Ectrodactyly, ectodermal dysplasia, and cleft lip-palate (EEC) syndrome without clefting: a rare case report.

Ectrodactyly, Ectodermal dysplasia, and Cleft lip-cleft palate (EEC) syndrome is a rare ectodermal dysplasia presenting with various combinations of its three components. It is an autosomal dominant disorder with variable expression and penetrance. Patients have features of ectodermal abnormalities and a split hand/foot deformity of the limbs. We report a case of this rare disorder in a 40-year-old male who had ectrodactyly, ectodermal dysplasia, but no clefting of lips or palate.

A clinico-etiological study of urethritis in men attending sexually transmitted disease clinic at a tertiary hospital.

INTRODUCTION: Urethritis is an important sexually transmitted infection in the present day. Causative agents are many for urethritis, ranging from bacteria, virus, to protozoa. AIM: The aim was to study the various etiological agents and clinical presentation of men with urethritis. MATERIALS AND METHODS: This was a cross-sectional study conducted at a tertiary care center in Bengaluru. After taking written informed consent, 100 men with symptoms of urethritis were enrolled in the study. Their demographic details, presenting symptoms, and examination findings were documented using a questionnaire and a pro forma. Results were tabulated and analyzed using mean. RESULTS: Nearly 68% had urethral discharge with dysuria as the presenting symptom. Nearly 27% had only dysuria without discharge as the main complaint. Almost 15% had coexistent genital ulcer disease. Human immunodeficiency virus infection was present in 10% in the population studied. The most prevalent organism isolated was Neisseria gonorrhoeae (45%). Chlamydia trachomatis was isolated in 13%. Trichomonas vaginalis was isolated in two patients. CONCLUSION: Our study highlights the high prevalence of gonorrhea in India when nongonococcal urethritis is the forerunner in the Western world. In addition, T. vaginalis as a cause of urethritis has to be considered in our setup.

Secukinumab efficacy and safety in indian patients with moderate-to-severe plaque psoriasis: Sub-analysis from FIXTURE, a randomized, placebo-controlled, phase 3 study.

TITLE: Secukinumab efficacy and safety in Indian patients with moderate-to-severe plaque psoriasis: sub-analysis from FIXTURE (Full Year Investigative Examination of Secukinumab vs. Etanercept Using Two Dosing Regimens to Determine Efficacy in Psoriasis), a randomized, placebo-controlled, phase 3 study. BACKGROUND: Evidence has suggested Interleukin (IL)-17A to be an important effector cytokine in the pathogenesis of psoriasis. Here, we report results for an Indian sub-population from a multinational study FIXTURE, designed to assess the safety, tolerability, and long-term efficacy of fully human anti-IL-17A monoclonal antibody secukinumab in patients with moderate-to-severe plaque psoriasis. MATERIALS AND METHODS: In this double-dummy, placebo controlled, 52-weeks phase 3 study FIXTURE, 149 Indian patients were randomized 1:1:1:1 to receive secukinumab at a dose of 300 mg or 150 mg, etanercept, or placebo. The study objective was to show the superiority of secukinumab over placebo at week 12, vis-à-vis proportion of patients achieving a reduction of 75% or more from the baseline in the psoriasis area-and-severity index score (PASI 75) and a score of 0 (clear) or 1 (almost clear) on a 5-point modified investigator's global assessment (IGA mod 2011) (co-primary end points). RESULTS: At week 12, 61.0% and 55.9% patients in secukinumab 300 mg and 150 mg groups, respectively, achieved PASI 75 response compared to 20.0% in the etanercept and 7.1% in the placebo groups. Similarly, IGA mod 2011 0 or 1 response was achieved by 43.9% and 20.6% in patients in the secukinumab 300 mg and 150 mg group, respectively, vs. 13.3% in the etanercept and 2.4% in the placebo groups at week 12. Likewise, higher proportions of patients in secukinumab 300 mg (41.5%) and 150 mg (20.6%) group were PASI 90 responders at week 12 than those in the etanercept (10.0%) or placebo (0.0%) groups. The incidences of adverse events (AEs), during the induction period were similar in all the treatment groups. Overall secukinumab was well-tolerated at both doses in the Indian sub-population. CONCLUSION: The results from the Indian sub-population suggest that secukinumab is an efficacious and safe drug for use in moderate-to-severe chronic plaque psoriasis.

