Unusual cutaneous manifestations of dracunculiasis: Two rare case reports.

A nematode parasite, Dracunculus medinensis, causes dracunculiasis. Despite being non-fatal, this condition causes significant morbidity. Dracunculiasis is considered an eradicated disease in India since 1999. We report two cases that document the unusual linear morphea-like morphology of the calcified D. medinensis and the rare periorbital location of the worm. The cases presented here are rare and a diagnostic challenge, considering the eradicated status of dracunculiasis.

A case of disseminated subcutaneous phaeohyphomycosis caused by Exserohilum rostratum with CARD9 mutation.

Phaeohypomycosis is a rare cutaneous and subcutaneous fungal infection caused by dematiaceous fungi. They have a widespread global distribution occasionally affecting humans. A 26-year-old woman presented with multiple skin lesions over her face and extremities for last 7 years, unresponsive to systemic amphotericin B and itraconazole. Further investigations revealed CARD9 mutation and phaeohyphomycosis caused by the pigmented fungus Exserohilum rosatratum. Lesions subsequently improved with oral flucytosine and itraconazole.

A descriptive observational study on clinical and dermoscopic features of benign melanocytic neoplasms.

BACKGROUND: Benign melanocytic neoplasms have nests of melanocytic cells and show characteristic dermoscopic features. Clinical and dermoscopic features have not been studied previously in the Indian population. AIMS: To study the clinical, epidemiological and dermoscopic patterns of benign melanocytic neoplasms. METHODS: This was a descriptive, observational, single centre study. In 107 patients with melanocytic neoplasms, 167 lesions were clinically examined and studied under the dermoscope and histopathological examination was done when indicated. The lesions were broadly divided as acquired and congenital. Five main dermoscopic patterns were seen-globular, homogenous, reticular, parallel and streaks. If there were two of these patterns in a particular lesion, it was termed 'mixed pattern'. The presence of three or more patterns was called 'multicomponent pattern'. Various other features were also observed. RESULTS: The majority of patients belonged to the third decade with a female preponderance. History of increased UV exposure and family history was significant in acquired nevi. The dermoscopic pattern progressed from predominantly reticular in junctional nevi to predominantly globular in compound nevi and lesser pigment in intradermal nevi, with more vascular structures. The congenital melanocytic nevi showed additional features of comedo- like lesions, milia- like cysts, perifollicular pigmentary changes and increased colour variation. Even though colour variation was observed in both acquired and congenital lesions, no signs of dysplasia were seen on histopathology. LIMITATIONS: A larger sample size is required, with follow up of lesions. No parallel studies in brown skinned population were found for exact comparison. CONCLUSION: Benign melanocytic proliferations are often neglected in our country. This study will help in understanding the course, clinical features and dermoscopic patterns of various benign melanocytic neoplasms, and will be a step forward towards research in our population. To the best of our knowledge, this is the first study of its kind in India.

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.

Localized unilateral basaloid follicular hamartoma along Blaschko's lines on face.

Basaloid follicular hamartoma (BFH) is a rare hamartoma of hair follicle. Clinical presentations may vary but are united by the same histopathological features in the form of folliculocentric basaloid or squamoid cell proliferation in the superficial dermis, which represents malformed and distorted hair follicles. It is important to recognize this entity as its simulant is basal cell carcinoma, a low-grade malignancy. Here, we report a case of localized unilateral BFH in a Blaschkoid distribution on the face of a 14-year-old female.

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.

Clinical profile and mutation analysis of xeroderma pigmentosum in Indian patients.

BACKGROUND: Xeroderma pigmentosum (XP) is an autosomal recessive genetic disorder characterized by cutaneous and ocular photosensitivity and an increased risk of developing cutaneous neoplasms. Progressive neurological abnormalities develop in a quarter of XP patients. AIM: To study the clinical profile and perform a mutation analysis in Indian patients with xeroderma pigmentosum. METHODS: Ten families with 13 patients with XP were referred to our clinic over 2 years. The genes XPA, XPB and XPC were sequentially analyzed till a pathogenic mutation was identified. RESULTS: Homozygous mutations in the XPA gene were seen in patients with moderate to severe mental retardation (6/10 families) but not in those without neurological features. Two unrelated families with a common family name and belonging to the same community from Maharashtra were found to have an identical mutation in the XPA gene, namely c.335_338delTTATinsCATAAGAAA (p.F112SfsX2). Testing of the XPC gene in two families with four affected children led to the identification of the novel mutations c.1243C>T or p.R415X and c.1677C>A or p.Y559X. In two families, mutations could not be identified in XPA, XPB and XPC genes. LIMITATION: The sample size is small. CONCLUSION: Indian patients who have neurological abnormalities associated with XP should be screened for mutations in the XPA gene.

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.