Use of rituximab in pemphigus patients with chronic viral hepatitis: Report of three cases.

Use of rituximab in patients with chronic viral hepatitis can worsen pre-existing hepatitis or reactivate occult infection. There are no reports of use of rituximab in pemphigus patients with co-existing viral hepatitis. Herein, we report three pemphigus patients with co-existing chronic viral hepatitis (hepatitis C (n = 2), hepatitis B (n = 1)), who were treated successfully with rituximab under close supervision and concurrent antiviral drug administration. There was no derangement of the liver function tests or increase in viral load in any of the patients. By incorporating good collaboration with a hepatologist and close follow-up, such patients can be managed successfully with biologic therapies when the conventional treatment modalities have failed.

IgG/IgA pemphigus reactive with desmoglein 1 with additional undetermined reactivity with epidermal basement membrane zone.

IgG/IgA pemphigus is an extremely rare subset of pemphigus, showing anti-keratinocyte cell surface antibodies of both IgG and IgA classes. Herein, we describe a unique case of IgG/IgA pemphigus with clinical features of edematous erythema and peripheral vesiculopustules. Histopathology showed the presence of subcorneal pustules and acantholytic blisters in the mid-epidermis with neutrophilic infiltration and eosinophilic spongiosis. Direct immunofluorescence of perilesional skin showed both IgG and IgA deposits to keratinocyte cell surfaces and unusual granular deposits of IgG, IgM, and C3 along basement membrane zone. On enzyme linked immunosorbent assay , the auto-antibodies were found to be reactive to desmoglein 1 antigen. Various clinical, histopathological, and immunological findings in our case overlapped with the features of IgA pemphigus, pemphigus herpetiformis, and pemphigus foliaceus. These findings indicate that IgG/IgA pemphigus may be a transitional form between IgA pemphigus and pemphigus herpetiformis, and thus provides insight into the pathogenicity of this rare disorder.

Anagen effluvium.

Disturbances of hair follicle cycling lie at the heart of most hair growth disorders, and have dramatic effects on visible hair growth and shedding. The two common disorders due to aberration in hair follicle cycling are telogen and anagen effluvium. Though a lot of literature addresses the problem of telogen effluvium, there are not many reviews on anagen effluvium or anagen hair loss. Anagen effluvium is considered synonymous with chemotherapy-induced alopecia and other causes are rarely considered. In this review, we try to discuss the etiopathogenesis, clinical presentation, differentials, and management issues in anagen effluvium. Anagen effluvium is the abrupt loss of hairs that are in their growing phase (anagen) due to an event that impairs the mitotic or metabolic activity of hair follicle. Chemotherapy, radiation and toxic chemicals, and sometimes inflammatory diseases like alopecia areata and pemphigus are also capable of diminishing the metabolic activity of hair follicles resulting in anagen hair loss. Although it is reversible, and hair regrowth occurs after a delay of 1-3 months; sometimes it can lead to permanent alopecia and can be psychologically devastating with negative impact on individual perceptions of appearance, body image, sexuality, and self-esteem. For some patients, the emotional trauma may be so severe that it may lead to discontinuing or refusing therapy that might otherwise be beneficial. In such cases, a psychosomatic approach as well as empathic consideration of the patients concerns and fears as well as the provision of practical medical-aesthetic and styling tips are equally important and can be integrated in management.

Acantholysis revisited: Back to basics.

Acantholysis means loss of coherence between epidermal cells due to the breakdown of intercellular bridges. It is an important pathogenetic mechanism underlying various bullous disorders, particularly the pemphigus group, as well as many non-blistering disorders. Although a well-known concept, the student often has to refer to many sources to comprehend acantholysis completely. Thorough knowledge of this topic helps in clinching many diagnoses. The etiopathogenesis, classification, clinical signs, and laboratory demonstration of acantholysis are discussed in detail to help students build clear concepts. We have focused on various distinguishing points in different disorders for an easy grasp of the topic.

