Hemorrhagic Skin Nodules and Plaques: A Diagnostic Clue to Underlying Primary Plasma Cell Leukemia.

Plasma cell leukemia (PCL) is a rare lymphoproliferative disorder characterized by a malignant proliferation of plasma cells (PC) in blood and marrow. Cutaneous involvement is very rare in PCL. We present the case of a 45-year-old lady who presented with multiple hemorrhagic nodules and plaques in the skin. Her total leucocyte count was 2,00,200/cmm with 85% abnormal plasmacytoid cells in peripheral smear. Biopsy of the skin lesions revealed diffuse infiltration by plasma cells with 'choked' blood vessels. A diagnosis of plasma cell leukemia with cutaneous involvement was made. On the second day of admission, the patient expired probably because of intracranial bleed due to thrombocytopenia. Post-mortem bone marrow and liver biopsy also showed diffuse infiltration by plasma cells. Monoclonality of the cells was proven by demonstrating the production of only kappa light chains.

Metabolic syndrome in patients with psoriasis: A comparative study.

BACKGROUND: Psoriasis patients are at increased risk of developing the metabolic syndrome (MS). Proinflammatory cytokines such as tumor necrosis factor-α, interleukin-6 that are increased in the psoriatic plaques are known to contribute to features of MS such as hypertension, dyslipidemia and insulin resistance. AIMS: (1) To establish the frequency of MS in patients with psoriasis. (2) To study the risk factors associated with MS in psoriasis. MATERIALS AND METHODS: A hospital based comparative study was conducted involving 40 adult patients with psoriasis and 40 age- and sex-matched controls. All participants were evaluated for components of MS. RESULTS: Both groups included 31 males and 9 females. The mean age of the cases and controls were 49.95 years and 49.35 years, respectively. Psoriasis patients with MS had a statistically significant higher mean age (56.31 ± 11.36 years) compared with those without MS (46.89 ± 11.51 years). MS was present in 13 out of 40 (32.5%) patients with psoriasis and 12 out of 40 (30%) controls; this difference was not statistically significant. Higher age and female gender correlated with the presence of MS in psoriasis patients. The presence of MS in psoriasis patients was statistically independent of psoriasis area severity index score, body surface area involvement or psoriatic arthropathy. CONCLUSION: Our results suggest that there is no close correlation between psoriasis and MS in South Indian patients.

Extensive Darier's disease with pityriasis amiantacea, alopecia and congenital facial nerve palsy.

We present a 65-year-old man with Darier disease with pityriasis amiantacea on the scalp, alopecia, and congenital facial nerve palsy.

Congenital insensitivity to pain and anhidrosis: a case report from South India.

Congenital insensitivity to pain with anhidrosis, also known as hereditary sensory and autonomic neuropathy type IV, is an autosomal recessive disorder characterized by the congenital lack of pain sensation, inability to sweat, episodes of recurrent hyperpyrexia, mental retardation, and self-mutilating behavior. It is an extremely rare disorder with only a handful of reports from India. A five- year- old boy, born to second-degree consanguineous parents after uneventful antenatal period, presented to us with history of recurrent unexplained fever, recurrent ulcers in the lower limbs, insensitivity to painful stimuli (like injections, vaccination) and self-mutilating behavior from early childhood. Cutaneous examination showed multiple ulcers, loss of teeth, loss of tip of the tongue (due to biting), scarring of finger tips, xerosis and lichenification. Sensory examination showed complete loss of pain and temperature sensations, but fine touch and vibration were preserved. Deep tendon reflexes were normal. Evaluation for Hansen's disease was non-contributory. An intradermal injection of histamine did not show any flare response. Based on clinical as well as compatible histological features a diagnosis of congenital insensitivity to pain with anhidrosis was made. The ulcers were treated with appropriate antibiotics and daily dressings. The parents were counseled about appropriate care of the child.

Mal de meleda with lip involvement: a report of two cases.

Mal de Meleda is a rare autosomal recessive transgradient palmoplantar keratoderma characterized by transgradient keratoderma with associated scleroatrophy, nail changes, pseudoainhum around digits and perioral erythema, without a tendency for spontaneous resolution. Involvement of the lip by keratoderma has not been reported in the English literature. Here we present two cases of Mal de Meleda with unusual lip involvement. The first case was a 15-year-old girl, born of second-degree consanguineous marriage, who presented with transgradient palmoplantar keratoderma from 6 months of age, with lichenoid papules and plaques on the elbows and knees, conical tapering of the distal digits, flexion deformity of several fingers, digital constriction, knuckle pads and lip involvement. The second case was a 24-year-old male with transgradient palmoplantar keratoderma since birth. He also had scaly plaques on the extensors of bilateral knees and elbows, knuckle pads, pseudosclerodermatous fingers with conical tapering, digital constrictions at various places with mild flexion deformity and lip involvement. Both patients were otherwise normal without any family history.

