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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.

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.

Arteriovenous malformation (AVM) is an uncommon vascular anomaly usually present in intracranial location, however may be present in other sites of the body, mostly in the head and neck region. The lesion may be present since birth or caused by trauma, but may become obvious during puberty or pregnancy. The diagnosis can be confirmed by selective angiography. Treatment usually includes super selective embolization followed by surgical excision within 48 h. We hereby present a rare case of a grotesquely disfiguring congenital auricular arteriovenous malformation in a 77-year-old elderly man, which has recently been complicated by ulceration, infection and hemorrhage in the past 6 months. The pinna showed a 15 cm × 8 cm pulsatile, erythematous, ulcerative hemorrhagic swelling which on angiography revealed tortuous superficial temporal and occipital arteries supplying the lesion. Complete auricular excision with split skin grafting was carried out. The option of preoperative embolization was not considered due to his renal dysfunction and the high dye load required for embolizing the fast flow arteriovenous malformation.

Hypohidrotic/anhidrotic ectodermal dysplasia is a rare inherited disorder characterized by hypohidrosis/anhidrosis, hypotrichosis, dysodontia and heat intolerance. Most common mode of transmission is X-linked recessive, showing complete expression in males, and only partial manifestations in the female carrier heterozygotes. Features like atrophic rhinitis, nasal and aural myiasis, syndactyly, cleft lip and/or palate, mental retardation and immunodeficiency are uncommonly seen in this syndrome. We hereby report a case of hypohidrotic ectodermal dysplasia with unusual features of atrophic rhinitis and nasal myiasis.

We present a 65-year-old man with Darier disease with pityriasis amiantacea on the scalp, 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.

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.

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.

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.

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.

Cutaneous candidiasis is a common childhood dermatoses but has a varied clinical presentation. We report an uncommon manifestation of candidiasis presenting as multiple erythematous penile erosions in a preschool child. The 4 year old boy presented with painful, reddish ulcers in the glans penis and mucosa of prepuce for 3 days without any history of itching or fever. KOH mount from the lesion revealed numerous budding yeasts and child responded well to antifungal therapy. As such presentation is uncommon, high index of suspicion is required to clinch the diagnosis.

This article takes a critical look at the pros and cons of human papillomavirus (HPV) vaccines. There is enough evidence to suggest that the prophylactic vaccines are efficacious in preventing various benign and malignant conditions (including cervical cancers) caused by HPV. Even though the vaccine is costly, hypothetical analysis has shown that HPV vaccination will be cost effective in the long run. Therapeutic HPV vaccines used to treat established disease are still undergoing evaluation in clinical studies, and results seem to be encouraging. Although several countries have started mandatory vaccination programs with the prophylactic HPV vaccines, conservatives have voiced concerns regarding the moral impact of such vaccination programs.

Rhinosporidiosis is a chronic granulomatous disorder caused by Rhinosporidium seeberi . It frequently involves the nasopharynx and occasionally affects the skin. We report a case of 45-year-old man who had disseminated cutaneous rhinosporidiosis with cutaneous pseudohorn, its base arising from rhinosporidiosis. The case presented with multiple reddish lesions over the nose of six years duration. In the past year, he developed skin lesions over the left arm, forearm, back, and chest. On examination, polymorphic lesions of rhinosporidiosis in form of verrucous plaque, unulcerated tumor, granulomatous growth, and furunculoid lesions were observed. Interestingly, there was a cutaneous horn over the chest which on histopathological examination showed hyperplastic epithelium with numerous globular cysts of varying shape, representing sporangia in different stages of development and transelimination. Computerized tomography scan of the chest showed bilateral opacities suggestive of lung involvement. On the basis of these clinical and histopathological findings, a diagnosis of nasal rhinosporidiosis with cutaneous and systemic dissemination was made.

