Clinical and pathological characterization of oral mucosal 'lichen planus-like lesions' in patients with pemphigus vulgaris: An observational study.

BACKGROUND: Lichen planus-like lesions on oral mucosa occasionally occur in Indian patients with pemphigus vulgaris. Its significance, both clinical and pathological, is yet to be elucidated. AIMS AND OBJECTIVES: To study the clinical and pathological characteristics of clinically apparent oral mucosal lichen planus-like lesions in pemphigus patients and to assess their relation with pemphigus disease activity. MATERIALS AND METHODS: A total of 32 patients with pemphigus vulgaris who had oral lichen planus-like lesions were included and classified as 'cases,' and eight diagnosed cases of pemphigus vulgaris without lichenoid 'hue' were included as controls. The biopsy specimens were subjected to routine histopathologic examination, immunohistochemistry with FasL, and caspase-3 and direct immunofluorescence. RESULTS: On histopathologic examination, the diagnosis of pemphigus vulgaris, lichen planus, 'overlap' and 'nonspecific' were rendered in 19 (59.4%), 4 (12.5%), 5 (15.6%) and 4 (12.5%) cases, respectively. On immunohistochemistry, FasL was positive in epithelial cells in 16 (50%) cases and 4 (12.5%) controls (P = 0.066). Caspase-3 stained positively in 18 (56.2%) cases and 20 (62.5%) controls (P = 0.77). Direct immunofluorescence was positive in 77.8% (21/27) of the cases. LIMITATIONS: Relatively small number of controls is the limitation of this study. CONCLUSION: Lichen planus-like lesions in pemphigus should not be labeled as inactive disease or postinflammatory hyperpigmentation. Apoptosis followed by pigment incontinence seems to explain such lesions with 'lichen planus-like appearance' in oral pemphigus vulgaris. Active pemphigus smoulders in a majority of these lesions.

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.

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 vegetans in a patient with colonic cancer.

Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by vegetating plaques in the flexural regions. The coexistence of pemphigus vegetans and internal neoplasm is rare, being described only in four cases in the literature. We report the case of a patient with a typical skin eruption of pemphigus vegetans, who was detected with colonic cancer.

Randomized open comparative trial of dexamethasone-cyclophosphamide pulse and daily oral cyclophosphamide versus cyclophosphamide pulse and daily oral prednisolone in pemphigus vulgaris.

BACKGROUND: In various case series, pulse therapy has shown good results in pemphigus vulgaris (PV), with long-term remissions. AIMS: To compare the efficacy and side-effects of dexamethasone-cyclophosphamide pulse and daily oral cyclophosphamide (DCP+C) versus cyclophosphamide pulse and daily oral prednisolone (CP+P) in PV. METHODS: Twenty-eight active PV patients were randomized to receive either DCP with daily oral cyclophosphamide (Group A, n = 15) or CP with tapering doses of daily oral prednisolone (Group B, n = 13) for 12 months and followed-up for at least 3 months after stopping therapy. They were compared for time taken to achieve mucocutaneous disease control, achieve remission, relapse during treatment period, relapse after stopping therapy and side-effects. RESULTS: Of 28 cases, 25 (Group A - 15, Group B - 10) completed the study period and were analyzed. The time for initiation of cutaneous response and time to achieve complete disease remission were significantly lesser in group B. However, other efficacy parameters were comparable. In Group A, significant adverse events were dysgusea, hiccups, palpitation, nail discoloration, bone pain and urinary tract infection while in Group B, they included nausea, moon facies, flushing, secondary amenorrhea, steroid withdrawal symptoms and dyspnea due to weight gain. CONCLUSIONS: Early remission was achieved in group B but the relapse rates during the treatment phase or after stopping therapy were comparable. Both therapies had comparable side-effect profiles, although Group B showed greater steroid-induced adverse events.

Outcome of dexamethasone-cyclophosphamide pulse therapy in pemphigus: a case series.

BACKGROUND: Pemphigus disorders are a group of serious and potentially life-threatening diseases affecting skin and/or mucus membranes. Dexamethasone-cyclophosphamide pulse (DCP) therapy has shown promising results in the management of these diseases. AIMS: The objective of the study was to assess the outcome of DCP therapy in pemphigus. METHODS: Pemphigus patients who had been treated with DCP therapy from 2001 to 2008 were prospectively and retrospectively analyzed. RESULTS: A total of 65 pemphigus patients were enrolled. Male to female ratio was 1 : 1.82. Mean age of patients was 44.65 +/- 11.85 years with a range of 14 to 73 years. Thirty two patients completed phase I, of which 28 (9 in phase II, 7 in phase III and 12 in phase III) were maintaining remission and four patients relapsed. Relapse was observed only in patients who discontinued or took irregular treatment. Six patients were declared cured of disease. Five patients died during phase I. Dexamethasone-cyclophosphamide pulse therapy is not absolutely free from adverse effects. Most of the immediate side effects were expected, tolerable and did not pose any problem in continuing treatment. There was a slight increased incidence of weight gain, hypertension, diabetes mellitus, cataract and Cushingoid habitus, since most of our patients also received additional daily oral steroids. Also, there was a high incidence of secondary pyogenic infections of skin lesions and oral candidiasis during phase I. Staphylococcus aureus was the commonest bacteria isolated from the pus of skin lesions. Most of the alterations in laboratory parameters were transitory or correctable, and did not pose a problem in continuing therapy. CONCLUSIONS: DCP therapy was found to be effective in inducing and maintaining remission in pemphigus, provided the patients receive regular and complete treatment.

Incidence of pemphigus in Thrissur district, south India.

BACKGROUND: Pemphigus is a common autoimmune blistering disorder in India. However, there are no population based surveys from India available to study the prevalence of this disease. AIMS: To estimate the incidence of pemphigus in Thrissur district, Kerala, South India. METHODS: Questionnaire based survey was conducted among the practicing dermatologists of Trichur district between 1 st January 2001 and 1 st January 2002 to determine the incidence of pemphigus in the district. RESULTS: Ten cases of pemphigus vulgaris, one case of pemphigus erythematosus, and two cases of pemphigus foliacious were diagnosed during the study period in a population of 2.9 million in Thrissur district. The average age of pemphigus vulgaris patients was 58years in males [42 to 82 years] and 37 in females [25 to 57 years]. The average age of the two male pemphigus foliacious patients was 46 years [52 and 40 years] and the only pemphigus erythematosus patient was a 45 year-old female.Five cases of pemphigus vulgaris were investigated clinically histopathologically and by using direct immunofluorescence, while two were investigated clinically and histopathologically, two cases clinically, and one case clinically and with Tzanck smear. Two cases ofpemphigus foliacious were diagnosed with the aid of direct immunofluorescence and the only case of pemphigus erythematosus was diagnosed with the aid of direct immunofluorescence. Pemphigus vulgaris was common among females. The incidence of pemphigus in Thrissur district is 4.4 per million. CONCLUSIONS: This study indicates that the incidence of pemphigus in Thrissur district is high in comparison to the available studies from Germany, France, and the North African country Tunisia.