Fibrotic Cytokine Interplay in Evaluation of Disease Activity in Treatment Naïve Systemic Sclerosis Patients from Western India.

BACKGROUND: : Systemic sclerosis (SSc) is a demyelinating disease of skin, subcutaneous tissue, muscles and internal organs, with fibrosis as an important pathological event. AIM: : To understand cytokine interplay of IL-1ß, IL-4 and IL-6 and their association with disease activity in treatment naïve active cases of systemic sclerosis from Western India. METHODS: Twenty-five SSc patients as per ACR-EULAR 2013 criteria (classified based on pulmonary fibrosis and generalized fibrosis) and 25 age-sex matched controls were enrolled. Serum cytokine levels of IL-1ß, IL-4 and IL-6 were assessed by multiplex bead based immunoassay. RESULTS: Ten patients had Interstitial lung disease (ILD), whereas, 16 patients had generalized fibrosis. Anti-nuclear antibodies were seen in 22 patients (88%); antiScl70 in 15 patients (60%) and anti-Centromere antibodies in 5 patients (20%). Serum levels of IL-1ß in patients were significantly higher than healthy controls (p=0.0006). IL-4 levels in all SSc patients were marginally raised (p=0.0102), while IL-6 levels were significantly raised (p<0.0001). IL-4 was found to be significantly raised in SSc patients with ILD (p=0.021) as compared to patients without ILD. IL-1ß (p=0.0293) and IL-4 (p<0.0001) were significantly higher in SSc patients with fibrosis. On the contrary, IL-6 levels in patients with fibrosis were found to be lower than in patients without fibrosis. CONCLUSION: Significantly raised cytokine levels among treatment naïve systemic sclerosis patients were found to be associated with higher disease severity in our study. Higher levels of IL-1ß and IL-6 indicated an active inflammatory status, whereas significantly raised IL-4 levels indicated at higher fibrotic activity.

Multiple acquired smooth muscle hamartomas with thick-walled blood vessels on scalp.

Smooth muscle hamartoma (SMH) is a benign hamartomatous condition that consists of a dermal proliferation of smooth muscle cells. We hereby report a case of multiple, acquired SMHs on scalp. A 25-year-old male had a 6-year history of multiple, asymptomatic, hyperpigmented plaques on the scalp which were progressively increasing in number and size. Histopathological examination revealed large thick-walled blood vessels, in addition to the haphazardly arranged discrete smooth muscle bundles in the reticular dermis. This case of SMH is unusual in being acquired, having multiple lesions, involving scalp, and having thick-walled blood vessels apart from arrector pili muscle bundles. It emphasizes the wide spectrum of clinical and histopathological variations that may be associated with acquired SMH.

Granulomatous variant of giant centrifugal miliaria profunda.

Two infants presented with multiple asymptomatic papules and geographic and annular plaques over the extensor aspect of the upper and lower extremities and trunk. Skin biopsy of both lesions showed plugged and hyperplastic dilated acrosryingia and deep dermal ducts, along with granulomatous inflammatory reaction. These lesions showed self-healing with complete resolution. A previous report described similar clinical and histopathologic features and labeled it giant centrifugal miliaria profunda. Because of the large granulomatous plaques and deep infiltrate, we propose that it was a granulomatous variant of giant centrifugal miliaria profunda. We report these cases for their rarity and self-healing nature.

A Cross-Sectional Study to Correlate Disease Severity in Bullous Pemphigoid Patients with Serum Levels of Autoantibodies Against BP180 and BP230.

