Pigmented transverse nasal band: A review.

Pigmented transverse nasal band/groove is an asymptomatic benign condition, characterized by the development of erythematous to hyperpigmented, well-demarcated, transverse groove at the junction of middle and lower two-third of the nasal dorsum. Although the pathogenesis is unclear, embryologic origin seems to be the most plausible hypothesis. This condition is often associated other related dermatological conditions such as milia, comedones, seborrheic dermatitis and atopic dermatitis. Diagnosis is mostly clinical, while reassurance is the mainstay of therapy. In persistent cases, topical retinoids have been used. In this article, we have reviewed the different aspects of this condition including treatment, along with the recent updates to create awareness about this dermatological entity.

Nonsegmental vitiligo follows Blaschko's lines and embryonic pigmentary segments.

BACKGROUND: Pathogenic mechanism that determines the localization of vitiligo patches and thus a patterned distribution in patients with nonsegmental vitiligo has remained poorly elucidated. A distributional similarity of the vitiligo patches with Blaschko's lines has been documented in patients with segmental vitiligo, both isolated segmental vitiligo and mixed vitiligo but never in cases of nonsegmental vitiligo. METHODS: Distribution of nonsegmental vitiligo patches on face and neck regions was assessed and compared with Blaschko's lines and also with embryonic pigmentary segments on the face. RESULTS: This study has documented distributional similarity of the nonsegmental vitiligo patches on face and neck with Blaschko's lines and the "embryonic pigmentary segments" among 154 (58.6%) cases. Patches around the palpebral and other fissures like periorbital, perinasal, perioral, and periaural were more common. In addition to the vitiligo patches, the spared areas were also found to respect the embryonic segmental outlines and follow the Blaschko's lines. CONCLUSION: Distributional pattern of the individual nonsegmental vitiligo patches along the Blaschko's lines and embryonic pigmentary segments suggests that mosaicism might control the susceptibility to the disease process in a patterned manner. LIMITATION: No genetic testing could be performed to confirm the hypothesis. Evaluation of nonsegmental vitiligo was done only on the face and neck areas.

Lichenoid pseudovesicular papular eruption on nose: A papular facial dermatosis probably related to actinic lichen nitidus or micropapular polymorphous light eruption.

BACKGROUND: Facial papules are a feature of several clinical conditions and may present both diagnostic and therapeutic challenges. AIM: To describe a grouped papular eruption on the nose and adjoining cheeks that has not been well characterized previously. MATERIALS AND METHODS: A series of consecutive patients with a papular eruption predominantly involving nose and cheeks were evaluated, treated and followed up prospectively at tertiary care centers. Demographic details, clinical features, histopathology and response to treatment were recorded. RESULTS: There were five men and six women (mean age 29.9 ± 6.9 years) who had disease for a mean duration of 17.3 ± 11.1 months. All patients presented with a predominantly asymptomatic eruption of monomorphic, pseudovesicular, grouped, skin colored to slightly erythematous papules prominently involving the tip of nose, nasal alae, philtrum and the adjoining cheeks. A total of 15 biopsies from 11 patients were analyzed and the predominant finding was a dense, focal lymphoid infiltrate restricted to the upper dermis with basal cell damage and atrophy of the overlying epidermis. The eruption ran a chronic course from several months to years. LIMITATIONS: Direct immunofluorescence could not be performed except in one case. Immunohistochemical stains for CD4 and CD8 could not be done owing to nonavailability. Phototesting was undertaken in one patient only. CONCLUSION: Small grouped papules on the nose and adjoining skin with a lichenoid histopathology appear to represent a distinct clinicopathological entity. It may be related to actinic lichen nitidus/micropapular variant of polymorphous light eruption.

Quantitative evaluation of maxillary bone deformation by computed tomography in patients with leprosy.

