Systemic Sclerosis: Current Concepts in Pathogenesis and Therapeutic Aspects of Dermatological Manifestations

Systemic sclerosis (SSc) is a chronic, multisystem connective tissue disease with protean clinical manifestations. Recent advances in understanding the pathogenic mechanisms have led to development of target-oriented and vasomodulatory drugs which play a pivotal role in treating various dermatological manifestations. An exhaustive literature search was done using Medline, Embase, and Cochrane library to review the recent concepts regarding pathogenesis and evidence-based treatment of salient dermatological manifestations. The concept of shared genetic risk factors for the development of autoimmune diseases is seen in SSc. It is divided into fibroproliferative and inflammatory groups based on genome-wide molecular profiling. Genetic, infectious, and environmental factors play a key role; vascular injury, fibrosis, and immune activation are the chief pathogenic factors. Vitamin D deficiency has been documented in SSc and correlates with the severity of skin involvement. Skin sclerosis, Raynaud''s phenomenon (RP) with digital vasculopathies, pigmentation, calcinosis, and leg ulcers affect the patient''s quality of life. Immunosuppressives, biologicals, and hematopoietic stem cell transplantation are efficacious in skin sclerosis. Endothelin A receptor antagonists, calcium-channel blockers, angiotensin receptor inhibitors, prostacyclin analogs, and phosphodiesterase type 5 (PDE-5) inhibitors are the mainstay in RP and digital vasculopathies. Pigmentation in SSc has been attributed to melanogenic potential of endothelin-1 (ET-1); the role of ET 1 antagonists and vitamin D analogs needs to be investigated. Sexual dysfunction in both male and female patients has been attributed to vasculopathy and fibrosis, wherein PDE-5 inhibitors are found to be useful. The future concepts of treating SSc may be based on the gene expression signature.     

Eponymous Dermatological Signs in Bullous Dermatoses

Clinical signs are evolved by clinicians through their careful clinical examination. Medical professionals are generally familiar with these signs because of the emphasis given to them by the teaching faculty while they were students. Some of these signs are eponymously named after the clinicians giving credit to their observation. Eponymous signs in vesiculobullous diseases such as Nikolsky sign and Asboe Hansen sign (Bulla spread sign) are well known and were described during the 19th and 20th century, respectively. Cerebriform tongue in pemphigus vegetans was described by Premalatha (1981) three decades ago and is well recognized and cited in several text books and articles in leading journals. All these signs are revisited below with an emphasis on cerebriform tongue in pemphigus vegetans which could eponymously be called as Premalatha sign.     

Primary Systemic Amyloidosis with Unusual Dermatological Manifestations: A Rare Case Report

Amyloidosis is a group of heterogeneous diseases characterized by pathological deposition of proteinaceous substance extracellularly in various tissues. The clinical presentation depends on the site of amyloid deposition, with predominant involvement of mesenchymal elements and cutaneous findings in 30–40% of patients in case of primary systemic amyloidosis. We present a case of idiopathic primary systemic amyloidosis presenting with an unusual finding of nodulo-ulcerative lesion over tongue along with multiple skin-colored nodules, mimicking squamous cell carcinoma of tongue with secondary cutaneous metastasis, as well as lacking the classical presentation of purpura, macroglossia, waxy papules, and plaques.     

Bacteriological Study of Pyodermas in a Tertiary Care Dermatological Center

Background:

Bacterial skin infection especially Pyoderma, commonly caused by Staphylococcus aureus and group A Streptococci, is quite common in Indian dermatology clinics. Despite a number of new antibiotics, the incidence of bacterial resistance is rising.

Aim:

To find out causative organisms and their latest antibiotic susceptibility patterns in pyodermas.

Materials and Methods:

All in-patients admitted in the Dermatology wards in Government Medical College, Amritsar were screened over 18 months and those with erosive skin lesions and/or purulent discharge were included in the study and swabs were sent for culture and sensitivity.

