Danielle Carvalho Quintella

Successful Treatment of Facial Papules in Frontal Fibrosing Alopecia with Oral Isotretinoin

licular inflammation. Our findings include variable vellus hair follicle involvement, elastic fiber involvement, and preserved sebaceous glands ( Fig. 1 ) (unpublished data). Due to the effects of oral isotretinoin over sebaceous glands and previous studies reporting a possible role on the therapeutic regimen of FFA [5, 6] , we opted to use this drug in the treatment of 3 patients with FFA with prominent facial papules. All patients were female, biopsy-confirmed cases of FFA, with the age range 49–53 years. Clinical information, concurrent, and previous treatments are presented in Table 1 . All patients were started on the dosage of 20 mg/day of oral isotretinoin for the first month, which was then titrated to 0.5 mg/kg/day for the following 2 months (40 mg/day in all 3 patients). The patients were seen monthly for isotretinoin laboratory monitoring and, at the end of the third month, on a visit scheduled to evaluate FFA treatment efficacy. At the end of the first month, a remarkable improvement was noted, with a reduction in the number and size of facial papules. Patients reported perceiving that by the second week of treatment, the aspect of their facial skin had already considerably improved. At the end of the third month, facial papules had completely disappeared or were considered minimal ( Fig. 2 ). The patients did not show a significant progression of FFA, and all reported decreased pruritus. However, signs of disease activity were still present over the hairline in all 3 patients, namely perifollicular erythema and scaling. Isotretinoin was then discontinued and hydroxychloroquine 400 mg/day was introduced. Facial papules are a characteristic feature of FFA, being present in 14% of the patients, according to a large multicenter study [7] . Dear Editor, Frontal fibrosing alopecia (FFA) is considered a variant of lichen planopilaris affecting mainly postmenopausal women. A progressive frontal or frontotemporal symmetric band of alopecia is the usual presentation, with eyebrows being commonly involved [1] . Currently, several authors consider FFA as a generalized skin condition, and many features other than hair loss have been associated with the disease, including facial papules [2] . Facial papules as a sign of facial vellus hair follicle involvement in FFA was first recognized by Abbas et al. [3] in 2007 and was later confirmed by Donati et al. [4] . In a current ongoing study, our group observed that histopathological features of facial papules might not be limited to perifolReceived: February 6, 2017 Accepted: February 20, 2017 Published online: March 21, 2017

Facial Papules in Frontal Fibrosing Alopecia: Beyond Vellus Hair Follicle Involvement

Background: Frontal fibrosing alopecia (FFA) is considered a variant of lichen planopilaris affecting mainly the frontotemporal hairline. Since the first report in 1994, several other clinical features have been associated with the disease, such as facial papules (FP). Even though FP have been linked to facial vellus hair follicle involvement, how this finding alone could lead to the formation of clinically evident FP in FFA patients had not yet been addressed. Objective: To describe histopathological findings of FP in the context of FFA and to highlight features that may be linked to their clinical formation. Methods: Cutaneous FP biopsies of FFA patients performed between January 2016 and May 2017 were retrieved from our pathology database and reexamined by 2 pathologists. Results: Histological sections of thirteen 3.0-mm punch biopsy specimens (2 horizontally and 11 vertically oriented) were collected from 7 patients. Eleven specimens demonstrated prominent sebaceous glands and 10 dilated sebaceous ducts. Pinkus acid orcein staining revealed reduction and fragmentation of the elastic fibers in 12 samples and, in 7 of these, this finding was observed in both the papillary and reticular dermis, particularly around sebaceous lobules. Vellus hair follicle involvement was only seen in 2 samples. Conclusions: Prominent sebaceous lobules with dilated ducts associated with an abnormal elastic framework seem to be the main explanation for the formation of FP in the context of FFA.