Sir, Papulonecrotic tuberculid causing penile ulcers is extremely rare.1 Herewith, we report a case of papulonecrotic tuberculide of the penis in a 56-years-old male. A 56-year-old married male patient was referred to our department with multiple asymptomatic non-healing ulcers over the glans penis of one month duration. He was a heterosexual individual and his wife did not have any genital lesions or discharge. The patient denied any history of pre-marital and extra-marital sexual contact. On physical examination, there were multiple, superficial and deep tender ulcers on the glans penis with ragged, irregular margins and floor covered with necrotic yellow slough (Fig. 1). The urethral meatus was hidden by these ulcerative lesions. Rest of the genital examination was normal. There was no inguinal lymphadenopathy. His systemic examination was unremarkable. Fig. 1 Glans penis showing multiple ulcers The hemogram revealed elevated erythrocyte sedimentation rate (50 mm in the first hour). Tuberculin (Mantoux) test was strongly positive (20 mm × 20 mm). Gram stain of the discharge from the ulcers demonstrated pus cells, Gram positive cocci, and Gram negative bacilli and discharge from the ulcers grew Staphylococcus aureus, Escherichia coli, and Enterococcus faecalis. Ziehl Neelsen stain of the pus did not demonstrate any acid-fast bacilli (AFB). Tzanck smear from ulcer was negative for multinucleated giant cells. Urine sediment examination for AFB and urine culture were noncontributory. Radiological and ultrasound evaluation of the genitourinary system was normal. HIV antibodies test and VDRL test were nonreactive. Systemic evaluation for any focus of tuberculosis was unremarkable. Biopsy from the edge of the ulcer (glans penis) revealed ulcerated epidermis. In the deep dermis, by the side of ulceration, there were caseating tuberculous granulomas along with perivascular infiltrate with vessel wall thickening and endothelial cell swelling. Fite stain for AFB was negative. These features were consistent with papulonecrotic tuberculide. AFB culture of biopsy specimen was negative. Repeated courses of antibiotic therapy did not yield desired results; hence, antitubercular therapy was initiated keeping in mind the possibility of papuloneurotic tuberculide of the penis. Four-drug combination therapy of rifampicin, isoniazid, pyrazinamide, and ethambutol was given for initial 2 months followed by combination of rifampicin and isoniazid to complete total 6 months of standard antitubercular therapy. The lesions started responding to therapy in next two weeks and complete healing with residual depressed scars occurred after three months of therapy (Fig. 2). Fig. 2 Healing with residual depressed scars after 3 months of therapy Tuberculosis of the penis is rare, even in third world countries where the prevalence of tuberculosis remains relatively high.1 Till 1999, only 161 cases of penile tuberculosis were reported.2 Understandably, papulonecrotic tuberculide involving the glans penis is even rarer.3,4 Tuberculides are hypersensitivity reactions to Mycobacterium tuberculosis or its products in individuals with good immunity.5 These cases are characterized by positive tuberculin test, evidence of present or past tuberculosis, absence of M. tuberculosis in the skin lesions and response to antitubercular treatment.5 However, a focus of tuberculosis elsewhere in the body may not be demonstrable in majority of the cases with papulonecrotic tuberculide as in our case.2,6 Papulonecrotic tuberculides are characterized by recurrent eruptions of asymptomatic, dusky red papules, which ulcerate and crust, and heal after a few weeks with varioliform scarring.5,7 These occur symmetrically and predominantly on the extensor aspects (legs, knees, elbows, hands and feet) of the extremities. Other areas that may be rarely affected by papulonecrotic tuberculides are the ears, face, buttocks, perniotic areas and penis.5 In Japan, penile tuberculide has been considered a disease entity.4 Thus, it is important to remember tuberculosis as an underlying cause of penile ulcers, more so in countries like India where prevalence of tuberculosis is still high.

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.

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.

Background: Psoriatic nail changes predispose to onychomycosis because it becomes easier for fungi to penetrate an already compromised nail plate. Moreover, some of the psoriatic nail changes closely resemble onychomycosis. Aim: To investigate cases of nail psoriasis for any evidence of onychomycosis. Methods: Seventy-two patients with psoriasis were included in the study. The patients were selected from the psoriasis clinic and dermatology in-patient ward. Direct microscopic examination with 20% KOH and culture were carried out in all patients showing psoriatic nail changes. Histopathological examination with Periodic Acid-Schiff (PAS) stain was done in cases negative by KOH examination and culture. Results: Nail changes were seen in 66.66% (48/72) of psoriasis patients. The most common fingernail changes observed were pitting, onycholysis and subungual hyperkeratosis, and the most common toenail changes were onycholysis and subungual hyperkeratosis. Nail changes were significantly more common in males. The duration of skin lesions of psoriasis and Psoriasis Area Severity Index scores were significantly higher in patients with nail changes. Out of 48 patients with psoriatic nail change, 23 (47.91%) had investigative evidence of onychomycosis. The fungal isolates on culture were non-dermatophytic molds in nine patients (18.75%) and yeast like fungi also in nine patients (18.75%). Conclusion: Coexistent onychomycosis in psoriatic nails does occur.

Eccrine spiradenoma (ES) very rarely presents in a linear or zosteriform distribution. It may be associated with foci of various other appendageal tumors. We report a 14-year-old boy who presented to us with multiple nodules in a linear distribution in the posterior aspect of the right lower limb since 2 years of age. The lesions became significantly painful for the past 2 years. Histopathology revealed dermal lobules of ES with smaller foci of chondroid syringoma.

