Atopic dermatitis is a well-recognized clinical entity, several facets of which continue to be mystified. Accordingly, its etio-pathogenesis is largely elusive. It appears to be an outcome of interplay of several undertones, namely: genetics, maternal factor and inheritance, pregnancy/intrauterine, environmental factors, immune dysregulation, immuno-globulins, role of diet, and infection. Besides, recent innovative breakthroughs consisting of nutritional supplementation, the highlights of which were considered worthwhile to take stock of to define its current status. An endeavor to enlighten the audience has been made for their benefit.

Genus Malassezia comprises of 14 species of "yeast like fungi," 13 of which are lipophilic and 1 is nonlipophilic. They are known commensals and in predisposed individuals they commonly cause a spectrum of chronic recurrent infections. They rarely also cause serious illnesses like catheter-related blood stream infections, CAPD associated peritonitis etc., Though these fungi have been known to man for over 150 years, their fastidious nature and cumbersome culture and speciation techniques have restricted research. Since the last taxonomic revision, seven new species have been added to this genus. Their ability to evade the host immune system and virulence has increased the spectrum of the diseases caused by them. These agents have been implicated as causal agents in common diseases like atopic dermatitis recently. Though culture-based research is difficult, the new molecular analysis techniques and facilities have increased research in this field such that we can devote more attention to this genus to study in detail, their characteristics and their growing implications implications in the clinical scenario.

Background: The mycological study of pityriasis versicolor is usually done by potassium hydroxide (KOH) mount and culture. However, KOH mount lacks a color contrast and requires a trained eye to interpret, while culture is difficult to perform, time consuming and has low sensitivity. Chicago Sky Blue 6B (CSB) is a new contrast stain that highlights the fungal hyphae and spores, blue against a purplish background. Aims and Objectives: This study was done to compare the utility of a novel contrast stain (CSB stain) with KOH mount and culture. Materials and Methods: Skin scrapings from the lesions of 100 clinically diagnosed cases of P. versicolor were subjected to (1) KOH mount and CSB stain for direct microscopic examination and (2) culture using Sabouraud's dextrose agar. The statistical analysis of CSB stain and culture was done using KOH mount as the reference method, as it is the most commonly performed and practical diagnostic test available for P. versicolor. An interrater reliability analysis using the Cohen's Kappa statistic was performed to determine consistency (agreement) among the different modalities. Observations and Results: Direct microscopy with CSB stain, KOH mount and mycological culture showed positive results in 98 (98%), 92 (92%) and 56 (56%) patients, respectively. Using KOH mount as the reference method, CSB stain had a sensitivity of 100% which was significantly higher than culture (60.9%). Statistically significant fair agreement was found between CSB stain and KOH mount (94% with κ=0.38, P < 0.001). Negligible agreement was found between CSB stain and culture (66%, κ=0.199, P = 0.001) as well as between KOH mount and culture (64%, κ=0.051, P = 0.107). Conclusion: CSB staining of skin scrapings is the most sensitive method for the diagnosis of pityriasis versicolor. Due to the distinct contrast provided by CSB, it is easy to perform, rapid and qualitatively superior to KOH mount.

Background: Tumor necrosis factor-alpha (TNFα) is an important inflammatory mediator in psoriasis and several genetic polymorphisms of this cytokine have been reported. Majority of studies have focused on the increased G- A polymorphism at the -308 position in psoriasis. There has been no comprehensive study evaluating the genetic polymorphisms, TNFα expression in the skin and histopathology. We are undertaking this study to outline TNFα genetic polymorphisms, its skin expression and histopathological correlation to help determine its role at the genetic and protein level. Materials and Methods : 112 patients of psoriasis and 243 healthy controls were included in this prospective study. 5 ml of peripheral blood was collected to study the TNFα genetic polymorphisms by polymerase chain reaction and restriction fragment length polymorphism analysis. Histopathological analysis of biopsies from the 112 patients were done using visual analogue scale and correlated with the findings. 61 of these cases were analyzed for TNFα expression by immunohistochemistry. The results of study were statistically analyzed using SPSS 13.0 statistical package program. Results: A strong association of TNFα -308 G/A polymorphism in psoriasis cases was detected. The A allele of the TNFα -308 G/A polymorphism occurs rarely in the Indian population, however there is an over representation of this allele in psoriatic patients. There was no association seen between TNFα genotype and histopathological severity of psoriasis. Conclusion: The study emphasized the central role of TNFα in the pathogenesis of psoriasis. TNFα genotyping may be helpful in identifying subjects in whom anti-TNFα therapeutic strategies may be tried.

