Namrata Chhabra

New insights in the pathogenesis of type 1 and type 2 lepra reaction

In the current scenario of leprosy elimination, lepra reactions (LRs) remain a major persistent problem. Type 1 LR (T1LR) and type 2 LR (T2LR) are the major causes of nerve damage and permanent disabilities. The immunopathogenesis of LR have recently become an important field of research, since it may provide the relevant targets for the early detection and control of these episodes. Presently, there are no uniformly acceptable laboratory markers for LR. Genetic and serum markers in human host may predict susceptibility to reactions as well as progression of nerve damage in leprosy. Therefore, a deeper understanding of the molecular mechanisms involved in LR may provide a rational strategy for early diagnosis and prevention of the catastrophic consequences of LR.

Leprosy Scenario at a Tertiary Level Hospital in Delhi: A 5-year Retrospective Study.

Background: Leprosy has been officially eliminated from India since December, 2005; still, there are districts and blocks reporting high prevalence indicating ongoing transmission. The present study aimed at determining the current clinical profile of leprosy from a tertiary level hospital in Delhi. Materials and Methods: A retrospective, record-based study was carried out on patients diagnosed and registered in the leprosy clinic of a tertiary level teaching hospital in East district of Delhi (April 2007 to March 2012). Data regarding demographic details, clinical features, treatment started and complications was analyzed. Results: A total of 849 patients were registered over a 5-year period, with M: F ratio of 2.3:1. 9.3% were children (≤14 years). 54.3% patients were immigrants from adjoining states. Multibacillary leprosy was the most common clinical type (86.9%). Borderline tuberculoid leprosy was the most frequent morphologic type, seen in 56.3% followed by borderline-borderline (1.5%), borderline lepromatous (24.9%), lepromatous leprosy (8.1%), pure neuritic (8.1%), histoid and indeterminate leprosy (0.5% each). 37.4% patients presented in reaction (Type I in 30.4% cases and Type II in 7% cases). WHO grade II deformities were diagnosed in 37.9% with claw hand being the most common paralytic deformity (23.3% cases). Conclusion: Our study offers insight into the current status of the disease in an area of otherwise low prevalence. It is seen that despite statistical elimination, multibacillary disease, leprosy reactions and deformities are commonly seen as presenting manifestations, in contrast to national projected trends. Delhis unique demography with a high degree of migrant workers, presenting to our center (near border location) could be a possible contributing factor towards these aberrations. It highlights the need for continuation of targeted leprosy control activities and active case detection.

Behcet's disease in India: A dermatological perspective

BACKGROUND Behcets disease (BD) is a chronic, recurrent, multi-system inflammatory disorder involving mucocutaneous (MC), ocular, intestinal, articular, vascular, urogenital and neurologic systems. BD occurs with a high prevalence in the Mediterranean population. There is scarcity of clinical data on BD from India with only three case series in the last two decades. AIMS To study demographic profile, clinical manifestations and treatment outcome of patients with BD presenting to the dermatologic clinic in a tertiary hospital in north India. METHODS Prospective analysis of all patients diagnosed to have BD between 1997 to 2011. RESULT Twenty nine patients were diagnosed to have BD. The disease had a female preponderance (M:F = 1:3.8) with a mean age of disease onset of 27.4 (range 16-61) years. The prevalence of various MC and systemic manifestations are as follows: oral aphthae (100%), genital aphthae (93.1%), erythema nodosum (62%), papulopustular and acneiform lesions (31%), articular involvement (68.9%), ocular involvement (31%) and gastrointestinal (GI) involvement (3.4%) . Pathergy test positivity was observed in 31%. The treatment comprised of colchicine (16/29 patients), dapsone (7/29), dapsone with pentoxiphylline (3/29), systemic steroid (2/29), systemic steroid with methotrexate (1/29). Colchicine was effective and well tolerated in all patients. CONCLUSION The disease occurs in a much milder form in India and is primarily mucocutaneous and arthritic. A high index of suspicion in patients with MC lesions may result in early diagnosis, management and prevention of complications of BD. We suggest colchicine as an effective and safe therapeutic option for MC and joint involvement.