Outcome of Dermal Grafting in the Management of Atrophic Facial Scars.

BACKGROUND: Scars over the face are cosmetically and psychologically disturbing. Various techniques have been described and are being practiced in the management of these scars. AIMS AND OBJECTIVES: This study was undertaken to study the safety, effectiveness of using dermal grafts as fillers in the management of facial scars due to acne, chickenpox, trauma or any others. MATERIALS AND METHODS: Fifteen patients with atrophic facial scars of varied aetiology and willing for surgery were considered for dermal graft technique. After pre-operative workup, subcision was done 2 weeks before planned surgery. Depending on the type of scar, grafts were inserted using pocket or road railing techniques. Scar improvement was assessed based on patient satisfaction. RESULTS: Linear scars showed excellent improvement. Acne, varicella and traumatic scars also showed good improvement. However, two patients did not appreciate improvement due to marked surface irregularities as the scars were elevated. They were further subjected to LASER and chemical peel resurfacing. CONCLUSION: Dermal grafting can be used in the management of any round to oval facial scar which is soft, prominent and at least 4-5 mm across; linear scars at least 2-3 mm across and 3-4 cm in length. However, scars with prominent surface irregularities need further resurfacing techniques along with dermal grafting. LIMITATIONS: Limitations of the study include small sample size, and only subjective assessment of the scar has been taken into consideration to assess the outcome.

A Study of the Outcome of Primary Excision and Closure Technique in the Management of Lip Leukoderma in 30 Patients.

CONTEXT: Lips being one of the graceful cosmetic units of the face, any disfigurement, including depigmentation, can be embarrassing, psychologically distressing, and socially stigmatizing. Even with the evolution of various surgical modalities, none of the techniques ensure 100% pigmentation with a good cosmetic appearance. With this background, this study was undertaken to study the efficacy of the technique-primary excision and closure in treatment of lip leukoderma (LL). AIMS: To study the efficacy, cosmetic outcome, and recurrence following primary excision and closure technique in the management of LL. SETTINGS AND DESIGN: A prospective single-center open-label uncontrolled study. MATERIALS AND METHODS: Thirty patients with LL involving inner margin of the lower lip and those who fulfilled inclusion and exclusion criteria were included in the study. After obtaining consent and physician fitness, patients were posted for primary excision and closure of the vitiliginous area of the lip. Patients were followed up at the immediate postoperative period and 6 months later. Patient satisfaction scale was done by asking the patient to grade the response between one and ten. STATISTICAL ANALYSIS USED: Mean and percentiles. RESULTS: Out of 30 patients, 29 patients achieved complete clearance that was cosmetically acceptable. Only one patient showed recurrence at the end of 6 months. Average patient satisfaction scale showed an average of 9.66. CONCLUSIONS: Primary excision and closure technique is one of the safe, inexpensive modality in the management of LL involving the inner margin of the lip with immediate result.

A comparative evaluation of skin and nerve histopathology in single skin lesion leprosy.

BACKGROUND: In spite of leprosy being a disease of nerves, ROM therapy for single skin lesion leprosy was based on clinical trials without much evidence-based studies of nerve pathology. The present study was undertaken to compare the histology of skin and nerve in single skin lesion leprosy, and to assess the scientific rationale and justification of single dose ROM therapy. METHODS: Twenty-seven untreated patients with single skin lesion without significantly thickened peripheral nerves were selected. Skin and nearby pure cutaneous nerve biopsies were studied under both H&E and Fite's stain. RESULTS: All the skin biopsies were negative for AFB and clinico-pathological correlation was positive in 51.85% of skin biopsy specimens. Histopathological diagnosis of leprosy was evident in 55.5% of clinically normal looking nerves, with AFB positivity in 29.6% of nerve biopsy specimens. Correlation between clinical diagnosis and nerve histopathology was poor (26%). CONCLUSIONS: Single skin lesion without thickened peripheral nerves as criteria for single dose ROM therapy is not logical, since the histological diagnosis of leprosy in normal looking nerves with presence of AFB is revealed in this study. Pure cutaneous nerve biopsy is a simple outpatient procedure, without complications. This study emphasizes the need to consider nerve pathology as an important tool for further therapeutic recommendations, than just clinical trials and skin pathology alone. Though single dose ROM therapy has been withdrawn recently, the principle holds good for any future therapeutic recommendations.

