Violaceous Maculopapular Rash in a Newborn: Congenital Rubella Syndrome

Congenital rubella syndrome involves a configuration of systemic and cutaneous manifestations in a neonate due to in utero infection caused by the rubella virus. The case of a preterm neonate with blueberry muffin lesions and classical as well as rare systemic features of congenital rubella syndrome is reported.     

A Tale of Two Tails: Not Just Skin Deep

The dorsal cutaneous appendage or the so called human tail is a rare congenital anomaly protruding from the lumbo-sacro-coccygeal area. These caudal appendages are divided into true-tails and pseudo-tails. We report here two cases of congenital pseudo-tail with underlying spina bifida and lipo-meningocele. In this article we seek to emphasize that, as the skin and nervous systems are intimately linked by their similar ectodermal origin, a dorsal appendage may be regarded as a cutaneous marker of the underlying spinal dysraphism.     

Disseminated cutaneous histoplasmosis in an immunocompetent adult

Histoplasmosis, a systemic mycosis caused by the dimorphic fungus Histoplasma capsulatum var capsulatum and Histoplasma capsulatum var duboisii is endemic to many parts of the world. The clinical manifestations range from acute or chronic pulmonary infection to a progressive disseminated disease. After initial exposure to the fungus, the infection is self-limited and restricted to the lungs in 99% of healthy individuals. The remaining 1%, however, progress to either disseminated or chronic disease involving the lungs, liver, spleen, lymph nodes, bone marrow or rarely, the skin and mucous membranes. Mucocutaneous histoplasmosis is frequently reported in patients with acquired immune deficiency syndrome (AIDS), but it is rare in immunocompetent hosts. A 60-year-old male presented with asymptomatic swelling of the hard palate and crusted papules and nodules over the extremities, face and trunk. Clinically, the diagnoses of cutaneous cryptococcosis versus histoplasmosis was considered in this patient. A chest X-ray revealed hilar lymphadenopathy. Enzyme-linked immunosorbent assay (ELISA) for human immunodeficiency virus (HIV) was nonreactive. Skin biopsy revealed multiple tiny intracellular round yeast forms with a halo in the mid-dermis. Culture of the skin biopsy in Sabouraud's dextrose agar showed colonies of Histoplasma capsulatum. Despite an investigation including no evidence of underlying immunosuppression was found, he was started on IV amphotericin-B (0.5 mg/kg/day). However, the patient succumbed to his disease 2 days after presentation. We report a rare case of disseminated cutaneous histoplasmosis in an immunocompetent individual.     

Comparison of intralesional two percent zinc sulfate and glucantime injection in treatment of acute cutaneous leishmaniasis

Introduction:

Cutaneous leishmaniasis is an endemic disease in developing countries caused by different species of leishmania parasite, and if left untreated, it will result in a deformed scar after a relatively long period. Although various systemic and topical treatments have been proposed for leishmaniasis, pentavalent Antimony compounds remain the first-line treatment for it. Considering the cases with treatment failure, potential side effects and reluctance of patients to receive the drug, there are continuing efforts to find better treatment alternatives.

Aim:

Comparison of the effect of intralesional 2% zinc sulfate injection with Glucantime in treatment of acute cutaneous leishmaniasis.

Materials and Methods:

In this clinical trial, 45 patients with clinical diagnosis of cutaneous leishmaniasis and positive direct smear for leishman body were treated by intralesional injection of either 2% zinc sulfate or Glucantime. After simple randomization, in one group the patients were treated with 2 bouts of intralesional 2% zinc sulfate with a 2-week interval, and in the other group they were treated with 6 weekly bouts of intralesional Glucantime. The patients were monitored in two week intervals for 8 weeks. Healing of the lesions was evaluated clinically and by direct smear, and the data were analyzed using SPSS (11.5) software, t-Student, Mann-Whitney and Analysis of covariance (ANCOVA) statistical tests.

Findings:

In the end of study, 34 patients completed the study, 10 of whom received intralesional Glucantime and 24 of whom received intralesional 2% zinc sulfate. The healing rate after 8 weeks was 80% in the group receiving intralesional Glucantime and 33.3% in the one receiving 2% zinc sulfate (P=0.009).

