Hair Disorders in Autoimmune Diseases.

Alopecia is a common feature in several autoimmune diseases. With a wide spectrum of clinical presentations, it may manifest with a scarring or non-scarring nature, in a diffuse, patchy, or localized pattern. We as dermatologists have the opportunity of assessing patients with hair loss who may have an underlying undiagnosed autoimmune disorder. This review aimed to describe the main clinical, trichoscopic, and histopathological features of hair disorders associated with autoimmune diseases.

Oral tranexamic acid with a triple combination cream versus oral tranexamic acid monotherapy in the treatment of severe melasma.

BACKGROUND: Melasma is an acquired pigmentation disorder with a complex multifactorial etiopathogenesis. Oral tranexamic acid (TA) is a promising drug for its treatment and may enhance outcomes when used in combination. OBJECTIVE: To provide evidence of the efficacy and safety of oral TA as a monotherapy, and in combination with a triple combination cream, for treating melasma in the Hispanic population. METHODS: Forty-four female Hispanic patients with melasma were randomly assigned to receive 325 mg of oral TA every 12 h plus f-TCC (fluocinolone-based triple combination cream) every 24 h (group A) or 325 mg of oral TA every 12 h (group B) for 8 weeks, after which both groups were crossed-over, and treated for an additional 8 weeks. Evaluations of the mMASI score, the melanin index, and the MelasQoL were made at baseline and Weeks 4, 8, 12, and 16. RESULTS: There was a 50.04% and 65.45% improvement in mMASI at Weeks 4 and 8, respectively, in group A, compared to baseline, while for Week 16, an improvement of 76.85% was achieved in group B compared to baseline. Highest scores were consistent with the use of the combined treatment modality in both groups, and were evidenced by the values of the melanin index obtained. There was no significant difference in MelasQoL scores between the 2 groups. No serious side effects were observed. CONCLUSION: The combination of oral TA and f-TCC is more effective than oral TA alone in the treatment of severe melasma in Hispanic patients.

Mycophenolate mofetil and mycophenolic acid for the treatment of eosinophilic fasciitis: report of two cases and literature review.

Eosinophilic fasciitis (EF) is an uncommon autoimmune connective tissue disorder characterized by edema, erythema, and subsequent induration of the extremities. It is commonly treated with corticosteroids but there is no treatment ladder for immunosuppressants or steroid-sparing agents. We report two EF cases treated effectively with mycophenolate mofetil (MMF) or mycophenolic acid (MPA) and present a literature review. We performed a MEDLINE search using the keywords 'eosinophilic fasciitis', 'Shulman syndrome', 'mycophenolic acid', or 'mofetil mycophenolate', and found 8 articles with 27 cases in which MMF or MPA was used. Twenty-nine cases were reviewed (2 reported herein and 27 from the literature search); all patients received a combination of systemic corticosteroids and MMF. MMF/MPA were given as a steroid-sparing agent in 27 (93.1%), in 1 (3.4%) as adjunctive therapy with other immunosuppressants, and in one, as monotherapy 1 (3.4%). Nineteen had a complete response, 6, a partial response, and 2 were unresponsive to diverse immunomodulators; in 2 cases, the outcome was not reported. MMF and MPA show promising therapeutic results and could be a treatment option to reduce corticosteroid related side effects.

Frontal Fibrosing Alopecia: Is There a Link in Relatives?

Frontal fibrosing alopecia (FFA) is an acquired primary lymphocytic cicatricial alopecia characterized by frontotemporal hairline recession, leading to scarring alopecia with a band-like distribution. Prevalence is increasing worldwide, being the most frequent cause of primary scarring alopecia. The natural history of this condition is variable; however, slow progression with spontaneous remission is the most frequent reported outcome. The etiopathogenesis of FFA remains to be elucidated; numerous hypotheses concerning hormonal effects, environmental factors, and genetic predisposition have been proposed. Special interest on genetic basis has emerged since the first familial case was reported. Only a few more familial cases have been published. We report 6 additional cases of female patients with familial FFA (F-FFA) from 3 different families. Sixty-six percent had a family history of autoimmune disease in first-degree relatives; these same patients had a personal history of autoimmune disease. The families described in this cohort study plus the personal and family history of autoimmune disease, as well as the recently described involved genomic loci; reinforced the hypothesis of this disease being genetic. It is important to consider studying this entity since there are scarce data regarding familial cases and this might give us a better insight toward understanding its pathogenesis.

Efficacy of Intralesional and Oral Dutasteride in the Treatment of Androgenetic Alopecia: A Systematic Review.

