Conradi-Hünermann-Happle syndrome: Clinical and trichoscopic findings.

Conradi-Hünermann-Happle syndrome (CHHS) is a rare genodermatosis resulting from mutations in the EBP (emopamil binding protein) gene. Dermatologic manifestations may include cicatricial alopecia, ichthyosis, follicular atrophoderma, pigmentary abnormalities, and nail dystrophy. In addition to genetic testing and clinical findings, trichoscopic findings may aid in the diagnosis. In this case report, we discuss the trichoscopic findings in a 3-year-old girl with CHHS and how these findings help us understand the pathophysiology of this disease.

In vitro determination of Mexican Mestizo hair shaft diameter using optical coherence tomography.

BACKGROUND: Mexican mestizo population has a pluriethnic mixture of Amerindian, European and African ancestry, predominant in most Latin American countries. Until now, there are no reports about hair characteristics in this population, necessary to define normal values, for hair diseases evaluation and comparison among other ethnic groups. METHODS: The VivoSight® swept-source multibeam optical coherence tomography system was used to evaluate hair diameter and shape in 30 females. Three hair samples from each volunteer were measured transversely along three distances, generating nine cross-sectional images, two measurements per image and 18 measurements per patient for a total of 540 diameter measurements. RESULTS: Minimum hair diameter (n = 540) was 0.06 mm while maximum was 0.14 mm. Mean diameter was 0.10 ± 0.01 mm as compared with Asian hair, which ranges from 0.08 mm to 0.12 mm. On morphological analysis, Mexican Mestizo hair tends to have a round shape with homogenous diameters, resembling Caucasian and Asian hair. CONCLUSION: Mexican hair is similar to Asian hair in diameter and shape and can be classified as 'thick' hair, which make it more resistant and with more volume. Cosmetic products intended to improve hair care in this population must to consider this characteristic.

Short anagen syndrome: Case series and literature review.

BACKGROUND/OBJECTIVES: Short anagen syndrome is a hair cycle disorder usually diagnosed in early childhood and characterized by short hair length due to short duration of the anagen phase. The objective was to review the presentation and demographic characteristics of short anagen syndrome and compare them with the most common differential diagnosis, loose anagen syndrome. METHODS: A retrospective review of eight children with short anagen syndrome was performed at the University of Miami Outpatient Dermatology Clinic. RESULTS: The diagnosis of short anagen syndrome was confirmed according to clinical findings and characteristic short telogen hairs with pointed tips on the hair pull test. CONCLUSION: This is the largest reported clinical series of short anagen syndrome thus far in the literature.

Scalp Rosacea: Rethinking Peripilar Scaling.

INTRODUCTION: Scalp rosacea is scarcely reported in the literature, but it is probably not uncommon. Trichoscopic findings have not been specifically established for this entity. CASE PRESENTATION: We report 4 cases of chronic scalp rosacea with trichoscopic evidence of peripilar scaling that resolved without scarring after treatment. DISCUSSION/CONCLUSION: Chronic and persistent inflammation around the isthmus produced in scalp rosacea may form peripilar scaling resembling that found in lichen planopilaris.

Association of Trichorhinophalangeal Syndrome and Loose Anagen Syndrome: A Case Report.

Trichorhinophalangeal syndrome (TRPS) is an autosomal dominant disease characterized by trichologic, craniofacial, and musculoskeletal abnormalities. Predominant clinical features include a pear-shaped nose with bulbous tip, long philtrum, protruding ears, and sparse hair on both the scalp and the lateral third of the eyebrows. Cone-shaped epiphyses are a common radiographic finding. Loose anagen syndrome (LAS) is a sporadic or autosomal dominant condition due to abnormalities in the hair's anchoring mechanism. It mostly affects children, who usually have reduced hair length, as well as hair that is easily plucked without pain. Recent contributions regarding trichoscopic findings of LAS have been made, describing rectangular black granular structures as a typical feature. An association between TRPS and LAS has been mentioned by hair experts, but no reports documenting this have been published. This case demonstrates the co-existence of both conditions in a patient with characteristic phenotypic traits of TRPS and LAS.

Scalp Itch: A Systematic Review.