Histopathology of facial papules in frontal fibrosing alopecia and therapeutic response to oral isotretinoin

To the Editor:We readwith great interest that Pedrosa et al were able to reproduce our findings regarding the histology of facial papules (FP) in frontal fibrosing alopecia (FFA) and the therapeutic response to isotretinoin. Our group first reported that histopathologic features of FPwere not limited to perifollicular inflammation, as previously described, and that structural changes involving elastic fibers and sebaceous glands could be responsible for the clinical formation of FP. Influenced by our observations, we performed a proof-of-concept study in which FP in a series of patients with FFA were successfully treated with oral isotretinoin. We wish to highlight some aspects and differences between our previously published observations and Pedrosa’s work. While Pedrosa et al hypothesize that facial papules result from hypertrophic sebaceous glands that shine through soft and thin skin, we presented a distinct explanation that was later supported by a subsequent study from our group. Besides prominent sebaceous glands and dilated sebaceous ducts, pinkus acid orcein staining showed a reduction and fragmentation of elastic fibers in most of our samples. For this reason, we proposed that an abnormal elastic framework could be responsible for the remodeling of the shape of sebaceous lobules and ducts in this anatomic microenvironment, leading to the popping out of sebaceous glands and the clinical formation of FP. Although an overlying atrophic epidermis could possibly highlight the presence of FP, our findings suggest that the pathogenesis of FP is not limited to this aspect. In addition, epidermal atrophy was present in only 5 out of 13 of the studied specimens in our study. Another interesting point is that the choice of therapeutic regimen seems to affect the time-toresponse with oral isotretinoin. While in our study (isotretinoin dosage 20 mg/day for the first month, then titrated to 0.5 mg/kg/day for the following 2 months) patients reported remarkable improvement by the second week of treatment, patients treated with 10 mg of isotretinoin every other day

Ingrown Hairs: A Recurrent Trichoscopic Feature in Scarring Alopecias

Tullia Cuzz. Fundacao Oswaldo Cruz. Instituto Nacional de Infectologia Evandro Chagas. Documento produzido em parceria ou por autor vinculado a Fiocruz, mas nao consta a informacao no documento.

Treatment of intralesional corticoisteroid-induced cutaneous atrophy with hyaluronic acid filling

alopecia localized to the frontal and temporal hairline as a band with atrophic and shiny skin. Follicular keratinization and perifollicular erythema were present. We prescribed treatment with oral dutasteride 0,5mg daily combined with 5% minoxidil lotion. We infiltrated intralesional corticosteroids along the line of frontal hair implantation. Injections were performed with triamcinolone acetonide 0,1ml diluted in 0,4 lidocaine 1%. We administered 0,03ml per point following a headband distribution. The patient attends a week later for the appearance of depressions located in sites of corticosteroid injections. We refilled the cutaneous atrophy by infiltrating hyaluronic acid (Belotero Soft®) with subcutaneous micro depot technic, obtaining excellent results in only one session (Figures 1) (Figures 2).

Hypopigmented mycosis fungoides: a 20-case retrospective series

Hypopigmented mycosis fungoides (hMF) is a rare subtype of mycosis fungoides. The aim of this study was to identify the clinical–epidemiological profile of our patient group and also to provide additional information about treatment responses and prognosis.

Idiopathic histiocytoid Sweet syndrome: a case report with clinical and histopathological considerations

Histiocytoid Sweet syndrome is characterized by a predominant neutrophilic dermal infiltrate. Usual clinical differential diagnosis includes erythema multiforme, drug eruption, and erythema nodosum. Histiocytoid Sweet syndrome is considered an uncommon histopathological variant of the disease.

Folliculitis spinulosa decalvans: case report and response to isotretinoin and acitretin

Keratosis pilaris atrophicans represents a group of rare diseases characterized by keratosis pilaris on the extensor surfaces, inflammation and atrophic scarring. The keratosis pilaris atrophicans spectrum includes ulerythema ophryogenes, atrophoderma vermiculatum and keratosis follicularis spinulosa decalvans. Folliculitis spinulosa decalvans is considered a variant of keratosis follicularis spinulosa decalvans, presenting with persistent pustules, keratotic papules and scarring alopecia. The treatment is challenging, with few cases reported in the literature. We report the case of a 20-year-old man with folliculitis spinulosa decalvans since childhood, which had worsened in the past 5 years, with frequent recurrences and a poor response to isotretinoin but with some improvement after acitretin.