Dyschromias: A Series of Five Interesting Cases from India.

Dyschromatosis is a pigmentary genodermatosis which presents with hyper and hypopigmented skin lesions giving a mottled appearance. It is a rare entity in India reported mainly in the East Asian population. Classically, two forms have been described; dyschromatosis universalis hereditaria (DUH) and dyschromatosis symmetrica hereditaria. Here we report four cases of DUH and one case of dyschromatosis symmetrica hereditaria from India.

Keratosis Follicularis Spinulosa Decalvans: A Report of Three Cases.

Keratosis follicularis spinulosa decalvans is a disorder affecting the hair follicles characterized by scarring alopecia of the scalp, eyebrows, and axillae, sometimes associated with photophobia and keratoderma. Being X-linked, it is more commonly seen in males but can be rarely seen in females also. We report three cases of this rare disorder including one in a female.

Efficacy of 15% trichloroacetic Acid and 50% glycolic Acid peel in the treatment of frictional melanosis: a comparative study.

BACKGROUND: Frictional dermal melanosis is aesthetically displeasing. Various modalities ranging from depigmenting agents to lasers have been tried but it continues to be a difficult problem to treat. OBJECTIVE: To study and compare the efficacy of 15% trichloroacetic acid (TCA) and 50% glycolic acid in the treatment of frictional melanosis of the forearm. MATERIALS AND METHODS: 40 patients of frictional melanosis of the forearm were included in the study. Patients were randomly divided into two equal groups A and B. Pre-peel priming was carried out with 12% glycolic acid and sunscreen for 2 weeks. Group A was treated with trichloroacetic acid (TCA-15%) peel and Group B with glycolic acid (GA-50%) peel. Four peels were done one every 15 days. Clinical photographs were taken to assess the response. Response to therapy was evaluated by both objective and subjective methods. The patients were followed up for 3 months after the last peel to note any relapse. RESULTS: Both TCA and glycolic acid peels were effective in frictional melanosis. TCA showed better response compared to glycolic acid at the end of the treatment, both by subjective and objective methods. However, this difference was not statistically significant (P > 0.05). No permanent side effects were seen in any of the treated patients and the improvement was sustained without any relapse at 3 months. CONCLUSION: Chemical peeling with both tricholoroacetic acid (15%) and glycolic acid (50%) is safe and effective for the treatment of frictional dermal melanosis. Tricholoroacetic acid was found to be marginally superior to glycolic acid.

A Study of Hair Follicular Transplantation as a Treatment Option for Vitiligo.

BACKGROUND: Repigmentation of vitiligo is closely related to hair follicles. Hence, replenishing melanocytes in vitiliginous patches utilizing undifferentiated stem cells of the hair follicles using follicular unit transplantation (FUT) is a possible treatment option. OBJECTIVES OF THE STUDY: To study the efficacy of FUT in cases of segmental/stabilized vitiligo as a treatment option for leukotrichia. MATERIALS AND METHODS: Fifty patients with 63 lesions of stable vitiligo over nonglabrous areas were treated with follicular unit grafts. Reduction in the size of vitiligo patches as well as improvement in the associated leukotrichia were evaluated using subjective and objective assessments. RESULTS: Of the 63 patches, good to excellent response was seen in 39 (61.9%), fair in 16 (25.4%), and poor in eight (12.7%) lesions. No repigmentation was seen in two (4.8%) lesions. The mean improvement seen was 61.17%. Excellent color match was observed in 44 lesions (69.8%). Repigmentation of the depigmented hairs occurred in 11 out of 46 patients with associated leukotrichia. CONCLUSION: FUT is a safe and effective method for treating localized and segmental vitiligo, especially on hairy parts of the skin. Though labor intensive, it was found to be associated with a quick patient recovery time, very low morbidity, and good color match.

Dexamethasone-cyclophosphamide pulse therapy for autoimmune-vesiculobullous disorders at Victoria hospital, Bangalore.