Conclusion:

Based on the results of this study, intralesional injection of 2% zinc sulfate was less effective in treatment of cutaneous leishmaniasis than intralesional Glucantime.     

Adult Onset of Xanthelasmoid Mastocytosis: Report of a Rare Entity

Xanthelasmoid or pseudoxanthomatous mastocytosis is an extremely rare variant of diffuse cutaneous mastocytosis. Herein, we describe an adult male with cutaneous mastocytosis showing multiple widespread yellowish ovoid papules like eruptive xanthoma. A 60-year-old male visited our outpatient clinic with a 1-year history of generalized yellowish, ovoid, and skin color papular eruption located on the trunk, groin, extremities, with the modest pruritus. Vital signs were stable, and Darier''s sign was negative. No other subjective and objective signs were detected during the examination. No abnormality was detected in his diagnostic laboratory tests. Skin biopsy was taken, and histopathologic examination revealed proliferation of mast cells with ovoid and spindle nuclei with distinct cytoplasm borders around the capillaries, which was compatible with mastocytosis. Antihistamine was prescribed for pruritus control which was successful, but eruptions were persistent, and even 1-year phototherapy was not useful.     

Blastomycosis presenting as solitary nodule: a rare presentation

Blastomycosis is a chronic granulomatous and suppurative mycosis, caused by Blastomyces dermatitidis, which in the great majority of cases presents as a primary pulmonary disease. Primary cutaneous blastomycosis is very rare. We present a 57-year-old female patient with a solitary, slowly progressive nodule over upper lip of 2½ months duration. Initially, differential diagnosis of cutaneous leishmaniasis, pyoderma and deep mycoses were entertained. Slit smear preparation was suspicious of deep mycotic infection which was subsequently confirmed by biopsy and culture.     

Topical Steroid-Damaged Skin

Topical steroids, commonly used for a wide range of skin disorders, are associated with side effects both systemic and cutaneous. This article aims at bringing awareness among practitioners, about the cutaneous side effects of easily available, over the counter, topical steroids. This makes it important for us as dermatologists to weigh the usefulness of topical steroids versus their side effects, and to make an informed decision regarding their use in each individual based on other factors such as age, site involved and type of skin disorder.     

DERMATOLOGICAL MANIFESTATIONS IN HIV-INFECTED PATIENTS AT A TERTIARY CARE HOSPITAL IN A TRIBAL (BASTAR) REGION OF CHHATTISGARH, INDIA

Background:

Cutaneous disorders during HIV infection are numerous and skin is often the first and only organ affected during most of the course of HIV disease. Some Cutaneous disorders reflect the progression of HIV disease; though the relation is still controversial.

Aims:

The objective of this study, conducted at a tertiary care centre in Bastar, Jagdalpur, is to estimate the status of cutaneous manifestation in HIV-infected patients and its relationship with CD4 cell counts.

Methods:

We enrolled 137 HIV positive subjects. Demographic information such as age, gender, weight, height, socioeconomic status, and educational status were recorded. Laboratory parameter (CD4 counts) and treatment regimen were noted. Patients were examined for skin disorders by a dermatologist. Data were analyzed using chi-square test for categorical variables.

Results:

Majority of the patients were from rural area (65.69%) and belonged to a low socioeconomic and educational status. 30.65% of the patients were housewives, 23.35% drivers, and 16.78% labourers. Predominant mode of transmission was heterosexual contact (94.16%). Most common HIV-related dermatological manifestations were seborrheic dermatitis (74.16%), xerosis (52.5%), generalized skin hyperpigmentation 56 (46.67%), onychomycosis 53 (44.16%), pruritic papular eruption 27 (22.5%), oral candidiasis 21 (17.5%), photo dermatitis 21 (17.5%), and scabies 4 (3.33%). Significant correlation with low CD4+ cell counts was found for oral candidiasis (P < 0.0001) and Kaposi''s sarcoma (P = 0.03), while other disorders such as seborrheic dermatitis (P = 0.22), xerosis (P = 0.25), and onychomycosis (P = 0.08) were not statistically significant.