Results:

Majority 49/61 cases (80.33%) comprised of secondary pyodermas while primary pyodermas constituted only 12/61 cases (19.67%). Single organism was isolated in 49 cases (80.33%). More than one type of organism was isolated in 3 cases (4.92%) while none could be isolated from 9 (14.75%) cases. Staphylococcus aureus spp. was the commonest organism isolated in 36 (59.01%) cases and out of these, coagulase positive strains were found to be highly susceptible to amikacin (21cases-100%). Coagulase negative strains were sensitive to amikacin (7 cases-77.7%) and gentamycin (6 cases-66.6%) respectively.

Conclusion:

This study gives an indication of the present pattern of bacteriological profile of pyodermas in a tertiary care hospital in north-west India. In-vitro testing is essential as knowledge of the causative organisms and resistance patterns can help us select appropriate antibiotics without wasting time in using resistant drugs.     

Nail Psoriasis: The Journey So Far

Nail involvement is an extremely common feature of psoriasis and affects approximately 10-78% of psoriasis patients with 5-10% of patients having isolated nail psoriasis. However, it is often an overlooked feature in the management of nail psoriasis, despite the significant burden it places on the patients as a result of functional impairment of manual dexterity, pain, and psychological stress. Affected nail plates often thicken and crumble, and because they are very visible, patients tend to avoid normal day-to-day activities and social interactions. Importantly, 70-80% of patients with psoriatic arthritis have nail psoriasis. In this overview, we review the clinical manifestations of psoriasis affecting the nails, the common differential diagnosis of nail psoriasis, Nail Psoriasis Severity Index and the various diagnostic aids for diagnosing nail psoriasis especially, the cases with isolated nail involvement. We have also discussed the available treatment options, including the topical, physical, systemic, and biological modalities, in great detail in order to equip the present day dermatologist in dealing with a big clinical challenge, that is, management of nail psoriasis.     

Human Papillomavirus Deoxyribonucleic Acid may not be Detected in Non-genital Benign Papillomatous Skin Lesions by Polymerase Chain Reaction

Background:

Papillomatosis is a known histopathologic pattern usually seen in human papillomavirus (HPV) infection and verruca vulgaris is the typical example. This pattern is also detected in some other benign cutaneous lesions such as nevus sebaceous (NS), seborrheic keratosis (SK), trichilemmoma (TL) and inverted follicular keratosis (IFK). The association between papillomatous lesions and HPV infection is questionable.

Objective:

The objective of this study was to investigate the presence of HPV deoxyribonucleic acid (DNA) in non-genital benign papillomatous skin lesions (NS, SK, TL and IFK) by polymerase chain reaction (PCR).

Materials and Methods:

A total of 100 specimens of non-genital NS, SK, TL and IFK were retrieved from archives of Dermatopathology Department of Razi Hospital, between 2003 and 2010. The conventional PCR using consensus GP5+/GP6+ primer and hydroxymethylbilane synthase gene as inner control was performed.

Results:

PCR for HPV DNA revealed no positive results in any of 28 seborrheic keratosis (SK), 28 nevus sebaceous (NS), 28 inverted follicular keratosis (IFK) and 13 trichilemmoma (TL) studied specimens.

Conclusion:

Papillomatosis is usually a characteristic pattern of HPV infection. However, we found no association between HPV infection and non-genital benign papillomatous lesions.     