Pemphigus is the commonest autoimmune vesiculobullous disorder on the Indian subcontinent. The mainstay treatment of the disease is systemic steroids and other immunosuppressive therapies. We evaluated the pattern of treatment as originally presented by Pasricha et al. in 1988 and by Surindar Kanwar in 1990. Starting in April 2001, we enrolled 50 patients with autoimmune vesiculobullous disorders for dexamethasone-cyclophosphamide pulse therapy [DCP] in our hospital. Of these, six (12 %) patients are currently in the fourth phase of treatment, ten (20 %) are in the third phase, fifteen (30 %) in the second phase, and twelve (24 %) patients are in the first phase. Treatment was discontinued by six patients, and one fatality secondary to hyperglycemia and mucormycosis. Our findings are consistent with previous reports that DCP therapy is very effective in the treatment of vesiculobullous disorders.

Therapeutic wounding - 88% phenol in idiopathic guttate hypomelanosis.

CONTEXT: Therapeutic wounding includes wounding the skin to induce pigmentation of the depigmented skin patches that was earlier used for repigmenting small patches of stable vitiligo. In this study, we have used the same principle to induce pigmentation in idiopathic guttate hypomelanosis (IGH) by spot peel with 88% phenol. AIMS: To study the efficacy of phenol in causing repigmentation in IGH and its adverse effect profile. SETTINGS AND DESIGN: Open prospective study. MATERIALS AND METHODS: Twenty patients with 139 IGH macules were subjected to spot peel. Eighty-eight percent phenol was applied with an ear bud once a month for two sittings. Patients were assessed both subjectively and objectively after every session and at the end of 3 months of initiation of therapy. RESULTS: Repigmentation was noted in 64% of IGH macules. More than 75% improvement was seen in 45% of the total IGH macules, while 41.5% showed 50-75% improvement at the end of three months. Persistent scabbing was the common adverse effect noted in 17.26% of lesions. CONCLUSION: Spot peel with 88% phenol is a safe, simple, cost-effective, outpatient procedure for IGH, which can be combined with other medical therapies.

Pattern of pediatric dermatoses at a referral centre.

OBJECTIVE: To determine the pattern of skin disorders seen among children attending a Medical College Hospital, Bangalore METHODS: All children 18 y and below attending the Pediatric Dermatology OPD with skin diseases between the period of January 2011 and June 2011 were included in the study. RESULTS: A total of 1,090 new cases (boys 589; girls 501) with 1,118 dermatoses were recorded during this period. Twenty eight children had more than one dermatoses. Most of the disorders were seen between 5 and 11 y age group. The most common dermatoses was infections and infestations (32.47 %) followed by eczematous eruptions and dermatitis (20.66 %), papulosquamous and related disorders (6.08 %) and pigmentary disorders (5.81 %). Insect bite reactions and papular urticaria formed 5.1 %. Acne was seen in 5.28 % of children followed by urticaria (3.67 %), photodermatoses 2.5 % and genetic disorders (3.04 %). CONCLUSIONS: In the present setting bacterial infections and scabies still remained the most common pediatric dermatoses followed by molluscum contagiosum and atopic dermatitis. Childhood psoriasis contributed to major part of the study among papulosquamous disorders.

Follicular unit extraction as a therapeutic option for vitiligo.

Follicular unit extraction (FUE) is a surgical procedure, which can be used to transplant follicular units into vitiliginous areas. Such follicular unit transplant has been recently used to repigment stable vitiligo patches. FUE was done for a 12-year-old female with a stable vitiligo patch with leukotrichia on the eyebrow. Repigmentation was noted in 6 weeks and complete pigmentation seen at 12 weeks. Leukotrichia resolved over a period of 6 months. No recurrence was noted at the end of 6 months follow-up with excellent colour match. This case is presented to highlight the simplicity, safety and effectiveness of FUE in stable vitiligo patches with leukotrichia.

Epithelioid hemangioendothelioma in a child.