CONTEXT: Enzyme-linked immunosorbent assay (ELISA) for BP 180 and 230 antibodies is commonly done in patients with bullous pemphigoid. We could not find much data regarding the usefulness of this test to predict the disease severity in Indian population. AIMS: We studied the correlation of IgG anti BP180 and anti BP230 antibody titer with disease severity and clinical features in bullous pemphigoid. SETTINGS AND DESIGN: This cross-sectional study was conducted at a tertiary care center in western India. MATERIALS AND METHODS: Forty-two clinically diagnosed treatment-naive cases of bullous pemphigoid were enrolled and investigated with skin punch biopsy, IgG anti BP180, and anti BP230 ELISA, direct immunofluorescence, and indirect immunofluorescence tests. Disease severity was assessed by calculating modified Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) score. Thirty patients with a final diagnosis of bullous pemphigoid were included in the statistical analysis. Pearson's correlation coefficient (r) was used to study correlation. RESULTS: The mean ABSIS skin score was 32.81 when both tests were negative, 42.13 when only BP230 was positive, 76.28 when only BP180 was positive, and 78.16 when both were positive. Pearson's correlation coefficient (r) for BP180 and ABSIS skin score was 0.6 (P value: 0.0005), and for BP230 was -0.055 (P value: 0.600). CONCLUSIONS: BP antibody titers correlate partially with disease severity. Anti-BP180 antibody is associated with more severe disease. Anti-BP230 antibody titer does not correlate with disease severity.

Cutaneous plasmacytosis with mast cell infiltration.

Cutaneous plasmacytosis is a rare disorder of uncertain etiology, described mainly in patients of Japanese descent. Clinically, it is characterized by multiple pigmented papules and plaques distributed primarily on the trunk. Histopathologically, it is marked by a dense dermal plasma cell infiltrate. Here, we describe a case of cutaneous plasmacytosis in a 55-year-old Indian male who presented with hyperpigmented plaques on the body. Histopathological examination revealed dense superficial and deep perivascular and periappendageal infiltrate composed mainly of plasma cells, lymphoid follicles with reactive germinal centres, perineural distribution of plasma cells, mast cell infiltration and increased dermal small blood vessels. Immunohistochemical analysis confirmed the polyclonal nature of the plasma cells. Laboratory investigations were within normal limits, except for the presence of polyclonal hypergammaglobulinemia without any M band. There was no evidence of autoimmune disease or any infection. There was no systemic involvement in this patient. The patient was diagnosed as cutaneous plasmacytosis and advised long-term follow-up. Peculiar histopathological finding in this case of cutaneous plasmacytosis was the presence of abundant mast cells in the dermis.

Comparison of Dermoscopic Patterns at the Center and Periphery of Alopecia Areata Patch - A Cross-sectional Study in 100 Patients.

BACKGROUND: Dermoscopy is a noninvasive tool for the diagnosis of various dermatological disorders. Dermoscopy of alopecia areata (AA) aids in confirming the diagnosis and severity of the disease. MATERIALS AND METHODS: A total of 100 patients of AA were evaluated with a dermoscope (×20 and × 200) to determine the dermoscopic features at the center and periphery of patches of AA. RESULTS: Black dots were the most common dermoscopic finding at the periphery of AA patch among the study population, followed by yellow dots, vellus hair, broken hair, tapering/exclamation mark hair, kinking at the point of emergence of a hair shaft, piggy tail hair, and depressed follicular opening. At the center of the patch, yellow dots were the most common dermoscopic findings among the study population, followed by black dots, vellus hair, broken hair, and piggy tail hair. CONCLUSION: Black dots were the most common dermoscopic findings at the periphery of the patch. Yellow dots were the most common dermoscopic findings at the center of patch among the study population. Kinking at the point of emergence of the hair shaft was a new sign in this study seen at the periphery of the patches in 19 patients. It is the latest finding which requires further elaboration with larger sample size studies.

Use of Topical Steroids in Dermatology: A Questionnaire Based Study.