Background: Genomic reduction helps obligate intracellular microbes to survive difficult host niches. Adaptation of Mycobacterium leprae in cases of pure neural leprosy (PNL) in the intracellular niche of peripheral nerves can be associated with some gene loss. Recently, a stable but variable number of tandem repefzats (TTC) have been reported in strains of M. leprae. FolP and rpoB genes are the two common mutation sites which deal with the susceptibility of the bacteria to drugs. Aim: We attempted to find if genomic reduction of M. leprae in context of these TTC repeats or mutations in folP1 and rpoB can be the reason for the restriction of M. leprae in the nerves in PNL. Materials and Methods: DNA extracts taken from fine needle aspiration of affected nerves of 24 PNL cases were studied for tandem repeats with 21TTC primer in multiplex-PCR. Mutations were also studied by PCR Amplification of SRDR (Sulphone Resistance Determining Region) of the folP1 and multiple primer PCR amplification refractory mutation system (MARS) of the rpoB. Results: Of the 24 PNL, only 1 patient showed mutation in the rpoB gene and none in the folp1 gene. Studying the mutation in TTC region of the M. leprae gene we found that all the cases have a loss of a few bases in the sequence. Conclusion: We can conclude that there is consistent loss in the bases in the TTC region in all cases of pure neural Hansen and we postulate that it may be an adaptive response of the bacteria to survive host niche resulting in its restriction to peripheral nerves.

Background: Basal cell carcinoma (BCC) is the most common skin cancer among whites, and several risk factors have been discussed in itsdevelopment and progress. Detection of human papilloma virus (HPV) deoxyribonucleic acid (DNA) BCCs in some studies suggests that the virus may play a role in the pathogenesis of this disease. Several molecular studies showed conflicting reports. Aims: The purpose of this study was to investigate the association between HPV and BCC using polymerase chain reaction (PCR). Materials and Methods: HPV DNA detection was done for 42 paraffin-embedded tissue specimens of BCC and 42 normal skin samples around the lesions by PCR using GP5+/GP6+ primers. Results: HPV DNA was not found in any of the 42 samples of BCC, and only one normal skin sample around the lesions was positive for HPV DNA by PCR. Conclusion: In this study, no statistically significant difference was seen between the presence of HPV DNA in BCC and normal skin around the lesion, and HPV is not likely to have an important role in pathogenesis of BCC.

Background: Although various therapies used for the treatment of psoriasis are able to produce remission, yet relapses, a common problem, remains frequent. It was observed that treatment with intermittent high dose (IHD) and continuous low dose (CLD) azathioprine can produce prolonged and durable remission in psoriasis. Aims : To see the long term effect of azathioprine pulse therapy (APT) in psoriasis. Methods: Ten patients with psoriasis who has completed more than 5 years in remission with azathioprine pulse therapy are being taken in the study. They were given IHD azathioprine (500 mg on 3 consecutive days which is repeated every month) with CLD azathioprine (100 mg orally) daily in between IHD. The entire treatment schedule was divided into four phases. During phase I, treatment with IHD and CLD azathioprine was started and continued till complete clearance of lesions after which, patients proceeded to Phase II. In phase II, they continued to get treatment with IHD and CLD. After continued remission for a period of nine months, they entered into phase III, when the treatment with IHD was stopped, but CLD continued. If there was no recurrence after nine months of phase III treatment, CLD was also withdrawn, and patients were followed-up without any treatment (Phase IV). Results: All 10 patients completed treatment and are in remission for more than five years without any treatment. Conclusions: Out of 60 patients in phase IV, 10 patients were in continuous remission for more than five years, after all treatment had been stopped. Thus, azathioprine pulse therapy regimen produces prolonged remission in psoriasis.

Background: Fractionated carbon dioxide (CO ₂ ) can treat hypopigmented scars. Latanoprost is a prostaglandin analog used to treat glaucoma. It can cause adverse effects, such as periocular hyperpigmentation. The aim of this study was to assess the efficacy and safety of latanoprost plus CO ₂ laser on the repigmentation of hypopigmented scars. Patients and Methods: 28 patients with hypopigmented scars were divided randomly into two groups. The patients in group A were treated in six sessions (1-month intervals) with 10600-nm fractional CO ₂ laser plus latanoprost 0.005% and those of group B fractionated CO ₂ laser plus placebo (distilled water). Digital photographs were taken at baseline and 3 months after the last treatment session. The blinded dermatologist compared the photographs and evaluated the efficacy of treatment in the hypopigmented scars using a 4-scale point (grade 1-4). Patient satisfaction was scored from 0 to 10 on a visual analog scale. Results: Follow-up results 12 weeks after the last treatment session demonstrated that 11 of the 14 patients in group A had more than 50% improvement in hypopigmentation The difference in improvement of the two groups was statistically significant (P = 0.027). The mean of the VAS scores of patients in group A was 6.50 ± 1.45 and in group B 4.57 ± 1.6. The difference in mean satisfaction of the two groups was statistically significant (P = 0.003). Side effects were mild and resolved within 1 to 5 days. Conclusion: The fractional CO ₂ laser resurfacing plus topical latanoprost can be used as a safe and efficacious method to treat hypopigmented scars.

