An Indian Expert Group Review of Acanthosis Nigricans with Recommendations for Early Detection and Timely Management.

INTRODUCTION: Acanthosis nigricans (AN) is a visible cutaneous manifestation of several underlying systemic conditions, such as prediabetes, diabetes mellitus (DM), polycystic ovarian syndrome (PCOS), metabolic syndrome (MS), and malignancy. AN is commonly seen in obese individuals and is a proven skin marker of insulin resistance (IR). Its typical clinical presentation and location, especially on the nape of the neck, allows for easy visual identification. Obesity, IR, and other noncommunicable diseases (NCD) are on the rise in India, with many cases being undiagnosed or missed. Every sixth diabetic in the world is Indian. AN can thus play a vital role in screening, early identification, and reduction of morbidity, especially of IR-related disorders. Currently, there are no national or international multidisciplinary guidelines or recommendations for AN. OBJECTIVE: To review published current literature and evidence on AN, its associations, and management. To recommend an approach to clinical identification of AN, associated/underlying disorders, investigations, and management, including lifestyle modifications. To sensitize and familiarize clinicians and healthcare workers across India with AN presentations. This will enable early detection of conditions such as IR, prediabetes, DM, PCOS, malignancy, etc., in practice and in the community. MATERIALS AND METHODS: The PCOS Society of India constituted a multispecialty Expert Working Group with representation from various national societies to review all published scientific evidence. Expert group meetings were conducted to provide recommendations. CONCLUSION: Acanthosis nigricans is a simple, non-invasive, cost-effective screening tool for early identification of IR. It is important to integrate and disseminate information and training on AN across medical specialties. The government of India has launched several National Health Programmes to help address the rising burden of NCD in the population. AN would provide a simple screening tool to pick up undiagnosed or missed cases.

Acquired reactive perforating collagenosis, a rare entity at uncommon site.

Acquired reactive perforating collagenosis (ARPC), rare disorder characterized by transepidermal elimination (TEE) of collagen fibers, is seen in adult diabetics. Genetic predisposition, familial aggregation, trauma, bites and scratching are implicated. Diabetics develop microvascular diseases leading to intense pruritus causing repeated micro trauma leading to necrosis of connective tissue of dermis, causing TEE. Isolated papules, plaques and nodules with central keratotic plugs, are mostly seen on extensor surfaces of limbs but trunk and face may be involved. Histopathology shows extrusion of abnormal collagen fibers through epidermis. Multiple treatment modalities show variable response. A 52 year old diabetic female had multiple, itchy, well defined, erythematous papules and plaques with central adherent crusting on lower back since 1 month. Histopathology showed cup shaped epidermal depression filled with plug of altered collagen, acanthotic epidermis with hyperkeratosis and parakeratosis. Underlying epidermis was thin with fine slits through which vertically oriented basophilic collagen fibers were extruded.

Lymphangiomas: Rare presentations in oral cavity and scrotum in pediatric age group.

The incidence of lymphangioma is 1.2 to 2.8/1000 newborns. They present at birth/before 2 years, with predilection for the head and neck (50%-70%). The buccal mucosa is the second most common site reported (14 cases reported) after the anterior two-thirds of tongue. The scrotum is a rare site with less than 50 cases reported (till 2002). Involvement of vital structures, aesthetic, and functional requirements may necessitate treatments such as surgical excision, radiation, cryotherapy, electrocautery, sclerotherapy, embolization, ligation, and laser. Two rare cases - the first being primary, late-onset buccal lymphangioma, with vesicular presentation, and the second being genital lymphangioma involving the right side of scrotum, thigh, and groin with extension to the left groin - are highlighted.

A descriptive study of facial acanthosis nigricans and its association with body mass index, waist circumference and insulin resistance using HOMA2 IR.

INTRODUCTION: The term facial acanthosis nigricans (FAN) lacks definition of precise clinical and histopathological features. We present a descriptive study of patients with FAN to define pigmentary patterns and estimate the prevalence of obesity and insulin resistance in these cases. MATERIALS AND METHODS: It is a prospective study that included all patients with classical AN of the neck and/or other areas with facial acanthosis nigricans described as brown-to-black macular pigmentation with blurred ill-defined margins, found on the zygomatic and malar areas. The body mass index (BMI) and waist circumference (WC) of the included patients were used as parameters of obesity. Homeostatic Model of Assessment of Insulin Resistance (HOMA2 IR) was used as a parameter to evaluate insulin resistance. Histopathological features of the 6 skin biopsies that were possible were reviewed. RESULTS: Among the 102 included individuals, the patterns of facial pigmentation seen in addition to the classic pattern involving zygomatic and malar areas were a hyperpigmented band on the forehead in 59.80%, periorbital darkening in 17.64%, perioral darkening in 12.74%, and generalized darkening in 9.8% of cases. 85.29% of the males and 100% of the females were found to be obese. Varying degrees of insulin resistance was noted in 82.34% of the individuals. Six biopsies available for evaluation showed changes such as mild epidermal hyperplasia with prominent basal melanin, however, without the typical papillomatosis seen in AN of the flexures. CONCLUSION: We document an increased prevalence of obesity and insulin resistance in patients presenting with FAN and its presentations in addition to the classical description. We propose that FAN can be considered a cutaneous marker of insulin resistance and that HOMA2 IR can serve as a parameter of insulin resistance in such cases.