Conclusion:

This study showed high prevalence of dermatological manifestations in HIV-infected subjects, and they occur more frequently with progression of HIV and decline in immune functions. Therefore, early diagnosis and management of skin disorders can improve the quality of life of HIV-infected subjects.     

Pattern of pediatric dermatoses in a tertiary care centre of South West Rajasthan

Background:

The evaluation for skin disorders forms an important component of primary health care practice for all including children. The prevalence of certain skin diseases in children can reflect status of health, hygiene, and personal cleanliness of a community.

Aims:

This study was planned to evaluate the magnitude of skin diseases, pattern of various dermatoses, factors contributing to these dermatoses and concurrent systemic disease among children up to five years of age.

Materials and Methods:

Consecutive 1000 children, aged up to five years, attending the Dermatology OPD of RNT Medical College and MB Government Hospital, Udaipur were the subjects of this study. A detailed general, systemic and cutaneous examination followed by relevant investigations were carried out. The findings were recorded in a proforma for analysis and interpretation of data.

Results:

One thousand twenty seven (1027) diagnoses were made in 1000 children. Etiological analysis revealed that majority (417; 40.60%) of dermatoses belonged to infection and infestation group followed by eczematous (358; 34.86%) and hypersensitivity (105; 10.22%) groups. Of the infection and infestation group, bacterial infection (141; 13.72%) was the most common entity followed by scabies (107; 10.42%), fungal (67; 6.52%), and viral infection (35; 3.40%).

Conclusion:

This study provides a preliminary baseline data for future clinical research. It might also help to assess the changing trends of pediatric dermatoses.     

THE DERMATOSES OF PREGNANCY

The skin changes in pregnancy can be either physiological (hormonal), changes in pre-existing skin diseases or development of new pregnancy specific dermatoses. Pregnancy-specific skin dermatoses include an ill-defined heterogeneous group of pruritic skin eruptions which are seen only in pregnancy. These include atopic eruption of pregnancy, polymorphic eruption of pregnancy, pemphigoid gestationis and intrahepatic cholestasis of pregnancy. Atopic eruption of pregnancy is the most common of these disorders. Most skin eruptions resolve postpartum and require only symptomatic treatment. Antepartum surveillance is recommended for patients with pemphigoid gestationis and intrahepatic cholestasis of pregnancy as they carry fetal risk. This article deals with the classification, clinical features and treatment of the specific dermatoses of pregnancy.     

Decreased effect of glucantime in cutaneous leishmaniasis complicated with secondary bacterial infection

Background:

Glucantime is regarded as the first-line treatment of cutaneous leishmaniasis (CL); however, failure to treatment is a problem in many cases.

Aim:

The aim was to evaluate the therapeutic effect of glucantime in CL complicated with secondary bacterial infection compared to uncomplicated lesions.

Methods:

This experimental study was performed in Skin Diseases and Leishmaniasis Research Center, Isfahan, Iran. A total of 161 patients enrolled in the study had CL confirmed by positive smear of lesions. All the patients were treated with systemic glucantime for 3 weeks and followed for 2 months. Response to treatment was defined as loss of infiltration, reepithelization, and negative smear. Depending on the results of bacterial cultures, the lesions were divided into two groups and the efficacy of glucantime was compared.

Results:

A total of 123 patients (76.4%) were negative, and 38 patients (23.6%) were positive for secondary bacterial infection. In groups with negative bacterial culture response to treatment was 65% (80 patients) and in the other positive group, it was 31.6% (12 patients), with a difference (χ² = 13.77, P < 0.01).

Conclusion:

Therapeutic effect of glucantime showed a decrease in CL lesions with secondary bacterial infection. Therefore, in the cases of unresponsiveness to treatment, the lesions should be evaluated for bacterial infection, before repeating the treatment.     