CONTEXT: Topical steroids, the most widely prescribed drugs in dermatology are being increasingly misused. AIMS: This study was conducted to assess knowledge and practices regarding the use of topical steroids and to analyze prescriptions containing topical steroids. SUBJECTS AND METHODS: Following approval from the institutional ethics committee, participants were recruited as per the selection criteria and divided into those treated in the institution and those having outside prescription. They were administered a pre-validated questionnaire to assess knowledge and practices regarding the use of topical steroids. STATISTICAL ANALYSIS USED: Comparison of awareness between two patient categories was done using Chi-square test. Prescription variables were analyzed using descriptive statistics. Significance of P value was set at 0.05. RESULTS: Out of 400 patients, 167 had external prescriptions whereas 233 were institutional patients. Only 5.5% of all patients knew about the type of drug prescribed whereas 31.25% were aware of the indication. A total of 33.75% of the patients knew topical steroids required a prescription and 5.6% said they were aware that topical steroid use was associated with side effects. Side effects were reported by 96 patients. Awareness regarding knowledge, indication, and need for prescription were significantly better in institutional patients whereas knowledge about side effects was lacking in both groups. Psoriasis was the most common indication overall whereas tinea was the most common indication (51.5%) among externally prescribed. CONCLUSIONS: Although this study showed that institutional patients had comparatively better knowledge than community-treated patients, there is a need to create more awareness among patients overall and implement measures to stop irrational prescribing practices in the community.

Clinicopathological and Dermoscopic Features in a Case of Linear and Unilateral Basaloid Follicular Hamartoma.

Linear and unilateral basaloid follicular hamartoma (BFH) is a rare disease that manifests with papules and plaques with distribution along lines of Blaschko. It runs a benign course but with the risk of basal cell carcinoma (BCC) in long term course. BCC can be differentiated from BFH with histopathology and immunohistochemistry. Dermoscopic features of BCC are well studied. Dermoscopic features of BFH are not yet described in literature in detail probably due to rarity of the disease. Here, we present a case of extensive linear and unilateral BFH with its clinicohistopathological and dermoscopic features. The lesions were extensive to involve scalp, face, neck, upper, and lower trunk. Dermoscopy revealed features similar to that of BCC including brown-grey globules and dots, in focus dots, brown linear and arciform structures, crown vessels, short fine telangiectasias, spoke wheel like structures without central dark point, white structureless areas with telangiectasias and keratotic plug. Other dermoscopic features of BCC like arborizing vessels, blue-grey ovoid nests, maple leaf-like areas, concentric structures, ulcerations, erosions and white streaks were absent in this case.

Granulomatous Variant of Giant Centrifugal Miliaria Profunda in a Hypothyroid Infant: A Case Report.

The miliarias are a clinically heterogeneous group of diseases which occur when the free flow of eccrine sweat to the skin surface is impeded. Miliaria profunda is a variant with obstruction of the duct at or below the level of dermoepidermal junction. The giant centrifugal variant of miliaria profunda has been described in the past at the sites of occlusive tapes and in febrile patients. Thyroid hormone has a regulatory effect on the skin and its appendages and an association of hypothyroidism with this variant of miliaria profunda has not been described in the past. We report a case of giant centrifugal miliaria profunda in an infant with congenital hypothyroidism.

A Case of Progressive Evolution of Multiple Woolly Hair Nevi in a Child.

Woolly hair nevus is a rarely acquired disorder of the scalp hair with well-circumscribed patch of curly and unruly hairs which are smaller in diameter than normal surrounding hair. We report a case of progressively evolving multiple woolly hair nevi in a 10-year-old child. Trichoscopy of unruly hairs showed abnormal kinking of hair shafts. Varying diameters of a single hair shaft, damaged cuticle, and trichorrhexis nodosa-like features were observed on hair microscopy. Histopathology showed abnormal bending of hair follicle above the hair bulb and irregularities of inner root sheath near the bulb at the bending. Most of the reported cases of woolly hair nevi had one or two stable patches, but this case presented with four patches which were progressively evolving.

Schopf-Schulz-Passarge Syndrome.

Schopf-Schulz-Passarge syndrome (SSPS) is a rare type of ectodermal dysplasia that has autosomal recessive inheritance. It is characterized by palmoplantar keratoderma, hypodontia, hypotrichosis, nail dystrophy, and multiple periocular and eyelid apocrine hidrocystomas. A 36-year-old male presented with multiple eyelid and periocular apocrine hidrocystomas, ichthyosis, palmoplantar keratoderma, hypodontia, nail dystrophy, and thin scalp hair. Skin biopsy from a periocular lesion revealed cyst lined with smooth, thin epithelium, and few areas revealed foci of decapitation secretion consistent with apocrine hidrocystoma. The patient was diagnosed with SSPS.

