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.

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

BACKGROUND: 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. METHODS: We evaluated clinical, histopathological, and immunohistochemical findings of a case categorized as idiopathic histiocytoid Sweet syndrome in which clinical-epidemiological data raised the possibilities of Sweet syndrome, leprosy, and drug reaction. RESULTS: Positive reaction to myeloperoxidase (MPO) in histiocytoid cells of the dermal infiltrate, response to oral corticosteroids, clinical and laboratory investigation, and absence of cutaneous lesions or clinical complaints within 1 year of follow-up are consistent with the diagnosis of idiopathic histiocytoid Sweet syndrome. CD68 (PG-M1) and CD15 positive cells were also present among dermal cells. CONCLUSIONS: Epidemiological data are relevant while considering a clinical differential diagnosis of Sweet syndrome that can be further expanded, from a histopathological point of view, when dealing with the histiocytoid variant since neutrophils, macrophages, and immature myelomonocytic cells with histiocytoid morphology are present. The significance of the MPO positive mononuclear dermal cells are not completely established.

Hypopigmented mycosis fungoides: a 20-case retrospective series.

BACKGROUND: 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. METHODS: This is a cross-sectional retrospective observational study, with exploratory analysis. The outcome variables were disease progression and related death. RESULTS: Twenty patients with hMF were selected from a group of 102 patients diagnosed with MF. There was no gender difference (10 females and 10 males). Mean age at diagnosis was 43.85 years, and most patients had mixed or black skin color. The mean time between the onset of the lesions and the diagnosis was 66.75 months. Patients were equally distributed in stages IA (50%) and IB (50%). Photochemotherapy (psoralen and ultraviolet A) was the predominant therapeutic modality. The mean follow-up time was 7.25 years. In 10%, disease progression was observed. Death related to the disease occurred in one patient. CONCLUSIONS: The clinical and epidemiological profile of patients with hypopigmented MF found in our sample is in agreement with what is described in the literature, with the exception of the age at diagnosis, higher than expected. Diagnostic delay time, despite long, is also consistent with the medical literature; however, in this sample, we had two cases of disease progression, with death of one patient, despite the treatment, which is extremely important since hypopigmented MF is usually associated with good prognosis.