Increased risk of severe course of pemphigus in patients with pemphigus-associated alopecia: a prospective observational study.

BACKGROUND: Pemphigus-associated alopecia is considered rare, and has not been studied in detail. AIM: To evaluate the clinical and immunological characteristics of patients with pemphigus-associated alopecia. METHODS: This prospective observational study included 80 consecutive patients with histopathologically and immunopathologically confirmed pemphigus, of whom 11 (13.8%) were found to have pemphigus-associated alopecia. Alopecia was observed in 11/52 patients with pemphigus and scalp involvement: [0/28 (35.7%) with pemphigus vulgaris and 1/24 (4.2%) with pemphigus foliaceus. The clinical and immunological characteristics of these patients were analysed. RESULTS: Patients with pemphigus-associated alopecia had a significantly higher Pemphigus Disease Area Index total activity score compared with patients who had no pemphigus-associated alopecia (21.8 ± 18.6 and 11.0 ± 20.5, respectively; P = 0.02). Mean serum anti-desmoglein (Dsg)1 antibody concentration was 141.8 ± 66.9 U/mL and 60.0 ± 52.6 U/mL, respectively (P = 0.03), and mean serum anti-Dsg3 concentration was 126.6 ± 36.7 U/mL and 67.4 ± 52.5 U/mL, respectively (P = 0.03). The values for achieving serological remission were 10% and 70%, respectively (P = 0.02). CONCLUSIONS: Pemphigus-associated alopecia is a marker of severe disease and a treatment-resistant disease course.

The value of dermoscopy in diagnosing eyebrow loss in patients with alopecia areata and frontal fibrosing alopecia.

INTRODUCTION: Alopecia areata and frontal fibrosing alopecia are common causes of eyebrow loss (madarosis). OBJECTIVE: Assessment of trichoscopic markers of eyebrow loss in alopecia areata and frontal fibrosing alopecia. MATERIALS AND METHODS: The analysis included 50 patients with scalp alopecia areata with madarosis, 50 patients with scalp frontal fibrosing alopecia with madarosis and 50 healthy controls. In every case, trichoscopy of the eyebrow area was performed. RESULTS: Empty follicular and eccrine duct openings were observed in all patients and presented predominantly as yellow dots. Exclamation mark hairs were only detected in patients with alopecia areata (30%). Tapered hairs, broken hair, black dots and Pohl-Pinkus constrictions were observed in 14%, 36%, 26% and 4% of patients with alopecia areata, respectively, 4%, 16%, 2% and 0% of patients with frontal fibrosing alopecia, respectively, and they were not present in healthy controls. Dystrophic hairs and whitish areas were observed only in patients with frontal fibrosing alopecia (28% and 32%, respectively). Eyebrow regrowth in distinct directions was present in 32% of patients with frontal fibrosing alopecia, 8% of patients with alopecia areata and 4% of healthy controls. Diffuse erythema was detected in 60% of patients with alopecia areata and frontal fibrosing alopecia and 56% of healthy controls. Vellus hairs and upright regrowing hairs were observed in patients with alopecia areata (62% and 58%, respectively), frontal fibrosing alopecia (60% and 84%, respectively) and healthy controls (100% and 100%, respectively). CONCLUSION: Trichoscopy of the eyebrow area is useful in diagnosing patients with isolated eyebrow loss. The most characteristic trichoscopic features of eyebrow loss in alopecia areata include exclamation mark hairs, tapered hairs, broken hairs and black dots. Frontal fibrosing alopecia of the eyebrows is characterized by the presence of dystrophic hairs, white areas and eyebrow regrowth in distinct directions.

Efficacy of perilesional and intralesional triamcinolone acetonide injections in pemphigus vulgaris lesions of the scalp: an effective therapeutic option.

The scalp is a common location for pemphigus vulgaris (PV), and scalp lesions may be resistant to standard treatment. Perilesional/intralesional triamcinolone acetonide (TA) injections have been used successfully to treat oropharyngeal and ocular involvement in PV. Data on the efficacy of perilesional and intralesional triamcinolone acetonide injections in scalp lesions in PV are lacking. We report two patients with immunopathologically and histopathologically confirmed PV and residual scalp lesions resistant to standard treatment, who were treated with perilesional and intralesional injections of TA 10 mg/mL. Clearance of scalp lesions was achieved after one after, respectively, one and two perilesional and intralesional injections. Perilesional and intralesional TA injections may serve as an effective and safe treatment for recalcitrant scalp lesions in pemphigus.

Reflectance confocal microscopy as a non-invasive diagnostic tool for Hailey-Hailey disease.

BACKGROUND/PURPOSE: Reflectance confocal microscopy (RCM) is a non-invasive method for high-resolution, in vivo imaging of the epidermis and upper dermis. The purpose of the study was to evaluate the potential usefulness of RCM as a non-invasive diagnostic tool for Hailey-Hailey disease (familial benign chronic pemphigus). METHODS: Four patients with Hailey-Hailey disease were examined by RCM. Subsequently, punch biopsies were taken to compare RCM images with corresponding histopathologic findings. RESULTS: On RCM images, the most sticking feature was acantholysis at the level of the granular and spinous layer, resembling a 'dilapidated brick wall'. We suggest the term 'dilapidated brick wall RCM sign' to describe this feature and to distinguish from the corresponding histopathology finding. Other RCM features included: epidermal disarray, intraepidermal clefts, inflammatory cells in the epidermis and in the superficial dermis. These RCM abnormalities correlated with analogous histopathology findings. CONCLUSION: Reflectance confocal microscopy is a promising non-invasive diagnostic tool for Hailey-Hailey disease. The method may also be considered useful for choosing the best site for biopsy, which may aid pathology evaluation and spare time needed to establish the diagnosis.

'Black dots' seen under trichoscopy are not specific for alopecia areata.

BACKGROUND: 'Black dots' are macrocomedo-like round structures localized to the follicular ostium, and are considered a specific trichoscopic feature of alopecia areata (AA). AIM: To characterize specific features of 'black dots', and assess their possible presence in common hair and scalp disorders. METHODS: In total, 107 patients with hair loss [30 with alopecia areata (AA), 37 with androgenetic alopecia (AGA), 17 with chronic telogen effluvium (TE), 23 with other hair and scalp diseases] and 93 healthy controls were examined, using a videodermoscope with 20-70 times magnification. RESULTS: There was a correlation between the black dots and the early acute phase of the various alopecia types with the presence of the black dots. Black dots were found in 11% (22/107) of patients with hair loss, including 53.3% (16/30) with AA; in 40% (2/5) of patients with severe chemotherapy-induced alopecia, and in 100% of patients with dissecting cellulitis of the scalp (n = 2), hypotrichosis simplex (n = 1), and congenital aplasia cutis (n = 1). No black dots were seen in patients with AGA or TE. CONCLUSIONS: Black dots are not specific for AA, and may be present in other hair and scalp diseases.