Dermatological Side Effects of Epidermal Growth Factor Receptor Inhibitors: ‘PRIDE’ Complex

Epidermal growth factor receptor (EGFR) inhibitor therapy has become the standard treatment for non-small cell lung cancer and head neck malignancy. This class of drug comprises EGFR inhibitors (erlotinib and gefitinib) and monoclonal antibody (cetuximab). Use of this class of drugs has been associated frequently with dermatological side effects termed as PRIDE complex–Papulopustules and/or paronychia, Regulatory abnormalities of hair growth, Itching, Dryness due to EGFR inhibitors. We hereby report the cutaneous side effects of EGFR inhibitor therapy in 15 patients of lung and head/neck cancer. The major clinical findings being acneiform eruption and severe xerosis of skin. Management of these dermatological adverse effects rarely requires discontinuation of targeted therapy and can be managed symptomatically.     

CUTANEOUS MANIFESTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS IN A TERTIARY REFERRAL CENTER

Background:

Systemic lupus erythematosus (SLE) is an autoimmune disease with multiorgan involvement. The skin is the second most commonly affected organ. SLE with skin lesions can produce considerable morbidity resulting from painful skin lesions, alopecia, disfigurement, etc. Skin lesions in patients with lupus may be specific (LE specific) or may be non specific (LE non specific). Acute cutaneous LE (Lupus specific) has a strong association with systemic disease and non-specific skin lesions always indicate disease activity for which patients present to rheumatologists and internists. Therefore, a thorough understanding of the cutaneous manifestations of SLE is essential for most efficient management.

Aims:

The aims of this study were to evaluate the patterns and prevalence of skin lesions in patients with SLE and to assess the relationship between skin lesions and other systemic involvement.

Materials and Methods:

At the Department of Rheumatology and Clinical Immunology, IPGME&R in Kolkata, 150 patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatology Association (updated 1982) were examined and followed-up for cutaneous manifestations between January 2002 and January 2007.

Results:

Skin lesions were important clinical features. About 45 patients (30%) presented with skin lesions although all patients had skin lesions during the follow-up period. Skin changes noted were as follows: Lupus specific lesions: malar rash in 120 patients (80%), photosensitive dermatitis in 75 patients (50%), generalized maculopapular rash in 40 patients (26.67%), discoid rash in 30 patients (20%), subacute cutaneous lupus erythematosus (SCLE) in 5 patients (3.34%), lupus profundus in 5 patients (3.34%). The lupus non-specific lesions were non-scarring alopecia in 130 patients (86.67%), oral ulcers in 85 patients (56.67%), vasculitic lesions in 50 patients (33.34%), bullous lesions in 15 patients (10%), Raynaud''s phenomenon in 10 patients (6.67%), pyoderma gangrenosum in 2 patients (1.34%), erythema multiforme in 10 patients (6.67%), and nail fold infarcts in 2 patients (1.34%); however, mucosal discoid lupus, lichenoid discoid lupus, livedo reticularis, sclerodactyly, etc. were not detected. Patients having lupus-specific skin lesions e.g., malar rash were associated with systemic involvement, whereas those having lupus non-specific skin lesions were associated with disease flare.

Conclusions:

Skin lesions in patients with SLE are important disease manifestations and proper understanding is essential for diagnosis and efficient management.     

Exogenous Ochronosis

Exogenous ochronosis (EO) is a cutaneous disorder characterized by blue-black pigmentation resulting as a complication of long-term application of skin-lightening creams containing hydroquinone but may also occur due to topical contact with phenol or resorcinol in dark-skinned individuals. It can also occur following the use of systemic antimalarials such as quinine. EO is clinically and histologically similar to its endogenous counterpart viz., alkaptonuria, which, however, exhibits systemic effects and is an inherited disorder. Dermoscopy and in vivo skin reflectance confocal microscopy are noninvasive in vivo diagnostic tools. It is very difficult to treat EO, a cosmetically disfiguring and troubling disorder with disappointing treatment options.     

Radiation Port Cutaneous Metastases: Reports of Two Patients Whose Recurrent Visceral Cancers Presented as Skin Lesions at the Site of Previous Radiation and Literature Review

Radiation therapy is associated with a variety of complications, including the development of primary skin cancers in the radiated region. However, it is rare for patients with visceral cancers who are treated with radiation therapy to subsequently develop cutaneous metastasis within the radiation port. We describe two patients with internal malignancies who developed cutaneous metastases within their radiation ports following radiotherapy. In addition, we used PubMed to perform an extensive literature review and identify additional reports of cutaneous metastasis within a radiation port. We excluded patients who developed melanoma or primary skin cancers in the radiation port. We also excluded patients with non-solid organ malignancies. Herein, we summarize the characteristics of 23 additional patients who experienced radiation port cutaneous metastases and explore possible mechanisms for the occurrence of radiation port cutaneous metastases.     