BACKGROUND: Facial deformation as a sequela of leprosy is caused not only by a saddle nose but also by regression of the maxilla, as well documented in paleopathological observations of excavated skeletal remains of patients with leprosy. However, maxillary changes in living patients have been evaluated only by the subjective visual grading. Here, we attempted to evaluate maxillary bone deformation in patients with leprosy using three-dimensional computed tomography (3D-CT). METHODS: Three-dimensional images centered on the maxilla were reconstructed using multiplanar reconstruction methods in former patients with leprosy (n = 10) and control subjects (n = 5); the anterior-posterior length of the maxilla (MA-P) was then measured. The difference between the MA-P of the patients and those of controls was evaluated after compensating for individual skull size. These findings were also compared with those from previous paleopathological studies. FINDINGS: Three former patients with lepromatous leprosy showed marked atrophy of the maxilla at the prosthion (-8.6, -11.1 and -17.9 mm) which corresponded with the visual appearance of the maxillary deformity, and these results were consistent with paleopathological findings of excavated skeletal remains. Additionally, the precise bone defects of the maxilla could be individually calculated for accurate reconstructive surgery. INTERPRETATION: We have successfully illustrated maxillary bone deformities in living patients with leprosy. This study also confirmed the maxillary regression described in paleopathological studies.

Nasal PCR assay for the detection of Mycobacterium leprae pra gene to study subclinical infection in a community.

Leprosy is a chronic infectious disease caused by Mycobacterium leprae. Identification of Mycobacterium leprae is difficult in part due to the inability of the leprosy bacillus to grow in vitro. A number of diagnostic methods for leprosy diagnosis have been proposed. Both serological tests and molecular probes have shown certain potential for detection and identification of Mycobacterium leprae in patients. In this study, we have investigated whether Mycobacterium leprae DNA from the nasal secretion of healthy household contacts and the non contacts could be detected through PCR amplification as a method to study the sub clinical infection in a community. A total of 200 samples, 100 each from contacts and non contacts representing all age groups and sex were included in this study. The M. leprae specific primer (proline-rich region) of pra gene was selected and PCR was performed using extracted DNA from the sample. A total of 13 samples were found to be positive for nasal PCR for pra gene among the male and female contacts out of which 7% were males and 6% were females. Even though several diagnostic tools are available to detect the cases of leprosy, they lack the specificity and sensitivity. PCR technology has demonstrated the improved diagnostic accuracy for epidemiological studies and requires minimal time. Although nasal PCR studies have been reported from many countries it is not usually recommended due to the high percentage of negative results in the contact.

Molecular Evidence for the Aerial Route of Infection of Mycobacterium leprae and the Role of Asymptomatic Carriers in the Persistence of Leprosy.

BACKGROUND: Leprosy persists as a public health problem. The chain of transmission and mechanism of infection are not completely understood. In the current study, we investigated the route of infection and of disease onset, from airway exposure, colonization, and bloodstream dissemination. METHODS: Mycobacterium leprae DNA was detected through quantitative polymerase chain reaction in nasal vestibule, nasal turbinate mucosa, and peripheral blood samples, along with anti-phenolic glycolipid I serology and skin tests from the same individual, from 113 leprosy patients and 104 household contacts of patients (HHCs). Bivariate statistics and multiple correspondence analysis were employed. RESULTS: The rates of DNA positivity among patients were 66.4% (75 of 113) for nasal swab samples, 71.7% (81 of 113) for nasal turbinate biopsy samples, 19.5% (22 of 113) for blood samples, with seropositivity of 62.8% (71 of 113 samples) and with increasing incidences toward the multibacillary pole of the clinical spectrum. Positivity among HHCs were as follows: 49% (51 of 104) for nasal swab samples, 53.8% (56 of 104) for nasal biopsy samples, 6.7% (7 of 104) for blood samples, and 18.3% (19 of 104 samples) for anti-phenolic glycolipid I serology. During the follow-up of 5-7 years, out of 104 HHCs, 7 developed leprosy (6.7%). Risk for the disease outcome was estimated by comparing results in HHCs who develop leprosy with those not affected. Neither nasal passage nor mucosa positivity was determinant of later disease onset; however, blood presence increased the risk for disease development (relative risk/positive likelihood ratio, 5.54; 95% confidence interval, 1.30-23.62), as did seropositivity (positive likelihood ratio, 3.69 [1.67-8.16]; relative risk, 5.97 [1.45-24.5]). CONCLUSIONS: Our findings strongly suggest that the aerosol route of infection and transmission is predominant and that HHCs contribute to the infection risk to themselves and probably to others.