Background: Suction blister epidermal grafting (SBEG) is one of the most commonly performed types of vitiligo surgery for stable vitiligo. The advantages of SBEG include cost-effectiveness and a relatively easier learning curve for the surgeon. Aims: To evaluate the outcome in terms of both recipient and donor site changes, on long-term follow-up of the patients who underwent SBEG in our center. Methods: Thirty patients, 21 females and 9 males ages ranging from 9 to 55 years, all having either stable vitiligo not responding to medical line of treatment, were included in the study done which involved a variable follow-up period ranging from 2 to 62 months (mean 23.6, standard deviation 17.79). SBEG was done as day care procedure. The patients were reviewed after 1-week and thereafter followed-up in the subsequent months and years. The results of the procedures were graded as poor (0-24%), fair (24-64%), good (64-94) and excellent (95-100%) depending on the patient satisfaction. Donor site changes were also analyzed. Results: The face and lips showed an excellent result and color match and persistent pigment retention. The larger areas, especially the lesions on the limbs showed comparatively less response. Of the total, 6.7% showed poor, 13.3% fair, 30% good and 50% excellent response to treatment. Patient satisfaction wise, 53.3% of the patients were very happy, 26.7% were happy, 10% satisfied and 10% unhappy. Significant positive correlation between patient satisfaction and physician observation was seen (Spearman's rho 0.866). Conclusions: In spite of recent advances in surgical modalities like cellular grafting, SBEG continues to be a good, cost-effective, surgical method of treating vitiligo especially on the face and lip. The donor site also tends to show good healing tendency with minimal scarring and postinflammatory pigmentation.

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a part of the spectrum of CD30 ⁺ cutaneous lymphoproliferative disorder, characterized by variable degrees of CD2, CD3, CD4 and CD5 expression by lymphoid cells. PCALCLs with an expression of cytotoxic phenotype (CD8 ⁺ ) and cytotoxic proteins are uncommon. Cutaneous CD8 ⁺ CD30 ⁺ lymphoproliferative lesions are difficult to classify, diagnose and may be the cause of misdiagnose. CD8 ⁺ PCALCL must be distinguished from CD8 ⁺ mycosis fungoides, lymphomatoid papulosis type D and primary cutaneous aggressive epidermotropic CD8 ⁺ T-cell lymphoma. Usually CD8 ⁺ PCALCL is an indolent disease with a favorable prognosis, except few cases can show poor outcomes. The high Ki-67 index points toward advanced PCALCL. Treatment modalities include surgical excision, radiotherapy and clinical monitoring. Chemotherapy is reserved for disseminated disease. We report a 59-year-old male presented with rapid development of multiple painful reddish-brown plaques and nodular ulcerative skin lesions over the left thigh region since 2 months. A diagnosis of CD8 ⁺ PCALCL with a high Ki-67 index was made on the basis of histology and immunohistochemistry, in co-relation with clinical presentation.

Eyelash trichomegaly is increased length, curling, pigmentation or thickness of eyelashes. Various causes include congenital syndromes, acquired conditions and drugs. It can manifest at birth or present later in life. It can form a part of spectrum of manifestations of some congenital syndromes. Although it tends to have a benign course, it can lead to psychological disturbances and can result in corneal abrasions and visual disturbances, if trichiasis occurs. This article focuses on its etiology, pathogenesis and brief management.

Omalizumab is a recombinant, humanized, monoclonal antibody against human immunoglobulin E (IgE). The US FDA has approved this molecule for chronic urticaria.

We are reporting a rare case of amyloidosis cutis dyschromica in a 41-year-old man. This is a rare form of primary cutaneous amyloidosis characterized by reticulate pigmentation with hypopigmented and hyperpigmented macules, onset in childhood, familial tendency in some, occasional mild itching and deposition of amyloid in the papillary dermis. Our case also had multiple bilaterally symmetrical hyperpigmented keratotic papules abutting the axillary vault resembling those seen in Dowling-Deogs disease. The other unusual feature in this patient was the strong family history of vitiligo, which we are unable to explain. We have also tried to explain the mechanism leading to the hyperpigmentation and hypopigmentation in amyloidosis cutis dyschromica.

Progressive nodular histiocytosis (PNH) is a rare normolipemic macrophage disorder and belongs to a subgroup of non-Langerhans cell histiocytosis (LCHs) which is characterized by a progressive course with no sign of spontaneous resolution but without systemic involvement. We report a 30-year-old gentleman who presented with skin lesions all over the body associated with gradual bilateral painless loss of vision. On examination, approximately 30 to 40, skin-colored, firm, non-tender papules and nodules were noted over the body especially on the face and trunk. A skin biopsy revealed a cellular tumor in the dermis composed of oval to spindle-shaped cells, positive for CD68 but negative for S-100, CD34, CD21, CD35 and HMB45, supporting a diagnosis of spindle cell histiocytic tumor. Ophthalmic examination revealed a generalized arteriolar attenuation in both eyes. He received Tab Imatinib 400 mg OD for 5 months followed by Tab Pazopanib 800 mg OD for 4 months and both the drugs were stopped due to lack of any response in the skin lesions. We report this case due to its rarity, characteristic clinical presentation, and its association with Eale's disease. Primary treatment remains surgical excision of bothersome lesions and optimal systemic treatment is still unknown.