A study of prevalence of autoantibodies in patients with lichen planus from Mumbai, India.

BACKGROUND: Lichen planus is a common chronically relapsing autoimmune skin condition with poorly understood etiology. Apart from cellular immunity, presence of various antibodies has been hypothesized. Various studies have found the presence of serum anti-nuclear antibody, anti-mitochondrial antibody, anti-desmoglein 1 and 3 antibodies, anti-keratinocyte antibody and anti-thyroglobulin antibody in patients of cutaneous and oral lichen planus. AIM: To study the prevalence of autoantibodies and the clinical spectrum of disease in an Indian patient subpopulation with lichen planus. METHODS: A cross-sectional epidemiological study comprising 100 lichen planus patients was conducted in the dermatology outpatient department of Seth G.S Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra, India. Serum concentrations of circulating anti-nuclear antibodies, anti-desmoglein 1 antibody, anti-desmoglein 3 antibody, anti-keratinocyte antibodies, anti-mitochondrial antibodies and anti-thyroglobulin antibodies were determined by indirect immunofluorescence. Pairs of groups were compared using "Student's t-test" for normally distributed continuous data. The "χ2-test" was used for the categorical variables as needed. Statistical significance was set at P < 0.05. RESULTS: It was found that 65 (65%) patients showed the presence of at least one of the six autoantibodies that we studied, while 35 (35%) tested negative for all six of them. Positivity of anti-keratinocyte antibody in 26 (26%), anti-nuclear antibody in 22 (22%), anti-desmoglein 1 antibody in 19 (19%), anti-desmoglein 3 antibody in 16 (16%), anti-mitochondrial antibody in 9 (9%) and anti-thyroglobulin antibody in 6 (6%) patients was detected. It was observed that 55 (71.4%) patients of cutaneous lichen planus, 6 (46.1%) patients of mucosal lichen planus and 4 (40%) patients of cutaneous and mucosal lichen planus overlap showed presence of at least one autoantibody. CONCLUSION: This study provides the serological parameters of a population of lichen planus from western India. Presence of autoantibodies in lichen planus suggests the possible role of humoral immunity in lichen planus. Identifying antibodies linked to lichen planus may help in identifying suitable diagnostic tests and therapeutic targets. Well-controlled studies with larger sample size are the need of the hour to confirm the role of humoral immunity in lichen planus. LIMITATIONS: Studies with a larger number of patients as well as controls should be undertaken to further evaluate the role of autoantibodies in lichen planus.

Prevalence and Burden Related to Genital Warts in India.

The prevalence of genital warts (GW) and self-reported human papillomavirus (HPV) as well as disease-related psychosocial impact among male and female patients aged 18-60 years in India were assessed. GW prevalence was estimated using a 2-week daily log of patients examined from June 7-September 22, 2011 by 200 participating physicians in 6 regions of India. Psychosocial impact was estimated using one-time, self-administered surveys, including HPV Impact Profile (HIP), Cuestionario Específico para Condiloma Acuminado ([Spanish] CECA; "Specific questionnaire for Condylomata Acuminata") and EuroQol-5 Dimension survey. T-tests or Mann-Whitney U-tests were used for continuous comparisons and Chi-square or Fisher exact tests were applied for categorical comparisons. Overall GW prevalence in India was estimated at 1.07% (95% confidence interval = 0.97-1.17) and was higher among men than women. Regional prevalence ranged from high in Delhi (2.17%) to low in Bangalore (0.40%). Patients aged 25-29 years had the highest GW prevalence (1.42%). GW patients were most often newly diagnosed (74.07%). Among those with existing GW, 56.24% were recurrent, and 43.76% were resistant. According to total HIP scores, 55.4% of male GW patients and 20.0% of those without GW reported moderate psychological impact (p < 0.0001). HIP scores among women revealed that patients with abnormal Papanicolaou (Pap) test results (34.3%), precancerous lesions (46.2%), external GW (48.0%), and those without HPV-related disease (18.5%) reported moderate psychological impact (p = 0.0089) (Psychosocial impact results are reported in the Supplementary Data ). Estimated national GW prevalence, diagnosis, and treatment costs in India were higher for men than for women. GW in men and HPV infection in women had a negative psychosocial impact on well-being and health-related quality of life (HRQoL) scores, especially among women diagnosed with GW and precancerous lesions compared to those with other selected HPV-related diseases. Despite its limitations, this study provides an estimation of GW data in India not previously available.