The concept of stability of vitiligo: a reappraisal

Stability is taken as the most important parameter before opting for any transplantation technique to treat vitiligo. But, simultaneous donor site repigmentation and depigmentation of grafts at the recipient site has been noted. Similarly donor site depigmentation with complete repigmentation of the recipient area with pigment growing out from each graft has been observed. Successful repigmentation after regrafting in previous punch failure cases has also been reported. Koebner's phenomenon from history (Kp-h) and test grafting were the only available indicators to assess stability. It is quite ironic to note that even after four decades of experience in vitiligo surgery, there seems to be little consensus among workers regarding the optimal required period of stability. Moreover, the exact concept of stability in vitiligo is itself still not transparent and defined beyond doubt Overdependence on KpH or TG may be sometimes misleading in vitiligo. These two reveal the apparent clinical stability only and that may not be the true reflection of stability status of the disease at the molecular level. Antimelanocyte cytotoxic reactivity was observed among CD8+ TCC isolated from perilesional biopsies of patients with vitiligo. An attempt should be made to clearly fathom and define stability, not merely only on clinical ground but along with electron microscopy and histoenzymological analysis of the perilesional and nonlesional skin of vitiligo patients. Probably some growth factors which are responsible for both mitogenic and melanogenic stimulation of melanocytes should also be taken into account. Some serological test(s) could guide us to measure these growth factors.     

Moisturizers: The Slippery Road

Moisturizers are an important part of a dermatologist''s armamentarium although little is written and well, a less is truly known about them. There is a cornucopia of projected skin products in the market whose real scientific role is not proven. These products although at times are regarded as mere cosmetics but have a well-known role in many skin disorders. Adequate knowledge about their mechanism of action, dosage, usage, and adverse effects is must for a dermatologist in this era. This article aims to bring forth the ever hidden facts of the much-hyped moisturizers. It is probably the first of its kind covering all aspects of moisturizers ranging from basic science to clinical usage, a subject that receives a short shrift in the current dermatological text.     

Human Immunodeficiency Virus Associated Sporadic Nonfamilial Porphyria Cutanea Tarda

Porphyria cutanea tarda (PCT), a relatively uncommon metabolic disease, is the most common cutaneous porphyria. Here, we present the case of a patient diagnosed with sporadic, nonfamilial PCT that presented with classical cutaneous findings and multiple risk factors, including alcohol abuse, human immunodeficiency virus/AIDS, that have been strongly associated with the sporadic form of PCT.     

No Evidence of Human Papilloma Virus Infection in Basal Cell Carcinoma

Background:

Basal cell carcinoma (BCC) is the most common skin cancer among whites, and several risk factors have been discussed in itsdevelopment and progress. Detection of human papilloma virus (HPV) deoxyribonucleic acid (DNA) BCCs in some studies suggests that the virus may play a role in the pathogenesis of this disease. Several molecular studies showed conflicting reports.

Aims:

The purpose of this study was to investigate the association between HPV and BCC using polymerase chain reaction (PCR).

Materials and Methods:

HPV DNA detection was done for 42 paraffin-embedded tissue specimens of BCC and 42 normal skin samples around the lesions by PCR using GP5+/GP6+ primers.

Results:

HPV DNA was not found in any of the 42 samples of BCC, and only one normal skin sample around the lesions was positive for HPV DNA by PCR.

Conclusion:

In this study, no statistically significant difference was seen between the presence of HPV DNA in BCC and normal skin around the lesion, and HPV is not likely to have an important role in pathogenesis of BCC.     

The Spectrum of Cutaneous Infection in Diabetic Patients with Hepatitis C Virus Infection: A Single-Center Study from Egypt

Context:

Hepatitis-C virus (HCV) infection and diabetes mellitus (DM) have a significant association with skin disorders. Aims: The aim of this study was to assess the impact of HCV infection on the pattern of cutaneous infections among diabetic patients. Methods and Material: A prospective study included diabetic patients who attended Al-Hussein University hospital, Cairo during the period from 2008 to 2010. Patients were examined for skin infections, and investigated for HCV infection.

Statistical Analysis Used:

SPSS (version 11.5).