O'Brien first described the actinic granuloma in 1975, as an infrequent granulomatous disorder occurring in sun-exposed skin, with a slow but often self-limited course. Ever since its initial description, the actinic physiopathogenic hypothesis has been debated by many authors. We report a 60-year-old female rural worker that presented with a 14 × 7 cm annular lesion with erythematous elevated borders and an atrophic center on the right calf. The lesion was evolving for 2 years, and histopathology confirmed actinic granuloma. She started acitretin with halting of disease progression after 6 months of therapy. Our case can also be associated to actinic damage, despite its unusual location, therefore highlighting the role of solar elastosis in the development of O'Brien actinic granuloma.

Trichoepitheliomas (TEs) are benign cutaneous tumors that occur either as solitary non-familial or multiple familial. We report a case of multiple familial trichoepithelioma (MFT) in a 55-year-old female patient and her son who came with complaints of single ulcerated mass involving the left nasolabial fold and cheek. She had multiple papules and nodules all over the face and neck since 25 years. Histopathological examination of an ulcerated lesion revealed features of basal cell carcinoma (BCC) with squamous differentiation, which was confirmed by immunohistochemistry. A skin biopsy obtained from the papule on neck showed features of TE. However, whether BCC developed independently or by transformation from TE was uncertain. Her 36-year-old son presented with similar lesions on the face and a skin biopsy showed features of TE. Though malignant transformation of TE is quite rare, awareness of the potential for evolution of carcinoma in patients with MFT is important for management of these patients.

Verrucous epidermal nevi are congenital, noninflammatory cutaneous hamartomas composed of keratinocytes. They follow the lines of Blaschko and show hyperkeratosis without cellular atypia. The routine histology shows variable amount of hyperkeratosis, acanthosis and papillomatosis and rarely epidermolytic hyperkeratosis. We saw a 3-year-old boy with bilaterally symmetrical, systematized verrucous plaques along the lines of Blaschko extensively involving the trunk and extremities but sparing the face and palmoplantar skin. Histopathology showed features of epidermal nevi with prominent epidermolytic hyperkeratosis. We report here the case for the rarity of this entity.

The dorsal cutaneous appendage or the so called human tail is a rare congenital anomaly protruding from the lumbo-sacro-coccygeal area. These caudal appendages are divided into true-tails and pseudo-tails. We report here two cases of congenital pseudo-tail with underlying spina bifida and lipo-meningocele. In this article we seek to emphasize that, as the skin and nervous systems are intimately linked by their similar ectodermal origin, a dorsal appendage may be regarded as a cutaneous marker of the underlying spinal dysraphism.

Eccrine syringofibroadenoma (ESFA) is a rare cutaneous tumor with eccrine differentiation with varied clinical manifestations. We report a case of reactive eccrine syringofibroadenomatosis associated with chronic bilateral plantar ulcers in a patient with diabetes mellitus presenting as plantar hyperkeratosis and verrucous growth at margins.

D-penicillamine interferes with elastin and collagen metabolism and produces several cutaneous and multi-systemic side-effects. We present two cases of Wilson's disease who on long-term penicillamine therapy developed drug-induced degenerative dermopathy manifesting as skin fragility over pressure sites and cutis laxa-like changes.

Background: Acne vulgaris is known to impair many aspects of the quality of life (QoL) of its patients. Aim: To translate the Cardiff Acne Disability Index (CADI) from English into Hindi and to assess its validity and reliability in Hindi speaking patients with acne from India. Methods: Hindi version of CADI, translated and linguistically validated as per published international guidelines, along with a previously translated Hindi version of dermatology life quality index (DLQI) and a demographic questionnaire were administered to acne patients. The internal consistency reliability of the Hindi version of CADI and its concurrent validity were assessed by Cronbach's alpha co-efficient and Spearman's correlation co-efficient respectively. Construct validity was examined by factor analysis. Statistical analysis was carried out using the Statistical Package for the Social Sciences (SPSS) version 20 (SPSS Inc., Chicago, IL, USA) for Windows. Results: One hundred Hindi speaking patients with various grades of acne participated in the study. Hindi version of CADI showed high internal consistency reliability (Cronbach's alpha co-efficient = 0.722). Mean item-to-total correlation co-efficient ranged from 0.502 to 0.760. Concurrent validity of the scale was supported by a significant correlation with the Hindi DLQI. Factor analysis revealed the presence of two dimensions underlying the factor structure of the scale. Conclusion: Hindi CADI is equivalent to the original English version and constitutes a reliable and valid tool for clinical assessment of the impact of acne on QoL.