Favre-Racouchot syndrome

A 75-year-old man presented with a peculiar facial appearance, having a diffuse yellowish hue, atrophic skin, and deep wrinkles and furrows involving the entire face, most prominently the forehead, zygomatic, and malar areas [Figure 1]. Multiple large comedones and numerous 2-6 mm sized yellow-to-brown colored papules and nodules were present, clustered in the periocular region and over the dorsum of a patulous nose. The lesions had been present for at least 15 years with gradual coarsening of facial features. Lateral madarosis of both eyebrows was evident, but leprosy was clinically ruled out based on absence of history suggestive of any sensory or motor impairment and lack of cutaneous lesions suggestive of leprosy, Hypoesthesia, thickening of peripheral nerves, any motor deficit or deformity on examination. He also had depigmentation of lower lips but was not bothered by it and did not seek any treatment for the same. The patient was a farmer by occupation with history of significant daily sun exposure and had never followed any specific sun-protection measures or used sunscreens. He had been a chronic heavy smoker with 50 smoking pack years (25 cigarettes per day for 40 years). The patient was totally unconcerned about the facial disfigurement and wanted to consult regarding itching in his groin region, which on examination revealed tinea cruris. Histopathology from the cheek revealed grossly solar damaged epidermis with abundant grey-blue ribbon-like elastotic fibers in upper- and mid-dermis, agglomerating to form elastic globules at places. The upper dermis shows an increased number of thin-walled capillaries and sparse superficial perivascular lymphohistiocytic infiltrate with thinning of reticular dermis [Figure 2]. We diagnosed him as a case of Favre-Racouchot syndrome (FRS) with tinea cruris. Special stains were not done as the histopathology on hematoxylin and eosin staining was characteristic of this syndrome. He was prescribed a 2-week course of twice daily terbinafine cream that completely cleared the fungal infection when examined on follow-up. He refused any treatment for the facial skin. Figure 1 Deeply wrinkled, furrowed, and atrophic facial skin with multiple comedones, papules, and nodules over the nose, cheeks, and forehead Figure 2 Histopathology from the cheek revealing sparse superficial perivascular lymphohistiocytic infiltrate with abundant grey blue ribbon like elastotic fibers in upper- and middermis, agglomerating to from elastic globules at places. Overlying epidermis is ... FRS, also referred to as senile comedones, solar comedones, and nodular elastosis with cysts and comedones, was originally described in 1932 by Favre and later reviewed in detail by Favre and Racouchot in 1951.[1] Though mostly reported in Caucasian men (with an estimated prevalence of 6% in adults older than 50 years), cases have also been reported in dark-skinned people including Indians, albeit rarely.[2] Cases occurring as early as the second decade of life have also been documented.[3] Males clearly outnumber females, presumably due to more sun-exposure and the effect of smoking. Clinically, this peculiar syndrome is characterized by a diffuse yellowish hue with the presence of large, open, black comedones symmetrically distributed on the temporal and periorbital areas, and deep wrinkles and furrows over a background of actinically damaged and atrophic skin.[3] In severely affected patients, multiple small papules, nodules and cystic lesions develop, especially involving the periocular region and the nose. Uncommonly, the lateral neck, retroauricular areas, earlobes, and forearms may also be involved. The eruption is usually bilaterally symmetrical. The differential diagnosis includes acne comedones, milia, colloid milium, syringomas, trichoepitheliomas, and sebaceous hyperplasia.[3] Conditions closely linked to FRS include cutis rhomboidalis nuchae which it often accompanies and the actinic comedonal plaque, which is considered to be a variant of FRS.[3] Other conditions reported to be associated with FRS include cutaneous myxoma, actinic keratosis, basal and squamous cell carcinoma, trichostasis spinulosa, keratoacanthoma, and eyelid papilloma.[4] Diagnosis is primarily clinical and histopathology is rarely required. However, the histologic features of FRS are very characteristic with presence of dilated pilosebaceous openings, atrophic sebaceous glands and large, round cyst-like spaces lined by a flattened epithelium and filled with layered horny material.[5] Evidence of significant solar elastosis is often present with presence of epidermal atrophy and massive basophilic degeneration of the upper dermis.[3] Vellus hair shafts, copious amounts of bacteria, sebum, and eosinophilic laminated horny material have been demonstrated within the cyst-like spaces, suggesting that they actually represent oblique histologic cuts of closed comedones rather than true infundibular cysts.[3,5] The comedones are colonized with Propionibacterium acnes, Corynebacterium acnes, Staphylococcus albus, and Malassezia yeasts. Inflammation is conspicuously absent. Though the precise pathogenesis of FRS remains unclear, implicated factors include chonic exposure to ultraviolet radiation and heavy smoking, which may act synergistically. Additionally, radiation therapy has been linked to development of FRS in at least three reported cases till now.[3,5] Measures to halt the progression of the disease include meticulous sun-protective measures like avoiding outdoors activities during peak hours of sun-exposure and the use of broad spectrum sunscreens.[4] Cessation of smoking is strongly advised. Isolated use of pharmacologic treatments or surgical techniques has so far resulted in unsatisfactory results. For best therapeutic outcome, one needs to target the solar elastosis, nodulocystic lesions, and the small as well as large comedones that require mechanical removal. Thus, combining medical and surgical treatments seems to be the best approach at present. Owing to their exfoliative and collagen remodeling properties, topical retinoids constitute the mainstay of medical treatment. Their regular use results in expulsion of small comedones and improvement in photodamaged skin. Daily oral Isotretinoin (0.05-0.1 mg/kg/day) used in conjunction with topical tretinoin has also been found effective.[3] Surgical techniques include comedone extraction, curettage, simple or multiple-stage excision, dermabrasion, and laser resurfacing.[4] The most recent addition to the therapeutic armamentarium of FRS is the combination of superpulsed carbon dioxide laser to vaporize the epidermis followed by extraction of cystic and comedonic material with a pair of forceps, with the entire procedure performed without anesthesia. After an initial encouraging outcome observed in two cases of unilateral FRS, Mavilia et al.,[6] reported excellent cosmetic results, rapid recovery, and good safety profile of this combination therapy in 50 patients of FRS with Fitzpatrick skin type III, and have suggested that it may become the treatment of choice for FRS in future. The prognosis of this condition is excellent if the patient is properly counseled and treated.