Giant seborrheic keratosis of the genitalia.

Genital seborrheic keratosis (SK) is a rare entity, which can be easily misdiagnosed as genital warts. Dermoscopy is a useful tool to make diagnosis of SK in such cases. We report a 50-year-old woman with a large polypoidal growth on the external genitalia. Dermoscopic examination showed fissures and ridges, cerebriform appearance, and comedo-like openings consistent with SK. The histopathology confirmed the diagnosis of SK.

Multiple facial cylindromas: a case report.

Cylindroma is a benign skin appendageal tumor arising from pluripotent stem cells in the folliculo-sebaceous-apocrine unit. Multiple cylindromas are usually seen as a component of Brooke-Spiegler syndrome (BSS) or as the only skin lesions of familial cylindromatosis (FC). The usual site of occurrence of such tumors is the scalp. We report a case of multiple cylindromas involving the face without any other feature of BSS and no family history supporting the possibility of FC. Multiple cylindromas of 7 years duration, confirmed by histopathological examination of multiple biopsies, were seen on the face of a 70-year-old woman. There was no history of similar lesions in any of her family members. Examination of the scalp revealed no lesions. Surgical excision of the larger lesions was performed to improve the facial appearance of the patient. This case is being reported for the unusual occurrence of multiple cylindromas only on the face without any features of BSS or FC.

Congenital onychogryphosis: Leaning Tower nail.

A 45-year-old man presented with a thickened and raised nail of his left fifth finger since birth. He was otherwise healthy. On examination, the nail of the left little finger was markedly thickened, hyperkeratotic, and situated at an angle of approximately 45 degrees to the long axis of the distal phalanx. There was prominent subungual hyperkeratosis. A diagnosis of congenital onychogryphosis of the little finger of idiopathic nature was considered. Visual analogy to the leaning tower of Pisa encouraged us to describe it as congenital leaning tower nail.

Hypopigmentary disorders in children in South India.

BACKGROUND: Hypopigmentary disorders are common group of dermatoses in pediatric age group. AIM: To study the frequency and patterns of hypopigmentary disorders in children. MATERIALS AND METHODS: This study was a descriptive clinical study spanning over a period of 23 months. A total of 113 children (61 boys and 52 girls) were included in this study. RESULTS: The frequency of hypopigmentary disorders among children was 3.28 per 1000 children attending the dermatology out patient department. The mean age of the children was 7.2 years. The mean of age of onset was 7.36 years. Most common hypopigmentary disorder in our study was pityriasis alba (24.7%), followed by vitiligo (20.4%), leprosy (11.5%), nevus depigmentosus (10.18%), and tinea versicolor (6.2%). Others were hypomelanosis of Ito (5), post-inflammatory hypopigmentation (5), pityriasis rosea (4), steroid-induced hypopigmentation (4), lichen sclerosus et atrophicus (3), pityriasis lichenoides chronica (3), lichen striatus (2), oculocutaneous albinism (2), tuberous sclerosis complex (2), pigmentary mosaicism (1), and Griscelli syndrome (1). CONCLUSION: Pityriasis alba, vitiligo, leprosy, nevus depigmentosus and tinea versicolor are the five most common hypopigmentary disorders in children.

Utility of dermoscopy in alopecia areata.

BACKGROUND: Alopecia areata (AA) shows several well-defined dermoscopic features which may help in confirming diagnosis in AA. AIMS: We carried out a study to examine the dermoscopic features of AA and develop a protocol for diagnosis of AA by dermoscopy. MATERIALS AND METHODS: Dermoscopy was performed in 66 patients with AA. Hanse HVS-500NP dermoscope (magnification of ×32 and ×140) was used. RESULTS: The mean age of the patients (46 males and 20 females) was 26.85 years. The mean age of onset was 25.15 years. The mean duration of alopecia was 10.3 months. Most common AA in our study was patchy type (57/66, 87.7%). Single patch was seen in 24 patients and multiple patches in 33 patients. Diffuse AA was seen in five patients. Ophiasis and alopecia universalis were seen in two patients each. Nail changes were fine pitting (4), ridging (2), thinning of nail plate (2). Twenty nail dystrophy, distal onycholysis, striate leukonychia and coarse pitting were seen in one patient each. Intralesional triamcinolone acetonide was the most common therapy offered. Others were oral betamethasone minipulse therapy, dexamethasone pulse, minoxidil, anthralin and corticosteroids. The most common dermoscopic finding was yellow dots seen in 54 patients (81.8%), followed by black dots (44 patients, 66.6%), broken hairs (36 patients, 55.4%), short vellus hair (27 patients, 40.9%) and tapering hairs (8 patients, 12.1%). CONCLUSIONS: The most common dermoscopic finding of AA in our study was yellow dots, followed by black dots, broken hairs, short vellus hair and tapering hairs. Dermoscopic findings were not affected by the type of AA or the severity of the disease.