Background: Polycystic ovarian syndrome (PCOS) is a heterogenous collection of signs and symptoms that when gathered, form a spectrum of disorder with disturbance of reproductive, endocrine and metabolic functions. Aim: The aim of this study is to correlate the skin manifestations with hormonal changes and to know the incidence and prevalence of skin manifestations in patients with PCOS. Materials and Methods: A total of 40 patients with PCOS were examined during 1 year time period from May 2008 P to May 2009. Detailed clinical history was taken from each patient. PCOS was diagnosed on the basis of ultrasonography. Hormonal assays included fasting blood sugar, postprandial blood sugar, follicle-stimulating hormone, luteinizing hormone, thyroid stimulating hormone, dehydroepiandrostenedione, prolactin, free testosterone, fasting lipid profile and sex hormone binding globulin. The results obtained were statistically correlated. Results: In our study, the prevalence of cutaneous manifestations was 90%. Of all the cutaneous manifestations acne was seen in highest percentage (67.5%), followed by hirsutism (62.5%), seborrhea (52.5%), androgenetic alopecia (AGA) (30%), acanthosis nigricans (22.5%) and acrochordons (10%). Fasting insulin levels was the most common hormonal abnormality seen in both acne and hirsutism, whereas AGA was associated with high testosterone levels. Conclusion: The prevalence of cutaneous manifestations in PCOS was 90%. Hirsutism, acne, seborrhea, acanthosis nigricans and acrochordons were associated with increased levels of fasting insulin, whereas AGA showed higher levels of serum testosterone.

Background: Vitamin B ₁₂ deficiency is thought to be uncommon in the eastern parts of India including Bengal and the eastern states as compared to the northern and southern parts of India. The importance of cutaneous features in relation to vitamin B ₁₂ deficiency is not well described in literature. Aim: To know the clinical profile of vitamin B ₁₂ deficiency in this region and to find out if there is any relationship between dermatologic manifestations with vitamin B ₁₂ deficiency. Materials and Methods: All symptomatic patients of anemia requiring blood transfusions who had either raised mean corpuscular volume (MCV) or bicytopenia/pancytopenia on complete blood count or were symptomatic in the form of skin hyperpigmentation were screened for vitamin B ₁₂ deficiency. Results: Twenty-five patients were tested for vitamin B ₁₂ deficiency. Of them 19 patients were found to be having vitamin B ₁₂ deficiency. Conclusions: Vitamin B ₁₂ deficiency is not uncommon in the eastern parts of India, contrasting the previous thoughts that it was uncommon in this area, though larger studies are required to know it better. This study included only those requiring blood transfusions, thus a much higher prevalence is expected in this area. Patients with vitamin B ₁₂ deficiency do present with severe anemia requiring blood transfusions and often have skin hyperpigmentation.

Background: The epidemiological evaluation of androgenetic alopecia (AGA) is based mainly on direct observation and questionnaires. The international epidemiology and environmental risk factors of AGA in young Caucasian men remain unknown. Aim: To use photographs and data from the Internet to evaluate severe AGA and generate greater understanding of the international epidemiology of the disorder in young Caucasian men. Materials and Methods: A population-based cross-sectional study design was used. The sample included 26,340 Caucasian men aged 30 to 40 years who had uploaded profiles to two dating websites. Their photographs were evaluated for AGA and graded as follows: severe AGA (Norwood type VI-VII), non-severe AGA, and unknown. Epidemiological data were collected from the sites. Logistic regression was used to analyze the effect of risk factors on the prevalence of severe AGA. Results : The overall success rate for identifying severe AGA by indirect evaluation of Internet photographs was 94%. The prevalence of severe AGA was 15.33% overall and varied significantly by geographical region. The risk of having severe AGA was increased by 1.092 for every year of age between 30 and 40 years. Severe AGA was more prevalent in subjects with higher body mass index. Conclusions: Photographs from the Internet can be used to evaluate severe AGA in epidemiological studies. The prevalence of severe AGA in young Caucasian men increases with age and varies by geographical region. Body mass index is an environmental risk factor for severe AGA.

Pemphigus vulgaris is an autoimmune disorder that involves intraepithelial blistering and sores of skin and mucous membrane. It generally correlates with the levels of circulating autoantibodies; their removal seems a reasonable therapeutic approach. Therapeutic plasma exchange is hypothesized to remove pathogenic autoantibodies and has been used in refractory or severe cases. It may be considered for rapid control of severe or recalcitrant pemphigus vulgaris and should be combined with use of concomitant immuno-suppressive.