Clinical and epidemiological profile of patients with early stage mycosis fungoides

Background Mycosis fungoides is the most common form of primary cutaneous lymphoma, with an indolent, slowly progressive course and 88% five-year survival rate. The diagnosis is challenging, especially in the early stages, and usually relies on a good clinical-histopathological correlation. Objective The aim was to establish the clinical and epidemiological profile of patients with early-stage mycosis fungoides. Methods This was a retrospective cross-sectional observational study with an exploratory analysis. Outcome variables were disease progression and mycosis fungoides-related death. Results One hundred and two patients were included. The majority were white males, with a mean age of 55.6 years. Mean time from onset of lesions to diagnosis was 51.08 months. The majority of patients were classified as IB stage according to TNMB. Mean follow-up time was 7.85 years. Disease progression was seen in 29.4% of the patients. Death related to the disease occurred in 7.9% of patients. Plaque lesions, involvement of more than 10% of the body surface, altered lactate dehydrogenase and beta-2-microglobulin, and stage IB were significantly associated with disease progression, and altered lactate dehydrogenase and beta-2-microglobulin also correlated with higher frequency of deaths. Study limitations Small sample and retrospective design. Conclusions The clinical and epidemiological profile of patients with early-stage mycosis fungoides in our sample corroborates reports in the literature. Diagnostic delay in our series is also consistent with previous findings, but the rate of disease progression, despite treatment, was higher than reported in the literature.BACKGROUND Mycosis fungoides is the most common form of primary cutaneous lymphoma, with an indolent, slowly progressive course and 88% five-year survival rate. The diagnosis is challenging, especially in the early stages, and usually relies on a good clinical-histopathological correlation. OBJECTIVE The aim was to establish the clinical and epidemiological profile of patients with early-stage mycosis fungoides. METHODS This was a retrospective cross-sectional observational study with an exploratory analysis. Outcome variables were disease progression and mycosis fungoides-related death. RESULTS One hundred and two patients were included. The majority were white males, with a mean age of 55.6 years. Mean time from onset of lesions to diagnosis was 51.08 months. The majority of patients were classified as IB stage according to TNMB. Mean follow-up time was 7.85 years. Disease progression was seen in 29.4% of the patients. Death related to the disease occurred in 7.9% of patients. Plaque lesions, involvement of more than 10% of the body surface, altered lactate dehydrogenase and beta-2-microglobulin, and stage IB were significantly associated with disease progression, and altered lactate dehydrogenase and beta-2-microglobulin also correlated with higher frequency of deaths. STUDY LIMITATIONS Small sample and retrospective design. CONCLUSIONS The clinical and epidemiological profile of patients with early-stage mycosis fungoides in our sample corroborates reports in the literature. Diagnostic delay in our series is also consistent with previous findings, but the rate of disease progression, despite treatment, was higher than reported in the literature.

Evaluation of the Cutaneous Lymphoma International Prognostic Index in patients with early stage mycosis fungoides

Background Mycosis fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma. TNMB system is the staging method used in MF, and it not only guides therapeutic management, but represents the main prognostic factor. In order to improve the prognostic evaluation, the Cutaneous Lymphoma International Prognostic Index (CLIPi) was proposed. Objective To evaluate the performance of CLIPi score for prognostic analysis in patients with early stage MF. Methods This is a retrospective cross-sectional observational study, with exploratory analysis. The outcome variables were disease progression and related death. Results One hundred and two patients were stratified according to CLIPi score, being the majority classified as low risk. Patients with intermediate or high risk presented disease progression more frequently than those with low risk (PR: 1.2 / p = 0.004 / 95%CI: 1.0 - 1.6). The same did not occur with the variable related death. In addition, survival rates were not consistent with risk stratification. Study Limitations Small sample and its retrospective analysis. Conclusions Since CLIPi score was proposed, four other studies that we could consult showed conflicting results, similar to the present study. Further studies are necessary for a recommendation of its use.BACKGROUND Mycosis fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma. TNMB system is the staging method used in MF, and it not only guides therapeutic management, but represents the main prognostic factor. In order to improve the prognostic evaluation, the Cutaneous Lymphoma International Prognostic Index (CLIPi) was proposed. OBJECTIVE To evaluate the performance of CLIPi score for prognostic analysis in patients with early stage MF. METHODS This is a retrospective cross-sectional observational study, with exploratory analysis. The outcome variables were disease progression and related death. RESULTS One hundred and two patients were stratified according to CLIPi score, being the majority classified as low risk. Patients with intermediate or high risk presented disease progression more frequently than those with low risk (PR: 1.2 / p = 0.004 / 95%CI: 1.0 - 1.6). The same did not occur with the variable related death. In addition, survival rates were not consistent with risk stratification. STUDY LIMITATIONS: Small sample and its retrospective analysis. CONCLUSIONS Since CLIPi score was proposed, four other studies that we could consult showed conflicting results, similar to the present study. Further studies are necessary for a recommendation of its use.