-Weber syndrome is a rare sporadic condition of mesodermal phakomatosis, characterized by purple-colored flat cutaneous cranial (face) hemangiomas (most commonly along the trigeminal nerve), glaucoma and vascular lesions in the ipsilateral brain and meninges. Klippel-Trenaunay syndrome is also an uncommon mesodermal phakomatosis characterized by a triad of cutaneous and visceral hemangiomas, venous varicosities and soft tissue or bone hypertrophy. Sturge-Weber syndrome in combination with Klippel-Trenaunay syndrome is unusual. Because of the rarity, we report here a 3-year-old boy who presented with overlapping features of both the syndromes.

A retrospective analysis was conducted of eight cases of Wegener's granulomatosis (WG), who presented with cutaneous lesions. The clinical, immunopathologic and histopathologic features of the cutaneous lesions were reviewed. Antineutrophil cytoplasmic antibody (ANCA) status of the patients was established. When possible, a comparison of immunofluorescence findings of skin biopsies was made with those of renal biopsies taken at the same time. In all except one, systemic and cutaneous disease developed concurrently. On histopathology, leukocytoclastic vasculitis was noted in five patients and features of lupus erythematosus and pyoderma gangrenosum in one case each. Four patients showed immunoglobulin deposits in subepidermal blood vessel walls, while one patient showed granular immune deposits at dermo-epidermal junction only. Immunoglobulin G was the most common immunoreactant detected. C-ANCA/proteinase 3 (PR3)-ANCA was positive in six patients, P-ANCA/myeloperoxidase (MPO)-ANCA in one patient, while one patient did not show ANCA positivity on indirect immunofluorescence. All four renal biopsies showed pauci-immune glomerulonephritis, irrespective of the presence (n=3) or absence (n=1) of immune deposits in the skin biopsy. Skin manifestations are encountered in nearly half of the patients with WG, thus it is important to be familiar with cutaneous histopathologic as well as immunofluorescence findings in WG patients.

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome reflects a serious hypersensitivity reaction to drugs, and is characterized by skin rash, fever, lymph node enlargement, and internal organ involvement. So far, numerous drugs such as sulfonamides, phenobarbital, sulfasalazine, carbamazepine, and phenytoin have been reported to cause DRESS syndrome. We report a case of a 10-year-old girl who developed clinical manifestations of fever, rash, lymphadenopathy, hypereosinophilia, and visceral involvement (hepatitis and pneumonitis) after taking phenobarbital for seizures, with subsequent development of sepsis, acute respiratory distress syndrome (ARDS) and spontaneous air leak syndrome (pnemothorax and pneumomediastinum). She was put on steroids and various antibiotics and was ventilated, but ultimately succumbed to sepsis and pulmonary complications.

Neural fibrolipoma is an uncommon tumor-like lesion that involves the upper extremity and usually arises in the median nerve. It is associated with macrodactyly in one-third of the cases. A 3-year-old girl presented with increasing size of fingers of both the hands since birth. Clinical examination revealed macrodactyly of two fingers of the right hand and three fingers of the left. Surgical reduction was performed and microscopy of the biopsy specimen established the diagnosis of neural fibrolipoma. Knowledge of the clinicopathological features is necessary for accurate diagnosis and treatment of this rare entity.

To emphasize that solitary hydatid cyst can be localized in the soft tissue and present as a soft tissue mass even in an unusual site like face, we report the case of a 42-year-old male patient presenting with a slowly growing mass in right temporal region. Computed tomography (CT) scan showed an encapsulated mass with multiple cysts. Histopathological examination revealed the characteristic findings, which were consistent with soft-tissue hydatid disease. In the absence of visceral organ involvement, this is the first reported case of a primary subcutaneous hydatid cyst in the skin of face in India. In the English literature, only one case of this kind has been reported till date. When imaging methods confirm cystic nature of a swelling, even in unusual sites, one should always keep a possibility of hydatid cyst and manage accordingly during surgery to avoid precipitation of acute anaphylaxis.