Vitiligo in Children: A Birds Eye View.

Vitiligo in children is a distinct subset of vitiligo and differs from adult vitiligo. Characteristic features include family history of autoimmune or endocrine disease, higher incidence of segmental vitiligo, development of early or premature graying, increased incidence of autoantibodies and poor response to topical PUVA. The exact prevalence of vitiligo in children varies between 0.1-4% of the world population and seems to be higher in India than in other countries and it occurs more frequently in females. Around 12% to 35% of pediatric vitiligo patients have family members with the disease. The most common type of vitiligo in pediatric patients is vitiligo vulgaris, representing 78% of cases. The most commonly associated autoimmune disease is thyroiditis. Phototherapy and topical corticosteroids are the most commonly used treatments for adult vitiligo but are less useful in the pediatric population.

"Childhood Vitiligo".

Vitiligo in children is a distinct subset of vitiligo and differs from adult vitiligo. Characteristic features include family history of autoimmune or endocrine disease, higher incidence of segmental vitiligo , development of early or premature graying, increased incidence of autoantibodies and poor response to topical PUVA. The exact prevalence of vitiligo in children varies between 0.1-4% of the world population and seems to be higher in India than in other countries and it occurs more frequently in females. Around 12% to 35% of pediatric vitiligo patients have family members with the disease. The most common type of vitiligo in pediatric patients is vitiligo vulgaris, representing 78% of cases. The most commonly associated autoimmune disease is thyroiditis. Phototherapy and topical corticosteroids are the most commonly used treatments for adult vitiligo but are less useful in the pediatric population.

Side-effects of topical steroids: A long overdue revisit.

The introduction of topical steroids (TS) of varying potency have rendered the therapy of inflammatory cutaneous disorders more effective and less time-consuming. However the usefulness of these has become a double edged sword with constantly rising instances of abuse and misuse leading to serious local, systemic and psychological side effects. These side effects occur more with TS of higher potency and on particular areas of the body like face and genitalia. The article reviews the side effects of TS with special mention about peadiatric age group, also includes the measures for preventing the side effects.

An approach to acanthosis nigricans.

Acanthosis nigricans (AN) is characterized by dark, coarse and thickened skin with a velvety texture, being symmetrically distributed on the neck, the axillae, antecubital and popliteal fossae, and groin folds, histopathologically characterized by papillomatosis and hyperkeratosis of the skin. A high prevalence of AN has been observed recently. Different varieties of AN include benign, obesity associated, syndromic, malignant, acral, unilateral, medication-induced and mixed AN. Diagnosis is largely clinical with histopathology needed only for confirmation. Other investigations needed are fasting lipoprotein profile, fasting glucose, fasting insulin, hemoglobin and alanine aminotransferase for obesity associated AN and radiological investigations (plain radiography, ultrasonography, magnetic resonance imaging/computerized tomography) for malignancy associated AN. The most common treatment modalities include retinoids and metformin.

Trichosporon inkin and Trichosporon mucoides as unusual causes of white piedra of scalp hair.

White piedra of scalp hair is considered a rare entity. We report three cases of this disorder all of whom presented with nodules on the hair. Potassium hydroxide preparations of the hair revealed clustered arthrospores and mature, easily detachable nodules. Cultures grew Trichosporon inkin in 2 patients and Trichosporon mucoides in one patient. Both these fungi are unusual causes of white piedra.

Current trends in congenital syphilis.

In many parts of the world particularly sub-Saharan Africa, congenital syphilis is a significant public health problem. Though it is rare in most affluent countries there has been a slight resurgence recently in several European countries. The diagnosis of suspected cases and management of congenital syphilis may be confusing and the potential for severe disability is high when cases are missed. The cornerstone of congenital syphilis control is antenatal screening and treatment of mothers with penicillin and in affluent countries it should be strengthened among those at high risk. In developing countries antenatal care screening needs to be strengthened by implementing point-of-care decentralised screening and treatment, also alternative novel approaches to control congenital syphilis should be looked at and utilized. International health agencies and political parties should take steps urgently to support focused approaches to tackling the problem of continuing congenital syphilis.

Systemic sclerosis: current concepts in pathogenesis and therapeutic aspects of dermatological manifestations.

