Atypical leishmaniasis: A global perspective with emphasis on the Indian subcontinent.

BACKGROUND: Among the neglected tropical diseases, leishmaniasis continues to be prevalent in many tropical and subtropical countries despite international, national, and local efforts towards its control and elimination over the last decade. This warrants a critical evaluation of such factors as under-reporting, asymptomatic infections, post kala azar dermal leishmaniasis (PKDL) cases, and drug resistance. In this review, we highlight lesser-understood atypical presentations of the disease involving atypical parasite strains against a background of classical leishmaniasis with a focus on the Indian subcontinent. METHODS AND FINDINGS: A literature review based on endemic areas, the nature of disease manifestation, and underlying causative parasite was performed with data collected from WHO reports for each country. Searches on PubMed included the term ''leishmaniasis" and "leishmaniasis epidemiology" alone and in combination with each of the endemic countries, Leishmania species, cutaneous, visceral, endemic, non-endemic, typical, classical, atypical, and unusual with no date limit and published in English up to September 2017. Our findings portray a scenario with a wider distribution of the disease in new endemic foci, with new discoveries of parasite-driven atypical disease manifestations in different regions of the world. Unlike the classical picture, some Leishmania species are associated with more than one disease presentation, e.g., the L. donovani complex, generally associated with the visceral form, is now also associated with a cutaneous disease presentation, while L. tropica species complex, known to cause cutaneous disease, can cause viscerotropic disease. This phenomenon points towards the discovery of novel parasite variants as etiologic agents of atypical disease manifestations and represents an excellent opportunity to identify and study genes that control disease virulence and tropism. CONCLUSIONS: The increased recognition of atypical leishmaniasis as an outcome of parasite variants has major implications for leishmaniasis control and elimination. Identifying molecular correlates of parasite isolates from distinct regions associated with different disease phenotypes is required to understand the current epidemiology of leishmaniasis in regions with atypical disease.

Primary cutaneous anaplastic large cell lymphoma masquerading as large pyogenic granuloma.

Primary cutaneous anaplastic large cell lymphoma (pcALCL) forms 9% of the cutaneous T-cell lymphomas. It usually presents as solitary reddish brown ulcerating nodule or indurated plaque. Sometimes, it mimics other dermatological diseases such as eczema, pyoderma gangrenosum, pyogenic granuloma, morphea, and squamous cell carcinoma. Our case presented with large pyogenic granuloma like lesion with regional lymphadenopathy. Since pcALCL is rare, one can misdiagnose such cases and therefore high index of suspicion is necessary.

Ectodermal Dysplasia-Skin Fragility Syndrome: A Rare Case Report.

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.

Hutchinson - Gilford progeria syndrome: A rare case report.

Hutchinson - Gilford Progeria Syndrome is a rare genetic disorder characterized by premature aging involving the skin, bones, heart, and blood vessels. We report a three-year-old boy with clinical manifestations characteristic of this syndrome. He had a characteristic "plucked-bird" appearance, prominent eyes and scalp veins, senile look, loss of scalp hair, eyebrows, and eyelashes, stunted growth, and mottled pigmentation with sclerodermatous changes over the trunk and lower limbs. Radiological changes and decreased high-density lipoprotein (HDL) levels were also characteristic of the syndrome. This interesting case is reported for its rarity.

Pediatric cervicofacial actinomycosis disclosing an underlying congenital dermoid cyst.

Pediatric cervicofacial actinomycosis is a rare occurrence consequent to dental infections and manipulations or maxillofacial trauma. The clinical presentation ranges from multiple draining sinuses to swellings resembling tumors and cysts. The present unusual case had congenital dermoid cyst of mid upper lip with Actinomyces israelii infection identified on microscopy, culture, and histopathology. A successful outcome in the present case was obtained using combination of medical and surgical treatment.

A case of extensive chromoblastomycosis from North India.