Severe marking-nut dermatitis.

caused by p-tert-butylphenol formaldehyde resin and colophonium in neoprene thermal sauna shorts. Contact Dermatitis 2010;63:230Y232. 3. Corazza M, Virgili A. Allergic contact dermatitis due to nickel in a neoprene wetsuit. Contact Dermatitis 1998;39:257. 4. Martellotta D, Di Costanzo L, Cafiero M, et al. Contact allergy to p<tert-butylphenol formaldehyde resin and zinc diethyldithiocarbamate in a wet suit. Dermatitis 2008;19:E3YE4. 5. Uter W, Rämsch C, Aberer W, et al. The European baseline series in 10 European Countries, 2005/2006Vresults of the European Surveillance System on Contact Allergies (ESSCA). Contact Dermatitis 2009;61:31Y38.

Pityriasis rosea unilateralis with atypical morphology.

1 Requena L, Requena C. Histopathology of the more common viral skin infections. Actas Dermosifiliogr 2010; 101: 201–216. 2 Jablonska S, Orth G. Cutaneous warts: clinical, histological and virological correlations. Arch Dermatol Res 1995; 287: 616–618. 3 Pérez A, Rütten A, Gold R, et al. Circumscribed palmar or plantar hypokeratosis: a distinctive epidermal malformation of the palms or soles. J Am Acad Dermatol 2002; 47: 21–27. 4 Tezuka T. Two cases of hereditary palmoplantar keratoderma. Dermatologica 1984; 169: 138– 145.

Pleomorphic cutaneous sarcoidosis confined to lesions of vitiligo vulgaris in a patient with type 1 diabetes mellitus.

1. Baer MR, Barcos M, Farrell H, Raza A, Preisler HD. Acute myelogenous leukemia with leukemia cutis. Eighteen cases seen between 1969 and 1986. Cancer 1989;63:2192-200. 2. Cho-Vega JH, Medeiros LJ, Prieto VJ, Vega F. Leukemia cutis. Am J Clin Pathol 2008;129:130-42. 3. Baer MR, Barcos M, Farrell H, Raza A, Preisler HD. Acute myelogenous leukaemia with leukaemia cutis. Eighteen cases seen between 1969 and 1986. Cancer 1989;63:2192-200. 4. Gogălniceanu D, Trandafir V, Trandafir D, Popescu E. Generalized gingival enlargement-early clinic manifestation in acute leukemia. Case report. Rev Med Chir Soc Med Nat Iasi 2010;114:576-9. 5. Misri R, Khopkar U, Kharkar V, Mahajan S. Different faces of leukemia cutis: Presenting as purpura fulminans and lupus like butterfly rash. Indian J Dermatol Venereol Leprol 2010;76:7102. 6. Waller WL, Hughes SR, Boh E. Leukemia cutis masquerading as a chemical burn. J Am Acad Dermatol 2011;64:1003-4. 7. Aldabagh B, Patel RR, Honda K. Leukemia cutis in association With Grover’s disease. Am J Dermatopathol 2011;33:e41-3. 8. Ferreira M, Caetano M, Amorim I, Selores M. Leukemia cutis resembling a flare-up of psoriasis. Dermatol Online J 2006;12:13.

Inflammatory nevus comedonicus with epidermoid cyst.

We present here a case of inflammatory nevus comedonicus (NC) in a young male distributed along the Blaschkos lines only over the right lower limb and associated with epidermoid cysts. The case was unique in terms of isolated involvement of lower limb and the rare association of epidermoid cyst.

A comment on rapid progression of hidradenitis suppurativa in the lower leg of a patient with psoriasis vulgaris.

Sir, We read with interest the article by Tanaka et al. (1). However, we conclude that the patient could have botryomycosis rather than hidradenitis suppurativa. Botryomycosis is characterized by chronic, purulent and granulomatous lesions of the dermis and subdermal tissue. Morphologically, it is characterized by plaques with sinuses, superficial pustules and crusts (2). It is reported that the patient had a plaque with central purulent draining sinuses with a deep-seated nodule, and that the peripheral part had mild scar formation over the limb. Limbs are the most common sites of affliction (2). In contrast, hidradenitis suppurativa classically affects the pilosebaceous-apocrine unit of the axillary, inguinal and mammary region in females (3). Escherichia coli and Staphylococcus epidermidis were confirmed on culture of the discharge in the case under consideration, and these are well documented in the aetiology of botryomycosis (4). Furthermore, the lesion was refractory to antibiotics and responded to surgical resection, and this may also be the case for botryomycosis.