Elastophagocytosis and Elastolysis in Leprosy.

Elastophagocytosis is the engulfment of the elastic fibres by the histiocytes, multinucleated giant cells, or both. The cutaneous lesions showing elastophagocytosis are annular elastolytic giant cell granuloma, actinic keratoses, persistent insect-bite reactions, elastosis perforans serpiginosa, foreign body granuloma. Occasionally, it may occur in infectious diseases like leprosy, granulomatous syphilis, North-American blastomycosis, bacterial folliculitis, and cutaneous leishmaniasis. We report a case of lepromatous leprosy with necrotic erythema nodosum leprosum with secondary anetoderma. Histopathology from the atrophic macule of anetoderma revealed periappendageal, perineural infiltration, elastophagocytosis and reduction in elastic fibres.

Chanarin-Dorfman syndrome: clinical report and novel mutation in ABHD5 gene.

Chanarin-Dorfman syndrome (CDS) is a multisystem, autosomal recessive genetic disorder characterized by congenital non-bullous ichthyosiform erythroderma with accumulation of lipid droplets in granulocytes and basal keratinocytes. An 18-month-old female child presented with typical dermatological features of CDS. She was born as a collodion baby. Liver biopsy showed micronodular cirrhosis along with macrovesicular hepatic steatosis. Sequencing of all exons and exon-intron boundaries of the ABHD5 gene showed that the patient was homozygous for a novel mutation g.24947delG (c.773 + 1delG) in intron 5. This is the first Indian child with mutation proven CDS.

The angiotensin-converting enzyme gene insertion/deletion polymorphism in Indian patients with vitiligo: a case-control study and meta-analysis.

BACKGROUND: Vitiligo is a common, acquired, idiopathic depigmenting skin disorder. Although the exact pathogenesis remains unknown, genetic susceptibility and autoimmune responses play a role in vitiligo development. Previous studies have suggested that the D allele of the insertion/deletion (I/D) polymorphism of the angiotensin-converting enzyme (ACE) gene is associated with vitiligo in Indians and Koreans. Furthermore, significantly higher serum ACE levels have been demonstrated in patients with some autoimmune and autoinflammatory disorders. OBJECTIVES: The objectives were to investigate any association between the ACE I/D polymorphism and vitiligo susceptibility in an Indian population, and to compare serum ACE levels in patients with vitiligo and healthy subjects. METHODS: The ACE I/D genotypes of 79 patients with vitiligo and 100 normal individuals were determined by polymerase chain reaction amplification. A meta-analysis was done to compare the distribution of the ACE I/D alleles and genotypes in the current and three previous studies. Serum ACE levels were evaluated by enzyme-linked immunosorbent assay. RESULTS: A significant increase in the frequency of the ACE I/D D allele was evident in patients with vitiligo in both the case-control study [P=0·005; odds ratio (OR) 1·87; 95% confidence intervals (CI) 1·22-2·85] and the meta-analysis (P=0·044; OR 1·44; 95% CI 1·01-2·06). Serum ACE levels were significantly increased in patients with vitiligo compared with healthy subjects (P<0·0001). CONCLUSIONS: In agreement with earlier reports, the ACE I/D D allele is associated with vitiligo susceptibility in the Indian population. The significantly elevated serum ACE levels in our cohort of patients with vitiligo concur with those previously found in patients with some other autoimmune diseases.

Vitiligo patients from India (Mumbai) show differences in clinical, demographic and autoantibody profiles compared to patients in western countries.

BACKGROUND: Vitiligo is a common, idiopathic skin disorder characterized by depigmented skin due to the loss of cutaneous melanocytes. Several studies have reported the clinical and demographic characteristics of Indian vitiligo patients, however, none has characterized their antibody profiles. OBJECTIVE: To establish the clinical, demographic and serological details of a population of vitiligo patients from Mumbai, India, and to evaluate the data for any associations between clinical presentations and the occurrence of antibody responses. METHODS: Vitiligo patients (n = 79) were recruited to the study and their clinical and demographic details recorded. Serum antibodies, including those against melanocyte-specific antigens, thyroid antigens and keratinocytes, were evaluated. RESULTS: The prevalence of vitiligo was independent of sex, and non-segmental vitiligo was the most common form of the disease occurring in 65% of the patients. Patients with segmental vitiligo (mean age = 14.4 ± 4.6 years) presented at a younger age than those with non-segmental disease (mean age = 32.5 ± 17.8 years). Personal and family histories of other autoimmune diseases occurred in 3% and 8% of patients, respectively. Antibodies were detected against tyrosinase, tyrosine hydroxylase, thyroid peroxidase, thyroglobulin and keratinocytes at frequencies of 11%, 22%, 18%, 24% and 27%, respectively. Overall, antibodies were more common in patients with non-segmental vitiligo (50-67%) than in those with segmental disease (0-17%), and were detected more frequently in patients with shorter disease durations (<10 years). CONCLUSION: Our study provides novel information relative to the clinical details, demographic features and serological parameters of a population of vitiligo patients from Mumbai, India. Important distinctions from similar surveys conducted in European patients were evident such as an infrequency of family history, a low prevalence of clinical autoimmune disease, and an absence of particular antibody specificities. These differences may have a bearing on the pathogenesis and course of the disease in Indian patients.