A case of extensive chromoblastomycosis of the right leg and thigh with verruciform to nodular lesions evolving rapidly over five years duration is reported. The diagnosis was confirmed by visualizing pathognomonic pigmented muriform bodies with unique septate hyphae and mycological culture yielding Fonsecaea pedrosoi.

A case of extensive chromoblastomycosis from North India

A case of extensive chromoblastomycosis of the right leg and thigh with verruciform to nodular lesions evolving rapidly over five years duration is reported. The diagnosis was confirmed by visualizing pathognomonic pigmented muriform bodies with unique septate hyphae and mycological culture yielding Fonsecaea pedrosoi.

Dermatopathia pigmentosa reticularis: A rare reticulate pigmentary disorder.

Dermatopathia pigmentosa reticularis is a rare ectodermal dysplasia with a triad of generalized reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy. We report a case of a 21 year old woman who had generalized reticulate pigmentation, diffuse noncicatricial alopecia and onychodystrophy of finger and toe nails. Along with this triad she had palmoplantar keratoderma and poorly developed dermatoglyphics. There was no evidence of involvement of other ectodermally derived organ.

Clinico-investigative Profile of Patients of Hirsutism in a Tertiary Level Institution.

INTRODUCTION: Hirsutism has a significant impact on the quality of life and serves as a marker of underlying hormonal and systemic conditions. The aim of this study was to study the clinical, biochemical characteristics of these patients and other associations. MATERIALS AND METHODS: Fifty (n=50) consecutive newly diagnosed patients of hirsutism were assessed during a period from August 2009 to July 2010 using modified Ferriman Gallwey (mF-G) score. RESULTS: Idiopathic hirsutism (IH) was found in 30 (60%) patients followed by polycystic ovarian syndrome (PCOS) in 19 (38%) patients. Other causes included late-onset classic adrenal hyperplasia in two (4%) and hypothyroidism in four (8%) patients. The mean age at presentation was 23.8±6.657 years. Total (T) and free testosterone (fT), 17-hydroxyprogesterone was significantly higher in PCOS than IH. CONCLUSION: The present data show IH as the commonest cause of hirsutism in our study population. Face, chest, and lower abdomen have a higher impact on the hirsutism score while upper back, abdomen, and lower back are rarely involved.

A rare case of diffuse cutaneous leishmaniasis in an immunocompetent patient from sub-Himalayan India.

Diffuse cutaneous leishmaniasis (DCL) is a rare variant of cutaneous leishmaniasis (CL) characterised by multiple (≥10), widespread, slowly progressive, cosmetically disfiguring, non-ulcerating nodules without visceral involvement. The disease is resistant to chemotherapy and characterized by relapses. We present a rare case of a patient with DCL who had extensive lesions (282) and who was HIV negative from a new focus of leishmaniasis in sub-Himalayan India. A favourable response with a regression of the lesions was observed after a month of treatment with intravenous and intralesional sodium stibogluconate with oral pentoxiphyline. Familiarity with such exceptional cases in immunocompetent individuals may facilitate diagnosis and a promising treatment outcome.

Keratitis-Ichthyosis-Deafness syndrome: A rare congenital disorder.

Keratitis-Icthyosis-Deafness syndrome is a rare congenital disorder characterized by keratitis, ichthyosis, and deafness. We report a 13 year old female child who presented with diffuse alopecia of the scalp and body. There was erythrokeratoderma of face and discrete hyperkeratotic hyperpigmented papulo plaque lesions on the body. Patient also had reticulate hyperkeratosis of palms and soles. There was history of recurrent episodes of folliculitis over the scalp and body. There was no evidence of any malignancy. Eye involvement in the form of bilateral vascularising keratitis was present. There was bilateral mixed hearing loss.

Sporotrichosis in sub-himalayan India.