Cutaneous collagenous vasculopathy: A rare case report.

Cutaneous collagenous vasculopathy (CCV) is a distinct, rare, and underdiagnosed condition. We report a case of CCV in a 50-year-old woman presenting as asymptomatic, erythematous to hyperpigmented nonblanchable macules over both the lower extremities. The clinical differential diagnosis of the lesions was pigmented purpuric dermatoses (Schamberg's purpura) and cutaneous small vessel vasculitis. Histology of the lesions revealed dilated superficial dermal vessels with abundant pink hyaline material in the vessel wall, which stained with periodic acid Schiff stain. The patient was diagnosed as CCV. This condition remains largely underdiagnosed and is commonly mistaken for pigmented purpuric dermatosis or generalized essential telangiectasia. Emphasis on the differentiation of CCV from its clinical and histological mimicks is made.

Fibroepithelioma of Pinkus in continuity with nodular basal cell carcinoma: A rare presentation.

Fibroepithelioma of Pinkus and nodular basal cell carcinoma (BCC) are different morphological variants of BCC. It is very rare to see both the variants together in a single lesion. Here we report a case of a 56-year-old female who presented with a nodule on the trunk, which on biopsy showed features of both nodular BCC and fibroepithelioma of Pinkus.

Asymptomatic Papulo-nodules Localized to One Finger.

Subcutaneous or deep granuloma annulare is a benign asymptomatic condition characterized by firm asymptomatic nodules in deep subcutaneous tissues that may be associated with intradermal lesions. A 53-year-old female presented with asymptomatic skin-colored, firm nodules over the right ring finger. Histopathology revealed a palisading granuloma with central degenerated collagen and mucin deposition in the dermis suggestive of granuloma annulare. Isolated and unilateral involvement of a single digit with clusters of nodules of subcutaneous granuloma annulare (GA) in an adult is rare and differentiation from its simulator rheumatoid nodule is essential.

Dermoscopy to Detect Signs of Subclinical Nail Involvement in Chronic Plaque Psoriasis: A Study of 68 Patients.

BACKGROUND: Onychopathies constitute one of the major challenges faced by a dermatologist in terms of its early detection and diagnosis. Utility of dermoscope as a tool for detection is increasing by the day and its use in onychopathies needs to be explored. AIMS: To study the dermoscopic features of nails in patients of chronic plaque psoriasis. MATERIALS AND METHODS: In a cross-sectional study, a total of 68 patients with chronic plaque psoriasis were recruited. Dermoscopy of nail plate was conducted and were compared with equal number of age and sex matched healthy volunteers. RESULTS: Forty-six patients showed dermoscopic findings. Twenty-two patients did not show any dermoscopic findings. Coarse pits (18/46, P < 0.0001), onycholysis (10/46, P < 0.001), oil drop sign (2/46, P = 0.12) and splinter hemorrhages (5/46, P = 0.05) were seen. In addition certain findings of interest were stout, globose, dilated, pink- to red-colored nail bed vessels arranged longitudinally at the onychodermal band surrounded by a prominent halo (9/46, P = 0.01). In contrast, splinter hemorrhages appeared as streaks and were purple in color. CONCLUSION: In a psoriasis patient, dermoscope can be a useful tool to detect early nail involvement in psoriasis and aid in differentiating it from other disorders of nails.