Vorinostat is a new drug used in the management of cutaneous T cell lymphoma when the disease persists, gets worse or comes back during or after treatment with other medicines. It is an efficacious and well tolerated drug and has been considered a novel drug in the treatment of this condition. Currently apart from cutaneous T cell lymphoma the role of Vorinostat for other types of cancers is being investigated both as mono-therapy and combination therapy.

Onychomadesis or proximal separation of the nail pate usually results from severe, generalized dermatoses like bullous diseases, hand foot and mouth disease, varicella or severe cutaneous drug reactions. Although lichen planus (LP) produces varied nail manifestations (longitudinal onychorrhexis, onychoschizia, nail pigmentation, pterygium etc.), to the best of our knowledge, onychomadesis as a manifestation of nail LP is not recorded. This report presents two children with onychomadesis arising with generalized eruptive LP.

Dirofilariasis is a parasitic infection transmitted by mosquitoes from domestic and wild animals. Humans accidentally get infected and can present with a lung nodule, subcutaneous mass or an ocular lesion which will be either subconjunctival or periorbital. Proper microbiological and histopathological examinations aid in proper diagnosis. Herein, we present a case report of a 64-year-old female patient from Kerala with an infraorbital mass diagnosed as dirofilariasis.

Here we report a case of a young male who developed full blown iatrogenic Cushing's syndrome after use of superpotent clobetasol propionate cream 0.05% for long duration to suppress psoriatic skin lesions. He also developed osteoporosis and hypogonadism. This case demonstrates that injudicious use of topical steroids can have disastrous consequences.

A 3-month-old female patient with a giant ulcerated nodule over the back since birth was diagnosed as congenital giant juvenile xanthogranuloma (JXG) based on clinical and histopathological examination. Congenital giant JXG with ulceration at birth is a rare presentation of JXG and commonly misdiagnosed. This case emphasizes the importance of being aware of the myriad presentations of JXG in order to make a correct diagnosis and avoid unnecessary investigations or treatment.

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis with anogenital and extragenital presentations. Extragenital lichen sclerosus is most common on the neck, shoulders and upper trunk. Linear lesions are uncommon in LSA. We report a case of linear extragenital LSA involving forehead and scalp, along with grouped white papules of LSA in the right side of the back in a postmenopausal woman. The patient showed atypical clinical presentation of LSA in face which clinically mimicked 'en coup de sabre' as seen in morphea, but other clinical features suggested the diagnosis of LSA and the histopathological findings confirmed it.

Clear-cell syringomas, a rare histologic variant of syringomas, frequently coexist with diabetes mellitus. Clinically, it presents as skin colored papules distributed symmetrically around periorbital region. However, asymmetrical distribution of syringomas is rare and much rarer is the ulceration in such syringomas. Ulceration in such asymmetrical syringomas has not been reported so far. Herein, we report ulceration of clear-cell syringomas involving left groin in a middle aged non-diabetic woman.

Homozygous familial hypercholesterolemia is an autosomal dominant disorder of lipid metabolism, characterized by reduced clearance of low-density lipoprotein-cholesterol and a high risk of rapid development of cardiovascular diseases. Its incidence is relatively rare and estimated to be one in one million in general populations. Here, we report homozygous familial hypercholesterolemia in two Egyptian young siblings, presented with cutaneous, tendinous xanthomas, and corneal arcus. One of them has symmetric subcutaneous lipomatosis, which has not been reported before in association with familial hypercholesterolemia.

Tuberous sclerosis complex (TSC) is a well-known clinical entity, characterized by facial angio-fibroma, shagreen patch, and hypo-melanotic, and confetti-like skin lesions. An exquisite fresh case is being narrated, emphasizing its microscopic pathology. The role of magnetic resonance imaging of the brain, in particular, is highlighted to define the large variety of neurological abrasions for determining its future progression.

Active bleeding in plexiform neurofibromatosis can be a life-threatening complication in neurofibromatosis type 1 (NF1). The prompt imaging support of 2D-3D ultrasound (US) and computed tomography (CT) during the active hemorrhage phase of cutaneous neurofibromas has not been previously reported. We report a case with NF1 who experienced a sudden swelling in the parieto-temporal region that corresponded to a massive and active hemorrhage within a plexiform neurofibroma. The US and CT imaging characteristics of this bleeding tumor are shown. Active hemorrhage in a plexiform neurofibroma of the scalp appeared in US as a heterogeneous hypodermal mass. CT demonstrated a fully hyperdense soft tissue mass. These characteristics differ from the non-complicated or old hemorrhagic imaging appearances of scalp plexiform neurofibromas and encourage prompt surgical treatment. This case report demonstrates the usefulness of imaging support in the early diagnosis of this hemorrhagic complication of NF1 in the scalp and also stimulates multispecialty management.