Epithelioid hemangioendothelioma (EHE) is a rare tumour arising from the vascular endothelium of soft-tissue, bone and viscera. Skin involvement is rare. The disease has an indolent course, with the potential for recurrence and often associated with multi-systemic localisations. We present a rare case of cutaneous EHE without systemic involvement in 9-year-old boy. The tumour was completely excised and histopathologic diagnosis was consistent with EHE. Complete systemic assessment showed no internal localisation. Wide excision is the mainstay of therapy and regular follow-up is suggested due to the potential for recurrence. The majority of documented cases of EHE is in adults and has developed in association with an underlying systemic involvement. To the best of our knowledge, this is the second reported case of isolated cutaneous EHE in paediatric age group.

Progressive symmetric erythrokeratoderma with nephrotic syndrome: Coincidence or new association?

Progressive symmetric erythrokeratoderma (PSEK) is a rare genodermatosis with variable inheritance. It is characterized by symmetrical, erythematous, and hyperkeratotic plaques on the extremities. We report a case of a 15-year-old boy with PSEK of autosomal recessive inheritance associated with nephrotic syndrome.

Sclerotherapy in the management of varicose veins and its dermatological complications.

BACKGROUND: Varicose veins and its dermatological complications like stasis dermatitis, ulcers, spontaneous bleeding are commonly seen in the dermatology clinics. Surgery has been the most often used treatment for varicose veins. Sclerotherapy refers to introduction of sclerosing solution into the varicose veins, which causes endothelial damage and subsequent fibrosis. Sclerotherapy is being practised extensively by dermatosurgeons in the west. However, there are no Indian studies which specifically evaluate the role of sclerotherapy in the management of varicose veins and its skin complications. Hence, this study aims to evaluate the efficacy of sclerotherapy in managing varicose veins and its complications. AIMS: To study the safety and efficacy of sclerotherapy in the treatment of varicose veins and its dermatological complications. METHODS: This is a prospective study involving 50 patients with varicose veins and its dermatological complications attending the dermatology out-patient department. The study was conducted over a period of 18 months. After thorough clinical, laboratory, and radiological evaluation, the patients were treated with sclerotherapy using Sodium Tetradecyl Sulphate of various concentrations depending on the vessel size. The patients were then followed up to look for disappearance of veins, healing of ulcers and eczema, and any complications. RESULTS: Patients showed a good response to treatment with sclerotherapy. 70-80% of patients showed symptomatic improvement along with disappearance of veins and healing of eczema and ulcers. Most of the complications were minor, which resolved over a period of few weeks. CONCLUSION: Sclerotherapy is a simple, safe and effective procedure for the treatment of varicose veins and its dermatological complications. The procedure is particularly effective for smaller, early varicosities and also for residual veins after surgery. Hence we recommend more and more of our fellow dermatologists to take up this procedure, which can be an efficient tool to manage patients with varicose veins and its related complications.

A Split-face Comparative Study of 70% Trichloroacetic Acid and 80% Phenol Spot Peel in the Treatment of Freckles.

AIM: To study and compare the efficacy of TCA (70%) and phenol (80%) in the treatment of freckles. SETTINGS AND DESIGN: A prospective study was done, over one year, on patients having freckles on the face. The freckles on the right side of the face were treated with TCA (70%) and those on the left side of the face were treated with phenol (80%). MATERIALS AND METHODS: Twenty patients of Fitzpatrick type II-IV were treated. The freckles on the left side of the face were treated with phenol, while those on the right were treated with TCA in all patients. The patients were reviewed at weekly intervals for three weeks. Evaluation of the results was done photographically, subjectively, and objectively. RESULTS: Fifteen out of 20 patients (75%) noted a complete clearance (90 - 100% lightening) of the lesions treated. Four patients (darker skin types) noted hyperpigmentation. One noticed recurrence where TCA was applied. CONCLUSIONS: Both TCA and phenol are found to be equally effective in the treatment of freckles in fair skin. Both are cheap and effective alternatives for the treatment of freckles.

Phenolisation as a mode of treatment of mucosal cysts.

Mucous cysts are one of the common cystic lesions of minor salivary glands seen mostly on mucous surface of lower lip and buccal mucosa. Many modalities of treatment for mucosal cysts exist including excision and suturing, deroofing, marsupialization, radiosurgery and cryotherapy. We have used 88% phenol as a mode of treatment of mucosal cysts.