Scalp itch is a frequent complaint in the dermatological setting. It is common for the dermatologist to encounter patients with no evident cause of scalp pruritus, making it a distressing situation for both the clinician and the patient. The aim of this paper is to propose a systematic approach to scalp itch, which classifies scalp pruritus into two types: (1) with or (2) without dermatological lesions, and presence or absence of hair loss. Also, it is important to think first about the most common causes and then rule out other, less common etiologies. The acronym SCALLP and the five steps for scalp evaluation (listen, look, touch, magnify, and sample) are useful tools to keep in mind for an assertive approach in these patients.

Frontal Fibrosing Alopecia Severity Index: A Trichoscopic Visual Scale That Correlates Thickness of Peripilar Casts with Severity of Inflammatory Changes at Pathology.

BACKGROUND: Frontal fibrosing alopecia (FFA) is a scarring alopecia that mainly affects postmenopausal women characterized by recession of the frontotemporal hairline and eyebrow loss. Current techniques to assess FFA activity are limited and involve noninvasive tools that assess disease progression or an invasive technique such as scalp biopsies. However, since progression of FFA is very slow, it is very important to develop a noninvasive technique to assess disease activity to monitor treatment response. OBJECTIVES: To provide a standardized and objective method to assess FFA activity. METHODS: We evaluated the correlation between trichoscopy and pathological features (degree of lymphocytic infiltration) in 20 dermoscopy-guided biopsies of FFA. At trichoscopy, we divided the severity of peripilar casts into 3 grades according to their thickness. To validate the trichoscopic visual scale, we showed the images to 7 dermatologists with interest in hair diseases. Concordance was assessed using the Kendall Tau-b concordance test. RESULTS: A strong correlation between severity of peripilar casts at trichoscopy and degree of lymphocytic infiltrate was observed by the Kendall Tau-b test. Validation showed very good inter- and intraobserver agreement. CONCLUSION: The trichoscopic visual scale allows noninvasive assessment of scalp inflammation in FFA in different scalp regions and therefore provides optimal guidance for treatment.

The Hair Shedding Visual Scale: A Quick Tool to Assess Hair Loss in Women.

INTRODUCTION: Hair shedding is a common consequence of the normal hair cycle that changes with internal and external factors. Female pattern hair loss (FPHL) is difficult to assess in terms of shedding severity as the conscious perception of hair shedding varies according to each individual, and most utilized methods are semi-invasive or very time consuming. In this study, we establish and validate a hair-shedding scale for women with thick hair of different lengths. METHODS: A visual analog scale was developed for thick hair of short, medium, and long lengths by dividing a bundle of hairs of each length into nine piles of increasing hair amount that were then photographed and arranged in order of size. Twenty women with no FPHL with each length of hair (60 total) were asked to select the photographed hair bundle that best correlated with the amount of hair they shed on an average day. A total of 94 women with FPHL with excessive shedding were then asked to repeat the same process. RESULTS: Women with no FPHL and short, medium and long hair had mean shedding scores of 2.5, 2.35 and 2.4, respectively. Women with FPHL and short, medium and long hair had mean shedding scores of 7.25, 7.0 and 7.14, respectively. Statistically significant Spearman's ρ coefficient and κ coefficient demonstrated correlation and inter-observer reliability. CONCLUSION: Our results show that women with FPHL not only shed considerable hair more than women with no FPHL, but that this hair-shedding visual scale is a fast and effective method of evaluating hair-shedding amounts in an office setting.

Angioimmunoblastic T-cell lymphoma: a diagnostic challenge.

Angioimmunoblastic T-cell lymphoma (AITL) accounts for 15-20% of all peripheral T-cell lymphomas. It is a rare subtype of CD4 T-cell peripheral lymphoma that affects aged individuals, causing B symptoms, generalized lymphadenopathy and hepatosplenomegaly. Its pathogenesis is still unclear, but in some cases it has been associated with infection, allergic reaction or drug exposure. The majority of patients are diagnosed in an advanced stage and anthracycline based regimen is considered the first-line therapy. Skin involvement is not well characterized, occurring in up to 50% of patients and presenting as nonspecific rash, macules, papules, petechiae, purpura, nodules and urticaria. We present the illustrative case of a 55-year-old woman with an AITL who presented prominent skin findings, arthritis, lymphadenopathy and hypereosinophilia. Skin biopsy reported a T-cell lymphoma and the diagnosis of AITL was confirmed by an axillary lymph node biopsy, which was also positive for Epstein-Barr virus. Chemotherapy with CHOP-21 and thalidomide was given, accomplishing complete remission after six cycles.