Cutaneous plasmacytosis with mast cell infiltration.

Cutaneous plasmacytosis is a rare disorder of uncertain etiology, described mainly in patients of Japanese descent. Clinically, it is characterized by multiple pigmented papules and plaques distributed primarily on the trunk. Histopathologically, it is marked by a dense dermal plasma cell infiltrate. Here, we describe a case of cutaneous plasmacytosis in a 55-year-old Indian male who presented with hyperpigmented plaques on the body. Histopathological examination revealed dense superficial and deep perivascular and periappendageal infiltrate composed mainly of plasma cells, lymphoid follicles with reactive germinal centres, perineural distribution of plasma cells, mast cell infiltration and increased dermal small blood vessels. Immunohistochemical analysis confirmed the polyclonal nature of the plasma cells. Laboratory investigations were within normal limits, except for the presence of polyclonal hypergammaglobulinemia without any M band. There was no evidence of autoimmune disease or any infection. There was no systemic involvement in this patient. The patient was diagnosed as cutaneous plasmacytosis and advised long-term follow-up. Peculiar histopathological finding in this case of cutaneous plasmacytosis was the presence of abundant mast cells in the dermis.

A study of prevalence of autoantibodies in patients with lichen planus from Mumbai, India.

BACKGROUND: Lichen planus is a common chronically relapsing autoimmune skin condition with poorly understood etiology. Apart from cellular immunity, presence of various antibodies has been hypothesized. Various studies have found the presence of serum anti-nuclear antibody, anti-mitochondrial antibody, anti-desmoglein 1 and 3 antibodies, anti-keratinocyte antibody and anti-thyroglobulin antibody in patients of cutaneous and oral lichen planus. AIM: To study the prevalence of autoantibodies and the clinical spectrum of disease in an Indian patient subpopulation with lichen planus. METHODS: A cross-sectional epidemiological study comprising 100 lichen planus patients was conducted in the dermatology outpatient department of Seth G.S Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra, India. Serum concentrations of circulating anti-nuclear antibodies, anti-desmoglein 1 antibody, anti-desmoglein 3 antibody, anti-keratinocyte antibodies, anti-mitochondrial antibodies and anti-thyroglobulin antibodies were determined by indirect immunofluorescence. Pairs of groups were compared using "Student's t-test" for normally distributed continuous data. The "χ2-test" was used for the categorical variables as needed. Statistical significance was set at P < 0.05. RESULTS: It was found that 65 (65%) patients showed the presence of at least one of the six autoantibodies that we studied, while 35 (35%) tested negative for all six of them. Positivity of anti-keratinocyte antibody in 26 (26%), anti-nuclear antibody in 22 (22%), anti-desmoglein 1 antibody in 19 (19%), anti-desmoglein 3 antibody in 16 (16%), anti-mitochondrial antibody in 9 (9%) and anti-thyroglobulin antibody in 6 (6%) patients was detected. It was observed that 55 (71.4%) patients of cutaneous lichen planus, 6 (46.1%) patients of mucosal lichen planus and 4 (40%) patients of cutaneous and mucosal lichen planus overlap showed presence of at least one autoantibody. CONCLUSION: This study provides the serological parameters of a population of lichen planus from western India. Presence of autoantibodies in lichen planus suggests the possible role of humoral immunity in lichen planus. Identifying antibodies linked to lichen planus may help in identifying suitable diagnostic tests and therapeutic targets. Well-controlled studies with larger sample size are the need of the hour to confirm the role of humoral immunity in lichen planus. LIMITATIONS: Studies with a larger number of patients as well as controls should be undertaken to further evaluate the role of autoantibodies in lichen planus.

The utility of dermoscopy in the diagnosis of evolving lesions of vitiligo.

BACKGROUND: Early lesions of vitiligo can be confused with various other causes of hypopigmentation and depigmentation. Few workers have utilized dermoscopy for the diagnosis of evolving lesions of vitiligo. AIM: To analyze the dermoscopic findings of evolving lesions in diagnosed cases of vitiligo and to correlate them histopathologically. METHODS: Dermoscopy of evolving lesions in 30 diagnosed cases of vitiligo was performed using both polarized light and ultraviolet light. RESULT: On polarized light examination, the pigmentary network was found to be reduced in 12 (40%) of 30 patients, absent in 9 (30%), and reversed in 6 (20%) patients; 2 patients (6.7%) showed perifollicular hyperpigmentation and 1 (3.3%) had perilesional hyperpigmentation. A diffuse white glow was demonstrable in 27 (90%) of 30 patients on ultraviolet light examination. Melanocytes were either reduced in number or absent in 12 (40%) of 30 patients on histopathology. CONCLUSION: Pigmentary network changes, and perifollicular and perilesional hyperpigmentation on polarized light examination, and a diffuse white glow on ultraviolet light examination were noted in evolving vitiligo lesions. Histopathological examination was comparatively less reliable. Dermoscopy appears to be better than routine histopathology in the diagnosis of evolving lesions of vitiligo and can obviate the need for a skin biopsy.

Intermediate doses of rituximab used as adjuvant therapy in refractory pemphigus.

BACKGROUND: Rituximab, a monoclonal anti-CD20 antibody, has been used with encouraging results in pemphigus. We describe herein refractory cases of pemphigus vulgaris (n = 23) and pemphigus foliaceus (n = 1) treated with rituximab in addition to steroids and immunosuppressants. AIMS: To assess the response to treatment, the duration of clinical remission, serology of the response and adverse effects of rituximab in pemphigus patients. METHODS: We recorded observations of 24 patients with pemphigus having either refractory disease in spite of high dose of steroids and immunosuppressants, corticosteroid-dependent disease, strong contraindications to corticosteroids, or severe disease. The patients were treated with infusions of one injection per week for three consecutive weeks of 375 mg of rituximab per m ² of body-surface area. One similar infusion was repeated after 3 months of 3 rd dose. We observed the clinical outcome after 6 months of 3 rd dose of rituximab and looked for complete healing of cutaneous and mucosal lesions (complete remission). OBSERVATIONS: After follow-up of 7-24 months, five patients showed only partial improvement while 19 of 24 patients had a complete remission 3 months after rituximab. Of these 19 patients, 12 patients achieved complete remission and are off all systemic therapy, and the rest are continuing with no or low dose of steroids with immunosuppressants. Two patients relapsed after initial improvement; one was given moderate dose of oral steroids and immunosuppressant and the other was given repeat single dose of rituximab to control relapse. CONCLUSION: Rituximab is able to induce a prolonged clinical remission in pemphigus after a single course of four infusions. The high cost and limited knowledge of long term adverse effects are limitations to the use of this biologic agent.

Tattoo reactions--an epidemic on the surge: a report of 3 cases.

Tattooing has been practiced in India since ancient era. It has tremendous religious and spiritual significance. In addition, tattooing for cosmetic purposes has become quite popular in recent times. With this increasing trend, there is also an increased risk of adverse effects. Here, we have described two cases of lichenoid reaction developing to red ink in double- colored tattoos and a case of sarcoidal reaction to green tattoo.

Epidermodysplasia verruciformis: an unusual malignant transformation.

Epidermodysplasia verruciformis (EV) is a rare, life-long heritable disease caused due to a unique susceptibility to human papilloma virus. The disseminated verrucous lesions and pityriasis versicolor-like lesions persist from early childhood and can transform into a cutaneous malignancy in a fourth of patients. Malignant transformation into syringoid eccrine carcinoma (SEC) has been reported only once so far. SEC is an extremely invasive, rare, locally destructive, slowly growing adnexal tumor. We hereby report the association of EV with SEC in a 29-year-old male.