A case of nevus comedonicus syndrome with atypical cutaneous presentation (widespread involvement without any particular pattern, midline lesions involving lower abdomen and involvement of bilateral pinna), and some unusual skeletal (adduction deformity involving bilateral metatarsal along with medial deviation at the level of tarsometatarsal joint), central nervous system (agenesis of corpus callosum with a interhemispheric cyst), visceral (pancreatic cyst) and neurological manifestations have been illustrated.

A 33-year-old male showed multiple pigmented patches on his trunk and extremities after he took tranexamic acid for common cold. He stated that similar eruptions appeared when he was treated with tranexamic acid for influenza 10 months before. Patch test showed positive results at 48 h and 72 h by 1% and 10% tranexamic acid at the lesional skin only. To our knowledge, nine cases of fixed drug eruption induced by tranexamic acid have been reported in Japan. Tranexamic acid is a safe drug and frequently used because of its anti-fibrinolytic and anti-inflammatory effects, but caution of inducing fixed drug eruption should be necessary.

Ectodermal dysplasia/skin fragility syndrome (ED-SFS) is a newly described autosomal recessive disorder characterized by skin fragility and blistering, palmoplantar keratoderma, abnormal hair growth, nail dystrophy, and occasionally defective sweating. It results from mutations in the PKP1 gene encoding plakophilin 1 (PKP1), which is an important component of stratifying epithelial desmosomes and a nuclear component of many cell types. Only 12 cases of this rare genodermatosis have been reported so far. We present an unusual case of ED-SFS in a 12-year boy who was normal at birth but subsequently developed skin fragility, hair and nail deformities, abnormal dentition, palmoplantar keratoderma, and abnormal sweating but no systemic abnormality.

Hidradenocarcinomas are very rare malignant sweat gland tumors that possess an infiltrative and/or low metastatic potential. Here we describe an interesting case of hidradenoma on the fronto-parietal region of the scalp of an elderly female, part of which had developed carcinomatous changes, infiltrating up to the pericranium. She developed intense itching, pain, spontaneous ulceration and rapid increase in the size of the tumor correlating with the expression of malignant behavior of the neoplasm. An initial incision biopsy suggested features of benign poroid hidradenoma, while the histology from the excised tumor exhibited a fairly well circumscribed epithelial neoplasm in dermis consisting of interconnected nodules as well as differentiated ducts, the neoplastic cells showing mild pleomorphism of nuclei, mitotic figures and abundant pale cytoplasm. Clefts, sclerotic stroma and foci of necrosis en mass were also seen. The final diagnosis was a well differentiated and slow growing hidradenocarcinoma. The tumor recurred locally despite total excision.

Tufted angioma (TA) is a localized benign hamartomatous vascular proliferation usually presenting in the childhood as an erythematous plaque. We report here a rare case of multifocal TA in an 8-year-old boy who presented which two large annular lesions as well as multiple papules and nodules on the back for the duration of 4 years. Histology showed typical well circumscribed poorly canalized vascular lobules with 'cannon ball' configuration.

Acute generalized exanthematous pustulosis is a rare severe cutaneous adverse reaction pattern that is mostly caused by the intake of drugs and rarely associated with viral infections, food allergens or toxins. Here we present the report of three patients who got admitted in our hospital for generalized pustulosis and fever after insect bites. The diagnosis of acute generalized exanthematous pustulosis was made by EuroSCAR scoring. The drug etiology was excluded and spider bite was implicated as the etiological agent in these cases of AGEP which are the first such reported cases in India.

Cutaneous larva migrans (CLM) is the most common tropically acquired dermatosis caused by infection with hookworm larvae. It is commonly seen in tropical areas, and in people who have a history of foreign travel and of walking barefoot on sandy soil or beaches. An increased incidence has been seen in non-endemic areas due to change in environmental and behavioral factors. The presence of this entity is questionable in Northern region as the environmental conditions and the type of soil is not favorable, both of which are required for the survival of nematode. We describe a case series of six patients presented during winter season in the outpatient department of Dermatology within a short period of 2 months. We also review the clinical features of various other creeping eruptions and factors that are responsible for boosting infection in North India.

Microsporum gypseum is a geographically widespread geophilic fungus that infects animals and humans. M. gypseum infection on the scrotum is very rare and can be easily misdiagnosed because of a lack of inflammatory reaction. Here we describe a patient with pseudomembranous-like tinea of the scrotum resulting from M. gypseum.

We report an unusual and dramatic presentation of a rare form of cutaneous lymphoma, known as subcutaneous panniculitis-like T-cell lymphoma (SPTCL). This patient presented with a pruritic, florid and purpuric rash that was diagnosed as lobular panniculitis and treated with oral steroids for 1 year with no success. His skin lesions would return each time oral corticosteroids were being weaned off. Upon presentation to our clinic, repeated deep skin biopsies with immunohistochemical analysis coupled with the clinical history of persistent B symptoms and the presence of pancytopenia helped clinched the rare diagnosis of SPTCL with hemophagocytosis. The patient was then started on cyclosporine and dexamethasone before definitive chemotherapy. This rare and diagnostically challenging condition is commonly misdiagnosed as benign panniculitis or eczema, and highlights the importance of repeated skin biopsies.