Spontaneous regression of a congenital melanocytic nevus.

Congenital melanocytic nevus (CMN) may rarely regress which may also be associated with a halo or vitiligo. We describe a 10-year-old girl who presented with CMN on the left leg since birth, which recently started to regress spontaneously with associated depigmentation in the lesion and at a distant site. Dermoscopy performed at different sites of the regressing lesion demonstrated loss of epidermal pigments first followed by loss of dermal pigments. Histopathology and Masson-Fontana stain demonstrated lymphocytic infiltration and loss of pigment production in the regressing area. Immunohistochemistry staining (S100 and HMB-45), however, showed that nevus cells were present in the regressing areas.

A case series of kaposi's varicelliform eruption in dermatology in-patients in a tertiary care centre.

BACKGROUND: Recently, we noted increasing number of cases of Kaposi's varicelliform eruption (KVE) among dermatology in-patients who were being treated for various dermatoses, some of which have not been reported earlier to be associated with KVE, and hence, this report. AIMS: This study was designed to identify various dermatoses in which KVE occurred, to study the clinical features, course and response to specific antiviral treatment, to establish the risk factors, and course of the primary dermatoses during the episode of KVE. MATERIALS AND METHODS: We analyzed our data of dermatology in-patients in a tertiary care centre in South India from April 2008 to November 2009 (20 months). The data were tabulated and analyzed. RESULTS: Twenty cases (12 female and 8 male patients) of KVE were seen. The mean age of the patients was 46.4 years. There were seven cases of erythroderma, four of pemphigus vulgaris, three of toxic epidermal necrolysis, two of airborne contact dermatitis (ABCD), one each of lichenoid drug rash, and drug rash with eosinophilia and systemic symptoms (DRESS). Underlying dermatoses for erythroderma were: ABCD (3), psoriasis (3), and cutaneous T-cell lymphoma (1). Possible source of infection could be identified in five cases: exogenous sources in four cases and endogenous source in one case. The mean incubation period for cases with known source was 5 days (range, 2-9 days). Eighteen patients responded favorably to acyclovir. None of our patients had recurrent KVE during the study period. CONCLUSION: KVE may complicate any dermatosis where the integrity of the skin is compromised. Diagnosis and early treatment are important and possible in most cases if suspected.

Dyschromatosis universalis hereditaria: a case report.

Dyschromatosis universalis hereditaria (DUH) is usually an autosomal dominantly inherited disorder characterized by the presence of hypopigmented as well as hyperpigmented macules. We report an Indian patient with DUH with widespread involvement of skin including the face, oral mucous membranes (including tongue), and palms and soles. Our patient also exhibited nail changes, involvement of scalp hair, and keratosis pilaris.

Lichen simplex chronicus of anogenital region: a clinico-etiological study.

BACKGROUND: Lichen simplex chronicus (LSC) of the anogenital region, is a benign, extremely uncomfortable disease. AIMS: As very little is known about the cause of anogenital LSC (AGLSC), we undertook this study to determine various clinico-etiological factors involved in it and to assess the frequency of AGLSC. METHODS: This was a descriptive study, including 105 patients with AGLSC, who attended the Dermatology clinic in our institute from September 2007 to June 2009. Detailed history, physical examination, and relevant investigations were done. The collected data were tabulated and analyzed. RESULTS: The frequency of AGLSC among patients presenting with anogenital pruritus was 2.54%. Primary AGLSC was more common than secondary AGLSC (69.5% vs. 30.5%). AGLSC manifested more commonly in males than in females (56.2% vs. 43.8%). The mean duration of the disease was 30.6 months. The common triggering factors for itching were sweating (41.9%), rubbing of thighs while walking for long distances (9.5%), and mental stress (5.7%). Pruritus of AGLSC was related to the intake of various food items in 37.1% of patients. In males, scrotum was the predominant site involved (89.8%), whereas in females, labia majora was the predominant site involved (78.2%). Nearly one-third of cases (30.5%) of AGLSC had some other dermatoses in the anogenital region. CONCLUSIONS: Though psychological factors are thought to play an important role in disease causation and perpetuation among AGLSC patients, their significance could not be ascertained by us.