Is methicillin-resistant Staphylococcus aureus involved in community acquired skin and soft tissue infections? Experience from a tertiary care centre in Mumbai.

BACKGROUND: To improve the empiric antimicrobial therapy of community-acquired (CA) skin and soft tissue infections (SSTIs), it is necessary to generate data on the current spectrum and susceptibility profile of associated bacteria. CA methicillin-resistant Staphylococcus aureus (CA MRSA) is increasingly being reported in SSTIs in India and globally. AIMS: The present study was undertaken to determine the bacterial profile of CA-SSTIs, to know the contribution of MRSA in these infections, to determine inducible clindamycin resistance in S. aureus and to compare the resistance patterns of isolates from hospital-acquired (HA) SSTIs. MATERIALS AND METHODS: Eight hundred and twenty patients with CA SSTIs were prospectively studied. Pus samples were cultured and antimicrobial susceptibility pattern determined. Inducible clindamycin resistance was detected by D-test. Laboratory records were analyzed retrospectively to generate data on HA SSTIs. RESULTS: 619 isolates were recovered in CA-SSTIs, of which S. aureus (73%) and Streptococci (12%) were the most common. Pseudomonas aeruginosa (28%) and Acinetobacter spp (18%) were the predominant HA-SSTI pathogens. Susceptibility of CA S. aureus to antibiotics tested was, penicillin (6%), co-trimoxazole (20%), ciprofloxacin (37%), cefazolin (100%), erythromycin (84%), clindamycin (97%), gentamicin (94%) and fusidic acid (95%). No MRSA was found in CA SSTIs whereas 45% of HA S. aureus strains were methicillin-resistant. HA strains demonstrated significantly higher resistance as compared to their CA counterparts (P<0.001). D test was positive in 22% of CA S. aureus tested. CONCLUSIONS: In CA SSTIs, methicillin-susceptible S. aureus is the predominant pathogen. Penicillinase-resistant penicillins, clindamycin and erythromycin in that order can be used as suitable antimicrobials for empiric therapy. D test should be carried out routinely. No CA MRSA was detected in the present series.

Hla-a and hla-B alleles associated in psoriasis patients from mumbai, Western India.

BACKGROUND: Psoriasis, a common autoimmune disorder characterized by T cell-mediated keratinocyte hyperproliferation, is known to be associated with the presence of certain specific Human Leukocyte Antigen (HLA) alleles. AIM: To evaluate distribution of HLA-A and HLA-B alleles and hence identify the susceptible allele of psoriasis from patients in Western India. MATERIALS AND METHODS: The study design included 84 psoriasis patients and 291 normal individuals as controls from same geographical region. HLA-A and HLA-B typing was done using Serology typing. Standard statistical analysis was followed to identify the odds ratio (OR), allele frequencies, and significant P value using Graphpad software. RESULTS: The study revealed significant increase in frequencies of HLA-A2 (OR-3.976, P<0.0001), B8 (OR-5.647, P<0.0001), B17 (OR-5.452, P<0.0001), and B44 (OR-50.460, P<0.0001), when compared with controls. Furthermore, the frequencies of HLA-A28 (OR-0.074, P=0.0024), B5 (OR-0.059, P<0.0001), B12 (OR-0.051, P=0.0002), and B15 (OR-0.237, P=0.0230) were significantly decreased in psoriasis patients. CONCLUSION: This study shows the strong association of HLA-A2, B8, and B17 antigens with psoriasis conferring susceptibility to psoriasis patients from Western India, while the antigens HLA-A28, B5, and B12 show strong negative association with the disease.

Esomeprazole-induced photoallergic dermatitis.

There are no published case reports of esomeprazole-induced photoallergic dermatitis. We report here a 58-year-old lady with prior history of propylthiouracil and carbimazole-induced photoallergy, who presented with heartburn and dysphagia. She was diagnosed to have erosive esophagitis and was treated with esomeprazole, following which she developed photoallergic dermatitis. It improved on cessation of the drug and did not recur on subsequent treatment with ranitidine. Naranjo score for this adverse drug event was 8, thereby making it a probable adverse drug reaction. This reaction may be due to sulphur moiety, which is common to all these drugs. Physicians must be aware of this possible side-effect, especially in patients with prior history of photoallergy to other drugs.

