Interchanging Autoimmunity - Lupus Mastitis Coexisting with Systemic Polyarteritis Nodosa

Lupus mastitis is an uncommon presentation of lupus erythematosus profundus (LE profundus), characterized by inflammation of the subcutaneous fat. LE profundus occurring as initial manifestation of LE is rare. We report a case where the patient presented with lupus mastitis and years later, she developed disseminated discoid LE (DLE) and polyarteritis nodosa (PAN). PAN and DLE are connective tissue diseases with different etiologies, clinical, immunological, and histopathological features. One connective tissue disease evolving into another is rare, and the reasons remain unexplained. This woman had histopathologically proven DLE and lupus mastitis with a negative antinuclear antibody profile. She satisfied the American College of Rheumatology criteria for PAN.     

Congenital lupus erythematosus

Neonatal lupus erythematosus (NLE) is an autoimmune disease affecting the fetus as a result of transplacental transfer of anti-Ro autoantibodies. Typically, it presents in the first few months of life with an annular form of subacute cutaneous lupus erythematosus. We report an unusual case of NLE presenting at birth with scaly erythematous telangiectatic patches and macules with skin atrophy involving the face, head, and upper trunk. Thrombocytopenia was discovered on laboratory investigations. Histopathology of skin biopsy was consistent with subacute cutaneous lupus. The mother was clinically free of disease and had no family history of autoimmune disease. Serology (extra-nuclear antigens) was positive in both the baby and the mother. This is a rare presentation of a rare disease.     

A case to illustrate the role of ophthalmologist in systemic lupus erythematosus

Systemic lupus erythematosus (SLE) affects the eye as part of the disease or due to the drugs used in therapy. Ocular involvement is seen in one third of the patients with SLE. SLE is rare in India and found less frequently in males and children. SLE retinopathy is usually bilateral. We report an unusual case of unilateral macular infarction in a boy caused by systemic lupus erythematosus. A fourteen year old boy was presented with skin rashes and loss of vision in left eye. Posterior segment examination showed hyperemic edematous disc, arteriolar attenuation, venous dilatation, multiple cotton wool spots around the disc and macula in the left eye. There was no improvement in vision with pulse steroids and cyclophosphamide. The clinical implication of SLE retinopathy is that the disease is severe and warrants systemic immunosuppressive therapy. SLE-induced macular infarction is rare and has poor visual prognosis. As serious ocular complications of SLE can be silent, routine ophthalmological evaluation is warranted in all patients.     

Toxic Epidermal Necrolysis-like Rash of Lupus: A Dermatologist's Dilemma

For most dermatologists, the challenge posed by toxic epidermal necrolysis (TEN) lies not in its diagnosis, but in pulling the patient out of this life-threatening condition. However, when a patient presents with a TEN-like picture in the background of lupus erythematosus (LE), it becomes difficult to decide whether the eruption is drug induced or a manifestation of lupus itself.     

BIOLOGICS: TARGET-SPECIFIC TREATMENT OF SYSTEMIC AND CUTANEOUS AUTOIMMUNE DISEASES

Biologics are becoming important in the treatment of systemic and cutaneous autoimmune diseases. They are designed to target specific components of immune system. As the new drugs are capable of targeting proteins in a more specific fashion, yet have lower risks of systemic side-effects, they have considerable advantages over the older immunomodulators. The development of TNF-alpha blockers in the treatment of psoriasis, psoriatic arthritis, rheumatoid arthritis, Crohn''s disease and ankylosing spondylitis have been major breakthroughs. Likewise, B-cell depletion has proved to be equally revolutionary for the treatment of lupus, pemphigus, certain vasculitides etc. But all said and done, the development of these molecules and their clinical usage are still at evolving stages. Consensus needs be formed to further categorize the clinical profiles of the patients in whom biologics are to be used in the future, given that the long-term safety profiles of these agents are very much unknown at present.     

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.     