Systemic sclerosis (SSc) is a chronic, multisystem connective tissue disease with protean clinical manifestations. Recent advances in understanding the pathogenic mechanisms have led to development of target-oriented and vasomodulatory drugs which play a pivotal role in treating various dermatological manifestations. An exhaustive literature search was done using Medline, Embase, and Cochrane library to review the recent concepts regarding pathogenesis and evidence-based treatment of salient dermatological manifestations. The concept of shared genetic risk factors for the development of autoimmune diseases is seen in SSc. It is divided into fibroproliferative and inflammatory groups based on genome-wide molecular profiling. Genetic, infectious, and environmental factors play a key role; vascular injury, fibrosis, and immune activation are the chief pathogenic factors. Vitamin D deficiency has been documented in SSc and correlates with the severity of skin involvement. Skin sclerosis, Raynaud's phenomenon (RP) with digital vasculopathies, pigmentation, calcinosis, and leg ulcers affect the patient's quality of life. Immunosuppressives, biologicals, and hematopoietic stem cell transplantation are efficacious in skin sclerosis. Endothelin A receptor antagonists, calcium-channel blockers, angiotensin receptor inhibitors, prostacyclin analogs, and phosphodiesterase type 5 (PDE-5) inhibitors are the mainstay in RP and digital vasculopathies. Pigmentation in SSc has been attributed to melanogenic potential of endothelin-1 (ET-1); the role of ET 1 antagonists and vitamin D analogs needs to be investigated. Sexual dysfunction in both male and female patients has been attributed to vasculopathy and fibrosis, wherein PDE-5 inhibitors are found to be useful. The future concepts of treating SSc may be based on the gene expression signature.

Comparative assessment of the efficacy and safety of sertaconazole (2%) cream versus terbinafine cream (1%) versus luliconazole (1%) cream in patients with dermatophytoses: a pilot study.

BACKGROUND: Sertaconazole is a new, broad spectrum, fungicidal and fungistatic imidazole with added antipruritic and anti-inflammatory activity that would provide greater symptomatic relief and hence would be beneficial in improving the quality of life for the patient with dermatophytoses. AIMS AND OBJECTIVES: To compare efficacy and safety of sertaconazole, terbinafine and luliconazole in patients with dermatophytoses. MATERIALS AND METHODS: 83 patients with tinea corporis and tinea cruris infections were enrolled in this multicentre, randomized, open label parallel study. The initial 'Treatment Phase' involved three groups receiving either sertaconazole 2% cream applied topically twice daily for four weeks, terbinafine 1% cream once daily for two weeks, luliconazole 1% cream once daily for two weeks. At the end of treatment phase, there was a 'Follow-up Phase' at end of 2 weeks, where the patients were assessed clinically and mycologically for relapse. RESULTS: Of the 83 patients, 62 completed the study, sertaconazole (n = 20), terbinafine (n = 22) and luliconazole (n = 20). The primary efficacy variables including change in pruritus, erythema, vesicle, desquamation and mycological cure were significantly improved in all the three groups, as compared to baseline, in the Treatment and Follow-up phase. Greater proportion of patients in sertaconazole group (85%) showed resolution of pruritus as compared to terbinafine (54.6%); and luliconazole (70%), (P < 0.05 sertaconazole vs terbinafine). There was a greater reduction in mean total composite score (pruritus, erythema, vesicle and desquamation) in sertaconazole group (97.1%) as compared to terbinafine (91.2%) and luliconazole (92.9%). All groups showed equal negative mycological assessment without any relapses. All three study drugs were well tolerated. Only one patient in sertaconazole group withdrew from the study due to suspected allergic contact dermatitis. CONCLUSION: Sertaconazole was better than terbinafine and luliconazole in relieving signs and symptoms during study and follow up period. At the end of 'Treatment Phase' and 'Follow-up' Phase, all patients showed negative mycological assessment in all three treatment groups suggesting no recurrence of the disease.

Keratoacanthoma centrifugum marginatum at an unusual site.

Keratoacanthoma (KA) is a rapidly evolving tumor, composed of keratinizing squamous cells originating in pilosebaceous follicles and resolving spontaneously if left untreated. It is relatively uncommon in dark-skinned and occurs in middle aged individuals. Males are three times more affected than females. It presents as firm, rounded, flesh-colored or reddish papule; with a rapid growth phase followed by spontaneous healing over three months. Two types of KA exist i.e., solitary and multiple. There are three rare clinical variants of solitary KA, namely giant KA, keratoacanthoma centrifugum marginatum (KCM) and subungual KA. In KCM, lesions are large, reaching upto 20cms. There is peripheral extension with raised, rolled border and atrophy in the center. There is no tendency toward spontaneous involution. The most common locations are dorsa of hands and legs, lesions on scalp being rare. A rare case of KCM occurring on scalp which is an unusual site is reported.

Blastomycosis-like pyoderma.

A 9-year-old female, presented with recurrent bilaterally symmetrically distributed flesh colored vegetative plaques, papules and nodules on trunk and upper and lower extremities since 15 days. Investigations revealed anemia, hypoproteinemia, decreased albumin and positive D-xylose test. Pus swab and biopsy for culture sensitivity showed Enterococci species. Biopsy showed spongiotic psoriasiform dermatitis with subcorneal pustule. She fulfilled criteria for the diagnosis of blastomycosis-like pyoderma viz. presentation of large verrucous plaques with pustules and elevated border, pseudoepitheliomatous hyperplasia with abscess histologically and growth of one pathogenic bacterium on culture or tissue biopsy. She responded to long-term amoxicillin-clavulanic acid therapy.