Keratotic vascular papules over the feet: a case of Waldenström's macroglobulinaemia-associated cutaneous macroglobulinosis.

Waldenström's macroglobulinaemia (WM) is a plasma-cell dyscrasia characterized by the monoclonal proliferation of lymphoplasmacytes. A 48-year-old man presented with a 4-year history of multiple painful, hyperkeratotic deep-seated papules over the pressure areas of both soles. He had a 1-year history of Raynaud's phenomenon, intermittent epistaxis, recurrent vomiting, tingling and numbness, and visual disturbances. Histological examination of a skin biopsy found amyloid-like deposits in the upper and mid dermis involving dermal blood vessels, but apart from periodic-acid-Schiff, various stains gave negative results for amyloid. Direct immunofluorescence was positive for IgM antibody. Hence, a diagnosis of WM with cutaneous macroglobulinosis was made. Immunoelectrophoresis found monoclonal IgM kappa antibody, and bone-marrow examination revealed a lymphoplasmacytoid malignancy. The patient's systemic systems were attributed to hyperviscosity syndrome associated with WM and the cutaneous papules were identified as deposits of excess IgM antibodies. The patient received five cycles of chemotherapy, resulting in nearly complete resolution of the skin lesions and systemic symptoms.

Kaposi's sarcoma: a presenting manifestation of HIV infection in an Indian.

Kaposi's sarcoma (KS) is a multifocal neoplastic proliferation of endothelial cells predominantly involving skin and other organs. HIV-associated Kaposi's sarcoma has been rarely reported from India. A 38-year-old male presented with persistent swelling on the left lower limb for one year along with multiple erythematous to dusky papules and plaques of one and half months duration and swelling and black discoloration of right lower limb for one month. Cutaneous examination revealed numerous skin colored and erythematous papules and plaques distributed on the left lower limb on the anteromedial aspect and verrucous plaque on the left sole. Multiple erythematous, grouped papules were present over the soft palate. Skin biopsy showed numerous slit like spaces dissecting into the collagen of the upper and mid-dermis along with 'promontory sign' suggestive of Kaposi's sarcoma. Patient was found to be HIV-positive by ELISA test.

Adult cutaneous myofibroma.

A 63-year-old male presented with an asymptomatic, slow-growing swelling on the right lower limb for the past one and half years. The histopathology revealed a lobular neoplasm with a biphasic pattern of spindle shaped cells and hemangiopericytoma like areas at the periphery of the lobule. The diagnosis of adult cutaneous myofibroma was made. This case highlights the importance of histopathology in reaching a definitive diagnosis.

An outbreak of methicillin-resistant Staphylococcus aureus (MRSA) infection in dermatology indoor patients.

BACKGROUND: Methicillin-resistant Staphylococcus aureus (MRSA) is a major nosocomial pathogen. Indiscriminate and increased use of systemic antibiotics has led to the emergence of MRSA. Infected or colonized ward patients are the main reservoir of infection. Once colonized, the risk of subsequent local and systemic infections is high, especially in the elderly, and in debilitated and immunosuppressed patients. METHODS: We report an outbreak of MRSA in the dermatology ward of a tertiary care hospital and describe measures taken to control it. RESULTS: Ten patients were found to be MRSA positive over a span of three months while screening swabs from wet lesions in indoor patients. On the basis of risk assessment, they were treated with appropriate systemic and topical therapy. One patient died while the remaining nine patients showed a good response to therapy. All the MRSA isolates were found to be sensitive to vancomycin, teicoplanin and linezolid. CONCLUSION: This is the first case report of MRSA infection in dermatology indoor patients in India.

Acanthosis nigricans: a dermatologic marker of metabolic disease.

Most patients with acanthosis nigricans have either clinical or subclinical insulin resistance. We undertook a study to estimate the insulin sensitivity of a group of patients referred from the dermatologist with biopsy proven acanthosis nigricans. Thirty-six patients were evaluated in the Endocrinology clinic. Plasma glucose and serum Insulin levels were estimated after a 75 gms oral glucose load (OGTT). An intravenous Insulin Tolerance Test (ITT) was performed with measurement of Glucose Disposal Rate (GDR). There were 28 females and 8 males (M:F--3.5:1; mean age 26.3+/-1.7 years) in the study. 25/36 patients were morbidly obese (BMI--36.0 +/- 1.2 Kg/m2) with an abnormal body fat distribution (WH ratio--0.9 +/ - 0.02). One patient had generalized lipoatrophy. 16/36 patients with acanthosis nigricans had IGT or overt diabetes and all had highly significant hyperinsulinemia (AUCI = 20825 +/ 1287.7 vs. 6340.0 +/- 984.2 mIU/ml/hr in controls, p < 0.0005). The GDR in patients with acanthosis nigricans was reduced (-0.66 +/- 0.07) compared to controls (-0.39 +/- 0.08; p < 0.01). There was a significant positive correlation between indices of adiposity and insulin resistance in subjects with impaired tolerance.