Results:

The study included 163 patients (102 males and 61 females) with a mean age of 46.2 ± 4.83 years. Ninety five patients (58.3%) were HCV+ve (group A) while 68 patients (41.8%) were HCV-ve (group B). Skin infections in group A included fungal (48.4%), viral (26.3%), bacterial (22.1%) and parasitic (3.2%) while in group B, the spectrum included bacterial (41.2%), fungal (39.7%), viral (11.7%) and parasitic (7.4%). Onychomycosis was the commonest infection in group A (25.2%) compared with folliculitis in group B (19.1%). Cutaneous infections in HCV+ patients were more characterized by increased severity, aggressive course, resistance to treatment and rapid relapse.

Conclusions:

HCV infection has a significant impact in increasing and changing the spectrum of skin infections in diabetic patients. Severe and resistant infections in diabetics could be an important sign of HCV infection.     

The Efficacy of Topical 0.1% Adapalene Gel for Use in the Treatment of Childhood Acanthosis Nigricans: A Pilot Study

Background:

Vitiligo is one of the disorder that has social impact. Both skin and mucous membrane show depigmentation in vitiligo. Depigmentation in oral cavity can be more easily observed and the patient can be given awareness regarding the condition if they are unaware of vitiligo elsewhere in their body and can be guided for treatment.

Aim and objectives:

The aim of this study is to determine the frequency of occurrence of oral mucosal vitiligo in vitiligo patients and to determine the most commonly involved oral mucosal site.

Materials and methods:

The study sample included 100 vitiligo patients. The patients of all age groups and both genders were included. Vitiligo patients associated with systemic conditions such as thyroid disorders, juvenile diabetes mellitus, pernicious anemia, Addison''s disease were excluded in this study.

Results:

Out of 100 vitiligo patients 44 % male and 56% were female. The oral presentation of vitiligo in this study showed depigmentation of buccal mucosa in 5% of patients, labial mucosa in 5% of patients, palate in 8% of patients, gingiva in 2% of patients and alveolar mucosa 1%. Depigmentation of lip was seen in 42% of patients. Lip involvement refers to depigmentation of both the lips or either lip. Also vermilion border involvement was noted in majority of cases. In some cases, the depigmentation of lip extended to the facial skin also.

Conclusion:

In this study 55 patients out of 100 patients showed depigmentation in the oral cavity. Lip involvement was most common in this study showing about 42% of patients. Intraoral mucosal involvement was found in 21% of patients. Among intraoral mucosal site palate was common followed by buccal and labial mucosa, gingiva. Two patients had lip pigmentation as the only manifestation without any depigmentation in the skin.     

The Relationship Between Alopecia Areata and Alexithymia,Anxiety and Depression: A Case-Control Study

Background:

Alopecia areata (AA) is a skin disease characterized by the sudden appearance of areas of hair loss on the scalp and other hair-bearing areas, but its aesthetic repercussions can lead to profound changes in patient''s psychological status and relationships.

Aim:

The goal was to investigate a possible relationship between AA and alexithymia as well as two other emotional dimensions, anxiety and depression.

Materials and Methods:

Fifty patients with AA seen in the Department of Dermatology of Hedi Chaker University Hospital, Sfax were included in this study. Anxiety and depression were evaluated by Hospital Anxiety and Depression scale questionnaire, alexithymia was assessed by Toronto Alexithymia scale 20, and severity of AA was measured by Severity of Alopecia Tool.

Results:

Patient''s mean age was 32.92 years. 52% of patients were females. Depression and anxiety were detected respectively in 38% and 62% of patients. There was statistically significant difference between patients and control group in terms of depression (P = 0.047) and anxiety (P = 0.005). Forty-two percent of patients scored positive for alexithymia. No significant difference was found between patient and control groups (P = 0.683) in terms of alexithymia. Anxiety was responsible for 14.7% of variation in alexithymia (P = 0.047).

Conclusions:

Our study shows a high prevalence of anxiety and depressive symptoms in AA patients. Dermatologists should be aware of the psychological impact of AA, especially as current treatments have limited effectiveness.     

