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1.
J Cutan Pathol ; 44(7): 647-654, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28345255

RESUMO

Skin diseases presenting with keratotic papules, atrophy, cicatricial alopecia and/or "lichenoid" histopathologic changes have been described under at least 30 names. This family of diseases contains 2 subgroups, largely based on clinical features: keratosis pilaris atrophicans (KPA; including keratosis pilaris atrophicans faciei/ulerythema ophryogenes, atrophoderma vermiculatum, and keratosis follicularis spinulosa decalvans); and the lichen planopilaris (LPP) subgroup (including LPP, frontal fibrosing alopecia, Graham-Little-Piccardi-Lassueur Syndrome and fibrosing alopecia in a pattern distribution). An interface dermatitis with lichenoid inflammation is characteristic of the LPP group of disorders, but the literature provides scant information about the histopathology of the KPA group. Our experience has been that the 2 subgroups show a unifying histologic similarity as well as considerable clinical overlap. Because these conditions overlap clinically and histologically, we propose that the term lichenoid folliculitis (LF) be used to refer to this group of diseases, thus simplifying cumbersome nomenclature and highlighting the possibility of shared pathogenesis and treatment options.

2.
Cureus ; 16(7): e65668, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-39205768

RESUMO

Keratosis follicularis spinulosa decalvans X-linked (KFSDX) is part of the spectrum of a rare disorder known as keratosis pilaris atrophicans. Here, we report the case of a 14-year-old boy who presented with a history of abnormal hair since birth. He also had a history of skin lesions and hair loss. There was no similar condition in the family, and the parents were not consanguine. Scalp examination revealed woolly hair, a solitary scarring alopecia patch, and follicular papules. There were also patches of scarring alopecia on the lateral portion of the eyebrows and whole eyelashes bilaterally. His nose showed multiple, skin-colored, non-scaly follicular papules. The differential diagnosis included lichen planopilaris, Graham Little-Piccardi-Lassueur syndrome, KFSDX, keratosis follicularis spinulosa decalvans, and structural hair anomalies. Hair examination under light microscopy was normal. Skin biopsy from the follicular papule on the nose revealed follicular plugging with normal epidermis and dermis. Based on the above clinicopathological findings, the patient was diagnosed with KFSDX associated with woolly hair. He was reassured, but he did not show up for further treatment during the follow-up.

3.
Cureus ; 16(4): e58830, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38784309

RESUMO

Keratosis pilaris atrophicans faciei (KPAF) and frontal fibrosing alopecia (FFA) present diagnostic challenges due to their similar clinical characteristics. Dermatologists often employ overlapping treatment regimens, which may hinder accurate diagnosis and treatment expectations. Genetic testing offers promise for precise diagnosis and tailored treatment strategies, yet its utility in these conditions remains underexplored. This manuscript presents a unique case study of a 36-year-old male with symptoms of both KPAF and FFA, who underwent genetic testing. Despite testing negative for this mutation, the case underscores the potential of genetic testing to enhance diagnostic accuracy and optimize treatment outcomes.

4.
Clin Cosmet Investig Dermatol ; 16: 3497-3501, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38077919

RESUMO

Keratosis pilaris atrophicans faciei (KPAF) is a rare, hereditary, follicular disorder categorized in the atrophicans subtypes of keratosis pilaris (KP). Nowadays it can be treated with light and laser devices. Lasers with wavelengths <600 nm, especially pulsed dye laser (PDL), are effective for treatments of KPAF. Here, we present a case with KPAF treated with 585 nm diode laser, a kind of laser system functioning with differential wavelength modified optically pumped semiconductor (D-WMOPS) technology. Our case is the first patient reported to have been treated with this laser technology in the literature.

5.
J Dermatolog Treat ; 33(3): 1231-1242, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-32886029

RESUMO

INTRODUCTION: Keratosis pilaris (KP) is a common, benign skin condition of follicular hyperkeratosis. Although KP is asymptomatic, the cosmetic appearance of KP can lead to psychosocial distress among patients. New emerging treatments are increasingly being utilized. Yet, there is little to no summative data on the treatments of KP and its subtypes. OBJECTIVE: To summarize existing literature on treatments for KP and its subtypes. METHODS: A comprehensive search was performed using Pubmed/MEDLINE, Embase and Web of Science databases. The search identified 1150 non-duplicated articles, and 47 articles were included in the review. The primary outcomes measured were KP treatment type and the degree of improvement following therapy. FINDINGS: Our findings demonstrate that the most supported form of treatment for KP is laser therapy, particularly the QS:Nd YAG laser. Topical treatments - including Mineral Oil-Hydrophil Petrolat, tacrolimus, azelaic acid, and salicylic acid - are also effective at least for improving the appearance of KP. CONCLUSION: While the measured treatment outcomes varied among studies, laser therapy appears to be the most effective form of treatment. Use of topicals also improved KP lesions.


Assuntos
Anormalidades Múltiplas , Doença de Darier , Terapia com Luz de Baixa Intensidade , Anormalidades Múltiplas/terapia , Doença de Darier/terapia , Sobrancelhas/anormalidades , Humanos
6.
Exp Ther Med ; 22(5): 1331, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34630685

RESUMO

Keratosis pilaris atrophicans faciei (KPAF) is a hereditary follicular disorder, an atrophicans subtype of keratosis pilaris (KP) with a highly elusive diagnosis. Clinically, it presents with follicular, horny papules surrounded by an erythematous halo of the cheeks, forehead, chin and eyebrows, and it is followed by a gradual hair loss on the lateral margins of the eyebrows. The onset is as early as a few months after birth, but it is mainly diagnosed in children and adolescents and it can persist through adulthood. At present, the natural progression of the disease is poorly understood, which makes a correct diagnosis highly unlikely. The aim of the present study was to describe the clinical characteristics of KPAF in patients encountered in daily practice, in order to find common characteristics that may aid in the earlier recognition of the disease. An observational, descriptive, retrospective study was performed on 14 patients diagnosed with KPAF between January 2000 and December 2020. The mean age at diagnosis was 17.04 years and the onset of clinical symptoms appeared at a mean age of 4.85 years. The first clinical symptom was KP involving either the upper or lower limbs, or both. Then, erythema of the face appeared at a mean age of 7.21 years, keratotic papules on the face at a mean age of 8.35 years and, finally, loss of hair on the lateral margins of the eyebrows at a mean age of 14 years. The patients also had concomitant xerosis cutis, multiple mole syndrome, acne, contact dermatitis and Laugier-Hunziker syndrome. Evidence of disease progression, associations, as well as efficacious treatment measures are lacking. An earlier diagnosis potentially allows for a more efficacious, targeted treatment option. Either topical emollients, systemic retinoids or laser therapy may prove effective for each patient individually.

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