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Copyright © 2019 Indian Dermatology Online Journal.Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by pustules and/or papillomatous vegetations, preferentially affecting intertriginous and periorificial areas. It has two subtypes Hallopeau variant and Neumann variant. Cerebriform tongue, a morphology with typical pattern of sulci and gyri over dorsum of the tongue, is a well-known sign in pemphigus vegetans. We are presenting an unusual case of pemphigus vegetans with extensive involvement of mucosae, trunk, extremities including both extensors and flexural areas with verrucous paronychia and fissured tongue. During clinical exam we recognized multiple cutaneous vesicles, erosions and ulcers on mucosal sites. Clinical, histopathological, and direct and indirect immunofluorescence findings were compatible with pemphigus vegetans. Patient had completed her family so treated with intravenous dexamethasone-cyclophosphamide pulse (DCP) regimen with excellent response. Copyright © 2019 Indian Dermatology Online Journal.Necrobiotic xanthogranuloma (NXG) is a rare form of inflammatory granulomatous disease of the skin characterized by the presence of pruritic to painful lesions, generally located in the periorbital area, although trunk and proximal extremities may also be involved. We report a case of a 69-year-old male patient with extensive involvement of the trunk, upper and lower extremities, and an associated gamma monoclonal gammopathy. The skin biopsy was consistent with the diagnosis of NXG. Necrobiosis lipoidica and granuloma annulare were the major differential diagnosis in this case. We highlight the importance of searching for clinical and histological features that may differentiate these entities, which is crucial to select an adequate therapeutic and surveillance strategy. Copyright © 2019 Indian Dermatology Online Journal.Congenital melanocytic nevi are benign proliferations of cutaneous melanocytes that arise as a result of abnormal growth, development, or migration of melanoblasts. Clinically, the giant congenital nevus is greater than 20 cm in size, pigmented and often hairy. The risk of malignant melanoma for the giant nevi is almost 6%. Fifty percent of the melanomas develop by the age of 2 years and, 80% of the melanomas develop by the age of 7 years. Hence, early management is of paramount importance. Herein, we present a case of giant nevi along with a review of the literature in order to bring awareness among clinicians towards this rare albeit significant entity. Copyright © 2020 Indian Dermatology Online Journal.Darier's disease is an uncommon chronic dermatosis of autosomal dominant inheritance with significant psychosocial morbidity and shows unsatisfactory response to several topical and systemic therapies or various resurfacing or surgical procedure. A 24-year-old woman having characteristic asymptomatic and progressive, hyperkeratotic papular lesions involving the scalp, face, upper trunk, and dorsal hands and feet was diagnosed clinicopathologically as Darier's disease. She was treated successfully with oral Vitamin A 25000 IU given twice daily. The response was apparent within 4 weeks and most lesions cleared in 2 months without any adverse effects. Oral Vitamin A provides an effective and safe low-cost therapeutic alternative in Darier's disease, especially when systemic retinoids, the only effective treatment, remain contraindicated or is not tolerated and for maintenance therapy in the long-term. Copyright © 2020 Indian Dermatology Online Journal.Perforating dermatoses are characterized by transepidermal elimination of altered dermal components or foreign particles. Owing to their common clinical presentation as umbilicated papules with a keratotic plug, histopathology and special staining play a very crucial role in the diagnosis. Omilancor nmr Perforating calcific elastosis, (periumbilical perforating pseudoxanthoma elasticum), an uncommon acquired localized cutaneous dermatoses, is characterized by transepidermal elimination of modified elastic fibres. It is usually seen in middle-aged obese multiparous women as well-defined periumbilical hyperpigmented atrophic plaques. We report a case of a 66-year-old female who presented with a mildly pruritic hyperpigmented periumbilical plaque of 2 years duration. Histopathology studies revealed multiple fragmented, thick, short, and curly eosinophilic fibers; along with granular basophilic material in the dermis, which stained positive for calcium and elastin, thus clinching the diagnosis of perforating calcific elastosis. There were no features of hereditary pseudoxanthoma elasticum. We report this case for its rarity. Copyright © 2020 Indian Dermatology Online Journal.Monilethrix is a rare hereditary disorder affecting hair resulting in hair fragility and alopecia. We report three patients of monilethrix who presented with complaints of sparse and brittle hair from early childhood. All three patients had multiple discrete hyperkeratotic papules over the scalp. Dermoscopy revealed beaded appearance of hair with the presence of elliptical nodes and intermittent constrictions on the hair shafts as well as broken hairs, which were confirmed with routine microscopic examination of hair. Dermoscopy helps in easier and faster diagnosis of monilethrix. Copyright © 2020 Indian Dermatology Online Journal.Symmetrical drug-related intertriginous and flexural exanthema (SDRIFE), and fixed drug eruption (FDE) are adverse cutaneous drug reactions. SDRIFE is most commonly associated with the use of beta-lactam antibiotics. There is only one case report describing SDRIFE due to intake of doxycycline in literature. Previously reported case describes the characteristic morphology of well-defined macular erythema over the flexural and intertriginous area. We here in report a 38-year-old male presented with unusual morphology of SDRIFE, and well circumscribed erythematous patches suggestive of FDE on the thigh and back after doxycycline intake. Histopathology was consistent with SDRIFE and FDE respectively. The skin lesions improved with 5 days of 40 mg oral prednisolone. After 6 weeks, drug provocation with doxycycline was done following which patient developed itching and erythema over the older sites. Though there is a single published report of SDRIFE due to doxycycline, our case had additional findings of having pigmented FDE lesions along with flexural lesions of SDRIFE.

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