Assessment of the therapeutic benefit of dexamethasone cyclophosphamide pulse versus only oral cyclophosphamide in phase II of the dexamethasone cyclophosphamide pulse therapy: A preliminary prospective randomized controlled study.

Background: Dexamethasone cyclophosphamide pulse (DCP) therapy is an established mode of treatment for pemphigus in India. Aims: To assess the therapeutic benefit of additional DCPs (phase II, consolidation phase) versus immediate oral cyclophosphamide, usually used in phase III (maintenance phase), after initial DCP therapy (phase I) and to assess which laboratory test (DIF or ELISA) will reflect the clinical relapse best. Methods: Nineteen newly recruited patients of pemphigus vulgaris (PV) received monthly DCPs in phase I and were then randomized into two groups. Group A (10 patients) received monthly DCPs for nine months and Group B (nine patients) received only oral cyclophosphamide for nine months. Direct immunofluorescence (DIF) and enzyme-linked immunosorbent assay (ELISA) were tested before starting DCP regimen, and at 0,3,6,9 months after randomization. Results: Clinical relapse by the end of follow-up period occurred in only one patient in each group. In these cases, DIF became (again) positive before the relapse. No statistically significant difference between the two groups was found at three, six and nine months by ELISA indices and DIF grading. Conclusion: Although the DCP regimen is the standard therapy for pemphigus in India, we found no difference in the clinical outcome between patients receiving nine DCPs in phase II and patients shifted directly from phase I to III. Periodic testing using DIF and Dsg ELISA were found to be useful to monitor disease activity and predict a relapse. Further large scale studies are required to assess if patients can be shifted directly from phase I to III and maintained only on oral cyclophosphamide.

Childhood pemphigus vulgaris successfully treated with rituximab.

Pemphigus is a potentially fatal autoimmune epidermal bullous disorder. Rituximab is a novel therapy for the treatment of refractory pemphigus. However, there is limited clinical data on safety and efficacy of rituximab in pediatric age group. Herein, we report an 11-year-old boy of childhood pemphigus vulgaris who failed to respond to dexamethasone pulse therapy and was subsequently treated with rituximab and achieved complete remission.

Pemphigus in India.

Pemphigus is a chronic epidermal immunobullous disease with potentially fatal outcome. The journey of literature on pemphigus in India has come a long way in last five decades. Pemphigus in Indian patients has unique genetic, clinical, and epidemiological differences from those in the Western countries. Corticosteroids remain the mainstay of treatment for pemphigus. Dexamethasone-cyclophosphamide pulse therapy has revolutionized the management of pemphigus in India and abroad for nearly 3 decades now. Corticosteroid-based treatment, along with adjuvants, has significantly brought down the high mortality rates that had been observed in precorticosteroid era. Present day research is largely based on elucidating the pathogenesis beyond the antidesmoglein antibodies, and newer diagnostic and treatment approaches. In this article, we review various aspects of literature on pemphigus in India, on Indians abroad, or literature from other countries that are considered relevant to the topic.

Psoriasis: What is new in nonbiologic systemic therapy in the era of biologics.

Psoriasis is a common debilitating disease significantly affecting the quality of life of the patients. Majority of the psoriasis patients have mild disease which can be managed by topical therapies. Around 30% of the psoriasis patients require systemic therapy during the course of their disease. There is a vast array of drugs for the treatment. Methotrexate, cyclosporine and retinoids are the most commonly used conventional systemic drugs. Newer studies provide insight into their more effective and safer use and as combination therapy with biologics. In recent times, many new drugs with novel mechanisms of action other than biologics have been tried in psoriasis. In this article, we have reviewed the current developments and new found role of the conventional drugs as well as the newer nonbiologic systemic drugs in the treatment of psoriasis.

Lichen planus in children.

Lichen planus in children is considered to be rare overall, though it does not appear to be so in Indian subcontinent. Most of the large studies on lichen planus in children have been undertaken in India. We review here the epidemiology, pathogenesis, clinical features, diagnosis, management and prognosis pertaining to lichen planus in children with emphasis on studies published from India.