Intrapatient comparison of Mycobacterium leprae by VNTR analysis in nasal secretions and skin biopsy in a Brazilian leprosy endemic region.

Background: This study compares the strains of genotypes of M. leprae from nasal secretions (NS) and skin biopsy (SB) in the same patient, supplementing conventional epidemiology to gain insight into the infection of leprosy in Fortaleza, Brazil. Methods: The sample consisted of 38 newly diagnosed leprosy patients attending the National Reference Center of Dermatology Dona Libania (CDERM), in Fortaleza, who tested positive for M. leprae by PCR in DNA extracts of nasal secretions. DNA was also extracted from skin biopsy (SB) scrapings of each patient and used for multiplex PCR amplification of M. leprae VNTR loci. The number of repeats at 15 loci were determined by the fragment length analysis method. Results: Locus VNTR genotypes were achieved in 38 NS, and in 38 SB specimens. M. leprae strains differed in their genotypes in paired specimens in all but two of 38 patients. The genotype similarity in the remainder ranged from 53% to 87%. Conclusion: M. leprae 15 VNTR loci genotypes of paired nasal and biopsy skin samples from five patients were identical, while as many as seven loci differed in the 33 other patients. When the NS and biopsy genotypes were pooled and compared, it was found that there was a great variability among different VNTR markers. It is important to investigate other molecular markers suitable for typing genetic variations of the bacilli.

The involvement of upper airway in Wegener's granulomatosis - about four cases.

The authors present four cases of Wegener's granulomatosis patients with multiorganic manifestation forms, but with a prevalent involvement in upper-airway. Granulomatosis diseases of the nose include bacterial infections (rhinoscleroma, tuberculosis, syphilis, lupus, and leprosy), fungal infections (rhinosporidiosis, aspergillosis, mucormycosis, candidosis, histoplasmosis, and blastomycosis) and diseases with unspecified etiology (Wegener's granulomatosis, mediofacial malignant granuloma, and sarcoidosis). We consider an interesting experience regarding Wegener's granulomatosis due to its rarity, being an autoimmune systemic disease, with continuous evolution and multiorganic involvement. The beginning of the disease is like upper airway affection, a kind of "persistent cold", being difficult to differentiate it from a common cold in the head, with a prolonged evolution. It is important to mention that we establish the diagnosis of Wegener's granulomatosis starting with Ear Nose and Throat (ENT) clinical exam, followed by other tests and investigations realized in our Clinic and completed with specialty tests (nephrology, internal medicine and dermatology), meaning that we need a close cooperation with these medical specialties. All the patients presented multiorganic involvement. Notably significant for our four cases is the prolonged evolution in a stable condition in one patient.

Trichorhinophalangeal syndrome type I--clinical, microscopic, and molecular features.

Trichorhinophalangeal syndrome type I (TRPS I) is an autosomal dominant malformation syndrome characterized by a triad of hair alteration, craniofacial and skeletal abnormalities. TRPS1 gene was first identified in 2000 and mapped on chromosome 8q23.3. A 39-year-old female patient with short stature (149 cm) visited for fine sparse and slow-growing hair with receded medio-occipital hairline of roughly triangular shape since infancy. A typical pear-shaped nose and elongated philtrum were noticeable. In addition, she reported deviation of middle phalanges, bilateral coxa varus in both hips and brachydactyly on bilateral fourth digits. Mutation analysis identified a transition of cytosine to thymine at position 1630 (exon 4), which results in amino acid change R544X and a premature stop of translation. There is no established treatment. But through careful evaluation of suspicious cases to identify potential mutation carriers, the patient can receive information about the disease and genetic counseling.