Acquired idiopathic generalized anhidrosis is a rare condition, where the exact pathomechanism is unknown. We report a case of acquired idiopathic generalized anhidrosis in a patient who later developed lichen planus. Here an autoimmune-mediated destruction of sweat glands may be the probable pathomechanism.

Pemphigoid vegetans is a very rare type of bullous pemphigoid which usually affects the elderly and has not been reported in children. It shows a clinical resemblance to pemphigus vegetans but has distinct histological and immunopathological features of bullous pemphigoid. A 9-year-old girl presented with recurrent purulent and verrucous vegetating lesions on her forehead, groin and vulva along with scaling, crusted, bullous and purulent lesions on the eyelids, periorbital, periauricular, perioral region and lips. She had oral lesions and a cerebriform tongue. Though she showed clinical features of pemphigus vegetans, histology revealed a subepidermal blister with the absence of acantholysis. Direct immunofluorescence studies were suggestive of bullous pemphigoid. On clinicopathological correlation, a diagnosis of pemphigoid vegetans was made. She responded well to oral corticosteroids and dapsone therapy with complete resolution of the lesions.

Darier's disease is an autosomal dominant genodermatosis resulting from ATP2A2 gene mutation. A 62-year-old male presented at our outpatient (OPD) with sudden-onset numerous dirty, warty papules over the head, neck, and back since 2 months. Histopathology of the skin lesions revealed acantholytic dyskeratosis suggestive of Darier's disease. He was referred to the gastroenterology department for some gastrointestinal (GI) symptoms where he was diagnosed with adenocarcinoma of the stomach and was subsequently operated. On his next visit to our department for follow-up, we found a marked diminution of the skin lesions in the absence of any specific treatment. In view of the above finding, we concluded that paraneoplastic dermatosis in the form of Darier's disease occurred in this patient. Paraneoplastic Darier's disease with gastric adenocarcinoma is not yet described in dermatology literature and is hence reported here.

Cirsoid aneurysm is a small vascular proliferation characterized by small to medium-sized channels with features of arteries and veins, that present as small, blue or red asymptomatic papule. We report a case of a crisoid aneurysm on the forhead of an HIV patient that suggested a Kaposi sarcoma as a differential diagnosis.

We report a case of recurrent localized pyogenic granulomas with underlying arteriovenous malformation in an 11-year-old male child on upper back with onset in early infancy.

Acanthosis nigricans (AN) is a dermatosis characterized by velvety, papillomatous, brownish-black, hyperkeratotic plaques, typically on the intertriginous surfaces and neck. The majority (80%) of AN occurs idiopathically or in benign conditions such as endocrinopathies like diabetes mellitus, polycystic ovary syndrome; metabolic syndrome and/or heritable disease. Malignancy-associated AN is rare. AN may rarely be associated with autoimmune diseases including systemic lupus erythematosus, due to antibodies to the insulin receptor, so-called type B insulin resistance. Here we report a case of AN in a case of diffuse progressive systemic sclerosis without evidence of insulin resistance.

Extramammary Paget's disease (EMPD), which is considered to be an adenocarcinoma of the apocrine glands, is a rare, slow-growing neoplasm. The standard treatment for local EMPD is surgical resection, however, so far, no effective treatment is found for advanced EMPD. Trastuzumab, which is a recombinant monoclonal antibody against the human epidermal growth factor receptor 2 HER2 has been proved to be effective in the treatment of advanced EMPD in some cases where HER2 protein is overexpressed. Herein, we report two cases of EMPD in two brothers. The younger brother who presented as local EMPD on the scrotum received surgical resection and had no recurrence in 15 months following. The older brother suffered from invasive EMPD also on the scrotum with inguinal region multiple metastatic lymph nodes and was treated with combination chemotherapy and Trastuzumab to target HER2 consecutively after a wide surgical excision.

A 24-year-old lady presented with the complaints of asymptomatic dark, raised lesions over her body since past 4 years. Dermatological examination revealed hyperkeratotic, hyperpigmented papules in a Blaschkoid distribution over trunk and extremities. The hyperkeratotic lesions could be removed leaving behind a small crater. Skin biopsy revealed hyperkeratosis, parakeratosis with epidermal invagination, focal cornified plug with basophilic degenerated material and dyskeratotic keratinocytes. Dermis showed dilated capillaries with moderate amount of perivascular lymphocytic infiltrate and granulomatous lymphocytic infiltrate in the deep dermis. A diagnosis of Kyrle's disease was made and patient was started on oral acitretin 25 mg daily with satisfactory response to therapy. This is the first reported instance of Kyrle's disease occurring in an extensive Blaschkoid distribution.