Evaluation and Management of Polymyositis

Polymyositis (PM) is one of the inflammatory myopathies, disorders characterized pathologically by the presence of inflammatory infiltrates in striated muscle. The principal clinical manifestation of PM is proximal muscle weakness. The cause of PM is unknown, but current evidence suggests that it is an autoimmune disorder. PM can affect people of any age, but most commonly presents between the ages of 50 to 70. PM is rarely seen in people younger than 18 years of age, and is twice as common among females than males. PM is more common in blacks than in whites. The overall prevalence of PM is 1 per 100,000. Muscle weakness may develop suddenly or more insidiously over a period of weeks to months. The classic symptom of PM is proximal weakness, which may manifest as difficulty holding the arms over the head, climbing stairs, or rising from a chair. Weakness of the striated muscle of the upper esophagus may result in dysphagia, dysphonia, and aspiration. The chest wall muscles may be affected, leading to ventilatory compromises. Involvement of cardiac muscle may lead to arrhythmias and congestive heart failure. Dermatomyositis (DM) is closely related to PM, and both are distinguished primarily by the occurrence of characteristic skin abnormalities in the former. PM and DM may be associated with a variety of malignancies. PM may also occur as part of the spectrum of other rheumatic diseases like systemic lupus erythematosus and mixed connective tissue disease. Moreover, inflammatory myopathy may be caused by some drugs (procainamide, D-penicillamine), and viruses, most notably the retroviruses. Corticosteroids and immunosuppressive agents are the mainstays of therapy for PM. The principal goals of therapy are to improve strength and improve physical functioning. Many patients require treatment for several years. The 5-year survival rate for treated patients is in the order of 95%. Up to one-third of PM patients may be left with some degree of residual muscle weakness.     

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.     

Venereology in India

Venereology-the study of venereal diseases or more recently, the sexually transmitted infections (STI) includes a variety of pathogens namely viruses, bacteria, fungi and protozoa for which the common factor is the mode of transmission and acquisition: Sexual relations between human beings. Medical and other historians have often suggested that well-known diseases such as syphilis, gonorrhea, chancroid and lymphogranuloma venereum have existed since earliest times. However, it is difficult to identify modern disease entities based on written historical record. Studying the origin of STIs helps us to learn the political, economic and moral conditions that led to the disease. Effective management of STI rests on three pillars of diagnosis, prevention and treatment. For most of past 50 years in India, the diagnostic pillar has been the least well-supported. Until well into present century, diagnosis of STI in India was clinical. Treatment of STIs in India followed the methods used in England. Of course in the 19(th) century, in many parts of the world, only a few had access to modern methods of treatment; in India, there was extensive use of Ayurvedic treatment with traditional medicines. This article thus gives just an overview and evolution of venereology in India with regard to venereal diseases (now more often known as STIs/disease), control measures, academic, association and journal development and finally future perspective.     

Metabolic Syndrome and Skin: Psoriasis and Beyond

Metabolic syndrome (Met S) is a clustering of risk factors comprising of abdominal obesity, dyslipidemia, elevated blood pressure, and abnormal glucose tolerance. The prevalence of Met S has been increasing in the last few years throughout the world. Psoriasis has consistently been associated with Met S as well as its various components. However, the association is no longer limited to psoriasis alone. Various dermatological conditions such as lichen planus, androgenetic alopecia, systemic lupus erythematosus, skin tags, acanthosis nigricans, and even cutaneous malignancies have also been found to be associated with this syndrome. Though chronic inflammation is thought to be the bridging link, the role of oxidative stress and endocrine abnormalities has recently been proposed in bringing them together.     

A clinical and histopathological profile of patients with cutaneous tuberculosis

Extrapulmonary tuberculosis constitutes about 10% of all cases of tuberculosis, and cutaneous tuberculosis makes up only a small proportion of these cases. Despite prevention programs, tuberculosis is still progressing endemically in developing countries. Commonest clinical variant of cutaneous tuberculosis in our study was lupus vulgaris seen in 55% patients followed by scrufuloderma seen in 25% patients followed by orificial tuberculosis, tuberculosis verrucosa cutis, papulonecrotic tuberculid, and erythema induratum seen in 5% each. The commonest site of involvement was limbs seen in 50% patients followed by neck seen in 25% patients, face in 15%, and trunk in 10% patients. Maximum percentage of patients (55%) had duration of cutaneous tuberculosis between 6-12 months followed by 35% between 13-24 months, 5% had duration of cutaneous tuberculosis less than 6 months, and the rest 5% had duration more than 24 months. The commonest histopathological feature in our study was tuberculoid granuloma with epitheloid cell and Langhans giant cells seen in 70% patients, hyperkeratosis was seen in 15% patients and AFB bacilli were seen in 5% patients.     