Androgenetic alopecia is the most common cause of hair loss [Br J Dermatol. 2011 Jan;164(1):5-15]. Finasteride and minoxidil are the only approved treatments [J Am Acad Dermatol. 2008 Oct;59(4):547-8 and J Eur Acad Dermatology Venereol. 2018 Jan;32(1):11-22]. Dutasteride is more potent than finasteride due to its ability to inhibit both 5-α-reductase type I and II [Our Dermatol Online. 2017 Sep;9(1):75-9] though its adverse effects and long half-life contribute to the reluctance on its oral use. Mesotherapy could be a feasible alternative to avoid systemic exposure and side effects [J Pan-Arab League Dermatologist. 2009 Feb;20(1):137-45]. We aim to perform a systematic review to analyze scientific literature with the purpose of comparing efficacy and adverse effects of both administration routes. Five clinical trials using oral route and 3 intralesional in comparison with placebo met criteria for inclusion. Regarding intralesional dutasteride, only one study [Clin Dermatol. 2001 Mar;19(2):149-54] reported the mean change in hair count. Although both interventions favor over placebo, there are not enough data to reliably compare outcomes obtained between both routes. Mean increase in hair count observed with oral dutasteride was higher (MD: 15.92 hairs [95% CI: 9.87-21.96]; p = <0.00001; I 2 = 90%) compared to intralesional dutasteride in Abdallah's study (MD: 7.90 hairs [95% CI: 7.14-8.66]; p = <0.00001). Future studies are required to assess the therapeutic efficacy of both treatment routes, including head-to-head treatments before well-supported conclusions can be established.

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.

Acral bullous lichen sclerosus intolerant to UVA-1 successfully treated with narrowband ultraviolet B phototherapy.

Lichen sclerosus (LS) is an uncommon, chronic, lymphocyte-mediated, inflammatory dermatosis characterized by ivory-white patches with scar-like atrophy. Extragenital bullous lichen sclerosus may rarely affect palms and soles, causing severe pain and substantially impairing quality of life. We present the first case of acral bullous lichen sclerosus intolerant to UVA-1 phototherapy successfully treated with low doses of narrowband ultraviolet B phototherapy.

Lichen planus pigmentosus and its variants: review and update.

Lichen planus pigmentosus (LPP) is considered a rare variant of lichen planus (LP). It is characterized by acquired dark brown to gray macular pigmentation located on sun-exposed areas of the face, neck, and flexures, commonly found in dark-skinned patients. In patients with LPP, an inflammatory lichenoid response results in marked pigmentary incontinence. It has been associated with hepatitis C virus, sun exposure, and contactants such as mustard oil and nickel. LPP-inversus affects fair and dark skin, predominantly involving flexural and intertriginous areas, while sun-exposed areas are spared; friction is an associated trigger. LPP along Blaschko's lines has been associated with susceptibility to genetic mosaicisms. LPP can present concomitantly with other variants of LP such as frontal fibrosing alopecia, as well as endocrinopathies, and autoimmune diseases. Treatment is difficult and consists of avoidance of triggers and topical and systemic medications in order to stop the inflammatory reaction and reduce pigmentation, improving aesthetic appearance and quality of life.

Crusted demodicosis in an immunocompetent pediatric patient.

Demodicosis refers to the infestation by Demodex spp., a saprophytic mite of the pilosebaceous unit. Demodex proliferation can result in a number of cutaneous disorders including pustular folliculitis, pityriasis folliculorum, papulopustular, and granulomatous rosacea, among others. We report the case of a 7-year-old female presenting with pruritic grayish crusted lesions over her nose and cheeks, along with facial erythema, papules, and pustules. The father referred chronic use of topical steroids. A potassium hydroxide mount of a pustule scraping revealed several D. folliculorum mites. Oral ivermectin (200 µg/kg, single dose) plus topical permethrin 5% lotion applied for 3 consecutive nights were administered. Oral ivermectin was repeated every week and oral erythromycin plus topical metronidazole cream was added. The facial lesions greatly improved within the following 3 months. While infestation of the pilosebaceous unit by Demodex folliculorum mites is common, only few individuals present symptoms. Demodicosis can present as pruritic papules, pustules, plaques, and granulomatous facial lesions. To our knowledge, this is the first reported case of facial crusted demodicosis in an immunocompetent child. The development of symptoms in this patient could be secondary to local immunosuppression caused by the chronic use of topical steroids.

Ulcerated radiodermatitis induced after fluoroscopically guided stent implantation angioplasty.

Cases of radiation-induced skin injury after fluoroscopically guided procedures have been reported since 1996, though the majority of them have been published in Radiology and Cardiology literature, less frequently in Dermatology journals. Chronic radiation dermatitis induced by fluoroscopy can be difficult to diagnose; a high grade of suspicion is required. We report a case of an obese 46-year-old man with hypertension, dyslipidemia, and severe coronary artery disease. He developed a pruritic and painful atrophic ulcerated skin plaque over his left scapula, six months after fluoroscopically guided stent implantation angioplasty. The diagnosis of radiodermatitis was confirmed histologically. We report this case to emphasize the importance of recognizing fluoroscopy as a cause of radiation dermatitis. A good clinical follow-up at regular intervals is important after long and complicated procedures, since the most prevalent factor for injury is long exposure time.

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.