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.

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.

Childhood granulomatous periorificial dermatitis in children with extra-facial involvement.

Childhood granulomatous periorificial dermatitis (CGPD) is a self-limiting and well-recognized entity. A six-year-old male child, a known case of juvenile rheumatoid arthritis (JRA) presented with multiple red raised and yellowish lesions over the face, neck, trunk and upper extremities since one month with occasional itching. Cutaneous examination revealed multiple erythematous scaly papules of size up to 5 mm around the mouth, nose and periorbital areas, neck, trunk and upper extremities with few excoriations. Lesional skin biopsy was pathognomic of CGPD. We report a six-year-old Indian male child with extra-facial involvement and healing with small atrophic pigmented scars in a known case of JRA.

Granulomatous mycosis fungoides with hypohidrosis mimicking lepromatous leprosy.

Granulomatous mycosis fungoides (GMF) is a rare type of cutaneous T cell lymphoma. A 38-year-old married male presented with decreased sweating all over the body for last 8 years, progressive redness and scaling over body for 2 years and multiple noduloulcerative lesions over the body for 1 year. Cutaneous examination revealed generalized erythema and scaling with poikilodermatous changes over chest and upper back along with multiple noduloulcerative lesions. Skin biopsy from a nodular lesion revealed dense granulomatous infiltrate of atypical lymphocytes with epidermotropism and sparing of appendages. Diagnosis of GMF was made. Computed tomographic scan of thorax, abdomen and pelvis revealed axillary and inguinal lymphadenopathy. Immunohistochemistry revealed leukocyte common antigen and CD3 positivity suggestive of T cell origin. Patient was started on CHOP (Cyclophosphamide, Hydroxydaunorubicin, Oncovin and Prednisolone) regimen of chemotherapy with marked improvement after three cycles of chemotherapy. This case had some clinical resemblance to lepromatous leprosy.

Nodular amelanotic melanoma.

We report a case of 65-year-old male patient who presented with multiple erythematous papules coalescing to form a nodular mass over posterior aspect of right thigh of six months duration. His general and systemic examinations were within normal range except for right inguinal lymphadenopathy. Biopsy from the lesion was done, which showed diffuse infiltrate of nests of atypical melanocytes extending upto reticular dermis. Malignant cells were positive for S100 and human melanin black 45(HMB 45). Hence, a diagnosis of amelanotic melanoma (AM) - Clarke level IV and TNM stage III was reached. MRI of involved leg showed fungating soft tissue mass in the posterolateral aspect of right thigh and metastatic right inguinal adenopathy. Fine needle aspiration cytology (FNAC) from the right inguinal nodes confirmed metastasis of melanoma. The patient was referred to oncosurgery department for further management.

Multiple capillary hemangiomas: a distinctive lesion of multicentric Castleman's disease and POEMS syndrome.

A diagnosed case of Castleman's disease, proven by biopsy from enlarged inguinal lymph nodes, presented with multiple, asymptomatic, erythematous papules and nodules prevalent since nine years over the trunk and extremities. The lesions had been gradually increasing in number and size. The patient had had plasmacytoma of the lower thoracic vertebra 12 years ago, for which he was adequately treated with chemotherapy and local radiotherapy. Dermatological examination revealed erythematous papules and nodules on the face, trunk, and extremities that were diagnostic of capillary hemangiomas. Histopathology of the erythematous, soft papule was suggestive of capillary hemangioma. Contrast-enhanced computerized tomography of the abdomen and pelvis showed multiple retroperitoneal nodes suggestive of Castleman's disease along with multiple osteolytic lesions in the pelvic girdle and vertebrae. The patient was treated with injection rituximab and is currently under follow-up. We report this case to highlight a rare association between Castleman's disease and POEMS syndrome.

Type I lepra reaction presenting as immune reconstitution inflammatory syndrome.

Immune reconstitution inflammatory syndrome (IRIS) is an unusual inflammatory reaction due to infectious and non-infectious causes occurring in human Immunodeficiency virus (HIV)-infected patients. IRIS occurs after the initiation of antiretroviral therapy. There are no reports of type I lepra reaction due to IRIS in published literature from India. We report two cases of HIV-infected males who presented with borderline tuberculoid leprosy in type 1 reaction after the initiation of highly active antiretroviral treatment (HAART). Case 1 presented with multiple, tender, erythematous and hypoesthetic plaques on the trunk and extremities after 3 months of antiretroviral therapy. In case 2, type I lepra reaction was observed 2 months after the initiation of HAART.

Cutaneous vasculitis as a presenting feature of multiple myeloma: a report of 2 cases.

We report two male patients who presented with symmetrical, painful purpura that evolved into bullae and necrotic ulcers, predominantly on the extremities, over two months in spite of conventional therapy including oral steroids. Examination showed livedoid and purpuric patches with necrotic centers in starburst pattern over the extremities and buttocks. The first case also had similar lesions over the ears. The clinical presentation and the histopathological examination suggested a diagnosis of necrotizing leukocytoclastic vasculitis (LCV). Blood testing ruled out connective tissue disease, hepatitis B or C infection or streptococcal infection as underlying cause of vasculitis. Serum antinuclear factor, antineutrophilic cytoplasmic antibody and anticardiolipin anticoagulant were negative in both cases. Cryoglobulins were positive in case 2. An incidental finding was raised serum proteins and globulins in case 2. Further investigations revealed M band on electrophoresis and features of multiple myeloma on bone marrow biopsy in both cases. These cases emphasize the importance of simple investigations like serum proteins in the evaluation of LCV.