BIOLOGICAL THERAPY OF PSORIASIS

The treatment of psoriasis has undergone a revolution with the advent of biologic therapies, including infliximab, etanercept, adalimumab, efalizumab, and alefacept. These medications are designed to target specific components of the immune system and are a major technological advancement over traditional immunosuppressive medications. These usually being well tolerated are being found useful in a growing number of immune-mediated diseases, psoriasis being just one example. The newest biologic, ustekinumab, is directed against the p40 subunit of the IL-12 and IL-23 cytokines. It has provided a new avenue of therapy for an array of T-cell-mediated diseases. Biologics are generally safe; however, there has been concern over the risk of lymphoma with use of these agents. All anti-TNF-α agents have been associated with a variety of serious and “routine” opportunistic infections.     

Mental health in patients with pemphigus: an issue to worth consideration

Background:

There exists a high prevalence of psychiatric disorders in dermatological patients. Although, investigators have evaluated psychiatric aspects of the patients suffering from skin diseases; there are rare studies concerning mental health in pemphigus patients. The objective of this study was to evaluate mental health status and quality of life of newly diagnosed pemphigus patients.

Materials and Methods:

Between April 2007 and June 2008, all newly diagnosed pemphigus patients attending the outpatient clinic of a dermatological hospital were given a questionnaire comprising the GHQ-28 and DLQI to fill out.

Results:

Of 283 patients, 212 complete forms were returned. The bimodal score of GHQ ranged from 0 to 26 (Mean = 9.4) and the Likert score of GHQ ranged from 6 to 68 (Mean = 31.9). The DLQI score ranged between 0 and 30 (Mean of 13.8). A total of 157 patients (73.7%) were yielded to be possible cases of mental disorder considering GHQ-28 bimodal scores. Significant correlation was detected between the DLQI score and bimodal and Likert scoring of GHQ-28.

Conclusion:

Our study has depicted high prevalence of psychiatric comorbidity in pemphigus patients. It underlines the fact that physicians, who are in-charge of care for these patients, are in an exceptional position to distinguish the psychiatric comorbidity and to take appropriate measures.     

HYPERTROPHIC LUPUS VULGARIS: AN UNUSUAL PRESENTATION

Lupus vulgaris is the most common form of cutaneous tuberculosis occurring in previously sensitized individuals with a high degree of tuberculin sensitivity. Various forms including plaque, ulcerative, hypertrophic, vegetative, papular, and nodular forms have been described. A 30-year-old male patient presented with a very large hypertrophic lupus vulgaris lesion over left side of chest since 22 years. Histopathological examination showed granulomatous infiltration without caseation necrosis. The Mantoux reaction was strongly positive. Hypertrophic lupus vulgaris of such a giant size and that too at an unusual site is extremely rare and hence is being reported.     

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.     

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.     

PATCH TESTING WITH DERMATOPHAGOIDES AND ITS CORRELATION WITH CHRONIC ECZEMA AND ATOPIC DERMATITIS

Background:

Chronic eczema is commonly encountered in the Indian set up. So also is atopic dermatitis. House dust mites (Dermatophagoides) are implicated in various diseases like atopic dermatitis, asthma, and perennial rhinitis. It has also been proven that patch testing with Dermatophagoides pteronyssinus (DP) is important for detection of contact sensitization in chronic dermatitis.

Aims:

To study clinical characteristics of DP mix positive patients with regards to chronic dermatitis and atopic dermatitis.

Methods:

Dermatology outpatients presenting to the department of Skin and STD of Kasturba Medical College (KMC), with clinically diagnosed atopic dermatitis and chronic eczema were chosen for the study. Inclusion and exclusion criteria were well demarked. Eighty six randomly selected patients of dermatitis were subjected to patch testing with standard series and DP mix.

Results:

Of the 86, 50 (58%) showed positive reaction to DP mix. Among these positive patients, chronic dermatitis was seen in 42 (84%) with involvement of exposed parts in 37 (74%). Atopic dermatitis was seen in 19 patients (38%) from DP positive group whereas it was observed in 4 patients (17%) from the other group.

Conclusion:

Dermatophagoides mix positivity was statistically significant in chronic eczema as well as atopic dermatitis. Patch testing is an important tool to detect delayed type allergy to house dust mite.