A study of insulin resistance in subjects with acanthosis nigricans.

AIM: To study the prevalence of insulin resistance (IR) and its sequelae in patients with acanthosis. METHODOLOGY: Thirty six patients (28 females; eight males) with biopsy proven acanthosis nigricans and eight controls were evaluated for insulin sensitivity (IS) by estimating (a) the glucose and insulin responses to a 75 gm glucose load (Oral glucose tolerance test-OGTT), (b) the glucose disposal rate (GDR) during an intravenous insulin tolerance test (ITT). Serum androgen levels (testosterone--Te, androstenedione--ASD, Dehydro-epiandrosterone sulphate--DHEAS) were estimated in the basal state and 60 min after a bolus of insulin. Thyroid function tests (tri-iodo-thyronine--T3, thyroxine--T4, thyroid stimulating hormone--TSH) were performed in all subjects. RESULTS: The acanthotic population, overall had insignificant hyperglycemia (Area under curve of glucose--AUC-G : 17,745.5 +/- 847.5 v/s 11,051.3 +/- 274.5 mg/dl/min) and hyperinsulinemia (Area under curve of insulin -AUC-I: 20,825.2 +/- 1,287.7 v/s 6,340.1 +/- 984.2 microlU/ml/min) compared to controls during OGTT. Eight patients with acanthosis nigricans had impaired glucose tolerance and eight had overt diabetes using WHO criteria. 69.4% of the acanthotic subjects were obese and 13.9% (5/36) were hypertensive. Thyroid dysfunction was present in three (one had hypothyroidism and two had thyrotoxicosis). Reproductive disorders--menstrual irregularity (46.5%), amenorrhea (21.4%), hirsuitism (21.4%) and infertility (3.6%) was encountered in a significant number of acanthotics. Acanthotics overall had statistically higher levels of androgens; Te (females)--0.74 +/- 0.09 v/s 0.27 +/- 0.09 ng/ml (p < 0.005), ASD--1.8 +/- 0.21 v/s 0.94 +/- 0.2 ng/ml (p < 0.005) and DHEAS--1,880.8 +/- 216.3 v/s 772.8 +/- 210.4 ng/ml (p < 0.005). An elevated DHEAS correlated positively to body mass index (BMI) and android obesity. Serum Te levels correlated positively with GDR. Serum insulin levels increased progressively with obesity and acanthosis. Serum insulin was associated with progressive worsening of hyperandrogenism (as seen in non-obese controls, non-obese and obese acanthotics). CONCLUSIONS: Subjects with acanthosis nigricans should be screened for insulin resistance and its clinical and metabolic sequelae. Thyroid dysfunction should be sought in these subjects as it can be easily treated.

A comparison of systemic photochemotherapy with 8-methoxypsoralen (8-MOP) and with trimethylpsoralen (TMP) in vitiligo.

Oral 8-MOP and TMP were compared in the PUVA therapy for vitiligo. Group A (25 cases) was initiated on 0.3 mg/kg of 8-MOP with 1/2 Joule/cm2 of UVA and weekly increments of 1/2 Joule/ cm2 and Group B was started on 0.6 mg/kg of TMP with 1 Joule/cm2 of UVA and weekly increments of 1 Joule/cm2. Therapy was given thrice a week. Repigmentation was evaluated by using a 0-6 scale. At the end of 60 sittings, on acceptable cosmetic response was seen over the face, neck and upper extremities in both groups, while trunk and lower extremities showed lesser response. 8-MOP gave earlier response, needing a lower cumulative UVA dose i.e. 75 J/cm2 as compared to TMP i.e. 106 J/cm2. Phototoxicity was seen more often with 8-MOP. In conclusion, in Indians, 8-MOP is the drug of choice in PUVA therapy of vitiligo provided precautions against phototoxicity are adequate.

Disseminate and persistent infundibulo-folliculitis.

An 18-year-old girl presented with a persistent skin eruption of 6 years duration. She had skin-coloured follicular papules involving the trunk, proximal extremities, neck and face. Histopathology revealed spongiosis of the follicular infundibulum and infiltration with lymphocytes and neutrophils. Treatment with oral vitamin A and topical retinoic acid was ineffective.