Sporotrichosis is endemic in the Sub-Himalayan belt, which ranges from the northern to the north-eastern Indian subcontinent. Similar to many parts of the developing world, sporotrichosis is commonly recognized clinically in this region however consolidated epidemiological data is lacking. We report epidemiological, clinical and microbiological data from a hundred culture positive cases of sporotrichosis. Out of 305 clinically suspicious cases of sporotrichosis, a total of 100 isolates were identified as Sporothrix schenckii species complex (S. schenckii) on culture. Out of the culture proven cases 71% of the cases presented with lymphocutaneous type of lesions while 28% had fixed localized type and 1% had disseminated sporotrichosis. Presentation with lesions on hands was most frequently seen in 32% with arm (23%) and face (21%) in that sequence. The male to female ratio was 1∶1.27. Age ranged from 1 ½ years to 88 years. Mean age was 43.25 years. Disease was predominantly seen in the fourth to sixth decade of life with 58% cases between 31 and 60 years of age. Since the first report from the region there has been a steady rise in the number of cases of sporotrichosis. Seasonal trends reveal that most of the patients visited for consultation in the beginning of the year between March and April. This is the first study, from the most endemic region of the Sub-Himalayan belt, to delve into epidemiological and clinical details of such a large number of culture proven cases over a period of more than eighteen years which would help in the understanding of the local disease pattern of sporotrichosis.

Evaluation of photopatch test allergens for Indian patients of photodermatitis: preliminary results.

BACKGROUND: There is a strong need to develop a photopatch test tray suitable for Indian patients of photodermatitis as European/Scandinavian photopatch test trays may not be wholly relevant for them. AIM: We carried out this study using photoallergens relevant in the Indian context to determine their relevance in patients of photodermatitis. METHODS: Thirty patients (M:F, 23:7) between 19 and 76 years of age of photodermatitis and 10 controls were patch- and photopatch tested with 20 common photoallergens. In addition, the patients were also (photo) patch tested with articles of daily use as and when these were suspected to be the cause. RESULTS: Forty-three positive reactions to one or more antigens were seen in 22 (74%) patients. Fourteen positive photopatch tests to seven allergens were observed in 10 (33%) patients, and nine (30%) of them had a definite relevance. The most common contact allergen was fragrance mix (FM) (30%), followed by p-phenylenediamine (20%) and Parthenium hysterophorous (17%). The definite relevance of the patch- and photopatch tests could be correlated in 47% of these patients. CONCLUSIONS: FM is the most common contact and photocontact allergen among the various photopatch test antigens. Although differences in technique and evaluation make direct comparison between different centers difficult, still photopatch testing remains an integral part and gold standard for the work-up of the photosensitive patients.

Clinicoepidemiologic features of chronic urticaria in patients having positive versus negative autologous serum skin test: a study of 100 Indian patients.

BACKGROUND: Chronic urticaria patients who demonstrate autoantibodies against the high-affinity receptor of IgE (FceRI) or IgE itself tend to have a high itch and wheal score, and systemic symptoms may have a significant bearing on their management in terms of super pharmacologic doses of antihistamines needed or use of immunomodulators. Most studies have used histamine release assays rather than autologous serum skin tests (ASSTs) for correlating urticaria severity and histamine releasing activity. METHODS: An ASST was performed in 100 (M:F, 31:69) chronic urticaria patients aged between 14 and 63 (mean, 32.69 ± 13) years with an objective to study the clinicoepidemiologic features like age, sex, age of onset and duration, frequency and distribution of wheals, urticaria severity, angioedema and systemic manifestations in ASST-positive and ASST-negative patients. RESULTS: ASST was positive in 46% of the patients and negative in 54% of the patients, respectively. Both groups showed no statistically significant difference for epidemiological details. However, the ASST-positive patients had a higher mean urticaria activity score, frequent involvement of more body sites, particularly palms and soles, presence of throat angioedema and general constitutional, respiratory or gastrointestinal symptoms in comparison with the ASST-negative patients. CONCLUSIONS: Apparently, ASST-positive patients have more severe clinical manifestations of chronic urticaria. The knowledge will be useful for the treating dermatologists and patients alike in view of its therapeutic implications.