Management of genital herpes is complex. Apart from using the standard antivirals, an ideal management protocol also needs to address various aspects of the disease, including the psychological morbidity. Oral acyclovir, valacyclovir or famciclovir are recommended for routine use. Long-term suppressive therapy is effective in reducing the number of recurrences and the risk of transmission to others. Severe or disseminated disease may require intravenous therapy. Resistant cases are managed with foscarnet or cidofovir. Genital herpes in human immunodeficiency virus-infected individuals usually needs a longer duration of antiviral therapy along with continuation of highly active anti retroviral therapy (HAART). Genital herpes in late pregnancy increases the risk of neonatal herpes. Antiviral therapy and/or cesarean delivery are indicated depending on the clinical circumstance. Acyclovir appears to be safe in pregnancy. But, there is limited data regarding the use of valacyclovir and famciclovir in pregnancy. Neonatal herpes requires a higher dose of acyclovir given intravenously for a longer duration. Management of the sex partner, counseling and prevention advice are equally important in appropriate management of genital herpes. Vaccines till date have been marginally effective. Helicase-primase inhibitors, needle-free mucosal vaccine and a new microbicide product named VivaGel may become promising treatment options in the future.

Fixed drug eruption (FDE) due to fluconazole is extremely rare. We recently had a 35-year-old female patient who presented with multifocal bullous FDE due to fluconazole hitherto not reported in the literature. A 35-year-old female, a known case of carcinoma stomach, operated and initiated on post-operative chemotherapy with cisplatin and adriamycin, presented with multiple itchy hyperpigmented elevated oval patches over the face, neck, trunk, upper limbs and lower limbs of three days duration following some medications for sore throat problem (details of the medication were not available at that time). On physical examination, she had multiple (200 in number) asymmetrically distributed, rounded to oval, hyperpigmented, edematous lesions of variable sizes surrounded by a rim of erythema over the body. Many lesions were bullous. She had a similar episode six months ago but at that time had developed only three lesions on the face and neck following oral medication for mouth problem. A diagnosis of multifocal bullous FDE was made. The skin lesions subsided over next one week leaving behind hyperpigmented macules without any intervention. Patch testing with cisplatin and adriamycin was negative. Oral rechallenge test to paracetamol and diclofenac sodium was negative. Subsequently, she was diagnosed to have oral candidiasis in our hospital and was prescribed oral fluconazole 200 mg on day one followed by 50 mg daily for two weeks. Within half an hour of intake of first dose, she developed itching in the skin lesions and in a couple of hours, developed reactivation of FDE lesions (Fig. 1). She was managed with systemic corticosteroids to control the reaction. Thus, a diagnosis of multifocal bullous FDE due to fluconazole was established. Fig. 1 Multifocal fixed drug eruption due to fluconazole Fluconazole is a triazole antifungal. It is fungisatic and is effective against many yeasts and dermatophytes.1 It acts by inhibiting 14 α-demethylase, a microsomal cytochrome P450 enzyme, in the fungal cell membrane.1 Despite it's widespread use, side-effects due to fluconazole are rarely reported. Side-effects are common when used in high doses (400 mg/day for months), especially in HIV individuals.2 Reported side-effects are nausea, vomiting, abdominal pain, diarrhea, elevated liver enzymes, mild to fatal hepatic necrosis, thrombocytopenia, agranulocytosis, leucopenia, eosinophilia, headache, dizziness, seizures, paresthesia, anaphylaxis, acute adrenal insufficiency, hypokalemia, teratogenicity, menstrual irregularities, and various cutaneous side effects like pruritus, exanthems, erythema multiforme, Stevens-Johnson syndrome, toxic epidermal necrolysis, exfoliative dermatitis, fixed drug eruption, angioedema, oral ulcers, purpura, and reversible alopecia.2 Most of the cutaneous side-effects due to fluconazole exist in isolated or a limited number of case reports. Fixed drug eruption due to fluconazole is extremely rare. To the best of our knowledge, there are only eight earlier reports of fixed drug eruption due to fluconazole in the literature.3–5 FDE is characterized by sudden onset of round and/or oval edematous, dusky-red, macules/plaques on the skin and/or mucosa arising precisely over the area of an earlier reaction due to the same drug.6 However, with subsequent exposure to the culprit drug, a few to numerous new lesions may appear and sometimes may become generalized. Acute lesions usually develop 30 min to eight hours after re-administration of the incriminating drug.6 After the initial acute phase lasting days to weeks, residual grayish or slate-colored hyperpigmentation develops.6 Various morphological types of lesions that may occur are morbiliform, scarlatiniform, erythema multiforme-like, Stevens-Johnson syndrome-like, eczematous, urticarial, nodular, vesicular, bullous (may become toxic epidermal necrolysis-like in severe cases), non-pigmenting and diffuse hypomelanosis.6 Confirmation of diagnosis requires re-challenge by topical provocation (in the form of patch test) and/or oral provocation test, of which oral provocation test is considered superior but unsafe.6 Our literature search on FDE due to fluconazole revealed that all cases reported so far had limited cutaneous or mucosal lesions. In contrast, our patient had extensive generalized lesions with many bullous lesions, a unique presentation of fixed drug eruption due to fluconazole which has not been described in the literature so far. With widespread use of any drug, new adverse effects come to light and hitherto unknown adverse effect may become more common occurrence. We recommend that fluconazole be included in the list of drugs causing multifocal bullous FDE so that similar cases may be diagnosed more often.