Pigmentary nevi on face have unique patterns and implications: The concept of Blaschko's lines for pigmentary nevi

Background:

Nevi are proposed to reflect the mosaicism and thus generally follow the different archetypal patterns of mosaicism. Blaschko''s lines are the most common pattern of mosaicism. There have been many attempts to elucidate the Blaschko''s lines on face from the distributional patterns of different nevi, but studies that evaluated exclusively pigmentary nevi are sparse. Aims: This study attempted to evaluate the patterns followed by different pigmentary nevi on face and utilized this to elucidate the pattern of embryological pigmentation on face.

Materials and Methods:

Spatial parameters like shape, orientation, and distribution patterns of different flat pigmentary nevi on face were analyzed and graphically drawn on human facial diagram. This was compared with existing facial Blaschko''s lines. All cases of palpable pigmented nevi like congenital and giant melanocytic nevi and nevus spilus were excluded.

Results:

A total of 68 cases of pigmentary nevi on face (male-39, female-29) were examined. The shape and distribution lines were found to have a close similarity with Blaschko''s lines on face with distinct differences.

Conclusions:

The concept of facial embryonic pigmentary ‘segment’, ‘unit’ and existence of separate Blaschko''s lines for facial pigmentary nevi is conceived. Some insight into the pathogenesis of Blaschko''s lines is also proposed in this study.     

The Girl Shedding Glass Pieces from Her Body Parts: Unfolding the Mystery Behind Dermatitis Artefacta

Patients presenting with unusual symptoms are uncommon. Unusual symptoms often catch the attention of the public including the health professionals. Atypicality in the symptoms is usually seen in patients with factitious disorder. In patients with factitious disorder, there occurs conscious production of symptoms with an unconscious motive, irrespective of the fact whether they are ill or not. Self-inflicted injuries to skin often give rise to typical skin lesions known as dermatitis artefacta, which many a times simulate other dermatological conditions. Patients with factitious disorder often have a narrow line of demarcation from malingering. We present a case of an adolescent girl presenting with spontaneous extrusion of glass pieces from her body parts with focus on diagnostic dilemma and management.     

Severe Adverse Events Related to Tattooing: An Retrospective Analysis of 11 Years

Background:

The incidence of tattoos has been increased markedly during the last 20 years.

Aims:

To analyze the patient files for severe adverse medical reactions related to tattooing.

Settings:

Academic Teaching Hospital in South-East Germany.

Materials and Methods:

Retrospective investigation from March 2001 to May 2012.

Results:

The incidence of severe adverse medical reactions has been estimated as 0.02%. Infectious and non-infectious severe reactions have been observed. The consequences were medical drug therapies and surgery.

Conclusions:

Tattooing may be associated with severe adverse medical reactions with significant morbidity. Regulations, education and at least hygienic controls are tools to increase consumer safety.     

Solitary Pilar Leiomyoma of the Nasal Dorsum: Case Report and Literature Review

Cutaneous leiomyoma is an infrequently occurring benign tumor that arises from smooth muscle in the skin. Pilar leiomyoma, a subtype of cutaneous leiomyoma, arises from the arrector pili muscle associated with hair follicles. Pilar leiomyoma, particularly in the head and neck region, is rare and frequently misdiagnosed. We report one patient who developed pilar leiomyoma of the nasal dorsum, explore the differential diagnosis and review the characteristics of previously reported individuals with pilar leiomyoma of the nasal dorsum.     

Chemical leucoderma: Indian scenario, prognosis, and treatment

Chemical leucoderma is an industrial disorder in developed countries and the common causative chemicals are phenols and catechols. Due to stringent controls and preventive measures the incidence has come down. In the recent past various chemicals in consumer products have also been documented to produce depigmentation. In India due to lax quality control measures chemical leucoderma due to consumer items is not uncommon.The various consumer items documented to cause contact depigmentation are sticker bindis, rain shoes, plastic chappals, hair dye/ black henna(kali mehndi), alta, wallets and even mobile plastic covers.