Prolonged Varicella-Zoster Virus Reinfection in an Adult After Unrelated Cord Blood Transplantation

Most varicella-zoster virus (VZV) infections after cord blood transplantation (CBT) present as localized herpes zoster. Here, we report a case of VZV reinfection in an adult patient after CBT that appeared clinically to be varicella. A 50-year-old Japanese man underwent CBT for the management of acute lymphoblastic leukemia. Seventeen months later, he developed a small number of vesicles with umbilicated centers. A skin biopsy showed an intraepidermal blister containing degenerated balloon cells. Subsequently, the skin eruption developed over his entire body. The patient was treated with intravenous acyclovir for 5 days, followed by oral valacyclovir for 9 days. It took more than 3 weeks for most of the skin lesions to scab. Serum levels of anti-VZV IgG on days 3 and 33 after the onset of the skin eruption were negative and 260 mIU/ml, respectively. Serum anti-VZV IgM on days 3 and 33 was not detected. Our patient was diagnosed with VZV reinfection.     

Adult T-cell leukemia/lymphoma: a retroviral malady

Adult T-cell leukemia/lymphoma (ATLL) is an aggressive leukemia/lymphoma of mature T-lymphocytes caused by human T-cell lymphotropic virus type 1 (HTLV-1). At a tertiary healthcare center in South India, a 58-year-old female presented with multiple erythematous, crusted, and umbilicated papules over the body along with cervical lymphadenopathy. The skin biopsy was consistent with cutaneous T-cell lymphoma. Although she responded initially to chemotherapy, the disease relapsed after 3 months, and she developed disseminated infiltrated skin lesions, generalized lymphadenopathy, and leukemia. Due to the unusual clinical findings we did HTLV-1 Enzyme-linked immunosorbent assay (ELISA), which turned out to be positive in high titers. Her mother had died at an early age from a hematological malignancy and her daughter was also found to be seropositive. To the best of our knowledge, this is the first case to be reported from India of the chronic type of ATLL associated with mother-to-child transmission of HTLV-1 in two generations. This case also emphasizes that the chronic type of ATLL can occur in nonendemic areas like India and should be suspected in nonresponding cases of mycosis fungoides. It should be kept in mind that the chronic type often presents without hypercalcemia or the characteristic 'flower cells' in the peripheral smear.     

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.     

Immune deposits in cutaneous lesions of Wegener's granulomatosis: predictor of an active disease

A retrospective analysis was conducted of eight cases of Wegener's granulomatosis (WG), who presented with cutaneous lesions. The clinical, immunopathologic and histopathologic features of the cutaneous lesions were reviewed. Antineutrophil cytoplasmic antibody (ANCA) status of the patients was established. When possible, a comparison of immunofluorescence findings of skin biopsies was made with those of renal biopsies taken at the same time. In all except one, systemic and cutaneous disease developed concurrently. On histopathology, leukocytoclastic vasculitis was noted in five patients and features of lupus erythematosus and pyoderma gangrenosum in one case each. Four patients showed immunoglobulin deposits in subepidermal blood vessel walls, while one patient showed granular immune deposits at dermo-epidermal junction only. Immunoglobulin G was the most common immunoreactant detected. C-ANCA/proteinase 3 (PR3)-ANCA was positive in six patients, P-ANCA/myeloperoxidase (MPO)-ANCA in one patient, while one patient did not show ANCA positivity on indirect immunofluorescence. All four renal biopsies showed pauci-immune glomerulonephritis, irrespective of the presence (n=3) or absence (n=1) of immune deposits in the skin biopsy. Skin manifestations are encountered in nearly half of the patients with WG, thus it is important to be familiar with cutaneous histopathologic as well as immunofluorescence findings in WG patients.