Hamartoma folicular basaloide / Basaloid follicular hamartoma

O hamartoma folicular basaloide (HFB) é um tumor anexial raro e benigno, que se assemelha ao carcinoma basocelular (CBC), e pode apresentar manifestações clínicas diversas. Uma mutação no gene PTCH, envolvido na síndrome de Gorlin-Goltz, poderia estar associada à patogênese dessa neoplasia. Descreve-se caso de menina, sete anos, apresentando múltiplas pápulas na face.

Basaloid follicular hamartoma (BFH) is a rare and benign adnexal tumor that resembles basal cell carcinoma (BCC) and may present with different clinical manifestations. A mutation in the PTCH gene, involved in Gorlin-Goltz syndrome, could be associated with the pathogenesis of this neoplasm. We describe the case of a 7-year-old girl with multiple papules on her face.

Lupus miliaris disseminatus faciei: when a rare skin disease involves the eyelid / Lupus miliaris disseminatus faciei: quando uma rara dermatose acomete a pálpebra

ABSTRACT The authors present a case of lupus miliaris disseminatus faciei , a rare skin disease of unknown etiology, which may cause unaesthetic scarring due to its difficult treatment. The histopathological examination of epithelioid granulomas with caseating necrosis, together with the clinical features, are important for diagnosis and early treatment with better results. Despite difficult and unsatisfactory treatment, there are ongoing studies on therapy to improve aesthetic and social impairment. This case report describes an initial misdiagnosis delaying appropriate treatment, and highlights the value of physical examination and clinical judgment for another pathological examination, whenever necessary, aiming at better treatment outcomes in daily practice.

RESUMO Os autores apresentam um caso de lupus miliaris disseminatus faciei , uma dermatose rara, de etiologia desconhecida, que pode deixar cicatrizes não estéticas, pela dificuldade de tratamento. O exame histopatológico de granulomas compostos por células epitelioides, com necrose caseosa, e as características clínicas, são importantes para o diagnóstico e tratamento precoce, com melhores resultados. Apesar do tratamento difícil e insatisfatório, há estudos em andamento sobre terapias para melhorar o comprometimento estético e social. Este relato de caso descreve um diagnóstico inicial errôneo, que atrasou o tratamento adequado, e destaca o valor do exame físico e raciocínio clínico para solicitar outro exame anatomopatológico, quando necessário, de forma a obter melhores desfechos com o tratamento, na prática diária.

A case of facial atrophic sarcoidosis in an adolescent, successfully treated with the combination of prednisone and hydroxychloroquine

Abstract Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology. Cutaneous involvement occurs in up to 30% of patients and skin findings are often the initial presenting symptom. The facial atrophic form of sarcoidosis without associated ulceration in adolescents has rarely been described in the literature. We report a case of 13-year-old male patient with a facial atrophic sarcoidosis who was successfully treated with the combination of prednisone and hydroxychloroquine.

Demodex folliculorum infestations in common facial dermatoses: acne vulgaris, rosacea, seborrheic dermatitis

Abstract Background: Demodex mites are found on the skin of many healthy individuals. Demodex mites in high densities are considered to play a pathogenic role. Objective: To investigate the association between Demodex infestation and the three most common facial dermatoses: acne vulgaris, rosacea and seborrheic dermatitis. Methods: This prospective, observational case-control study included 127 patients (43 with acne vulgaris, 43 with rosacea and 41 with seborrheic dermatitis) and 77 healthy controls. The presence of demodicosis was evaluated by standardized skin surface biopsy in both the patient and control groups. Results: In terms of gender and age, no significant difference was found between the patients and controls (p > 0.05). Demodex infestation rates were significantly higher in patients than in controls (p = 0.001). Demodex infestation rates were significantly higher in the rosacea group than acne vulgaris and seborrheic dermatitis groups and controls (p = 0.001; p = 0.024; p = 0.001, respectively). Demodex infestation was found to be significantly higher in the acne vulgaris and seborrheic dermatitis groups than in controls (p = 0.001 and p = 0.001, respectively). No difference was observed between the acne vulgaris and seborrheic dermatitis groups in terms of demodicosis (p = 0.294). Study limitations: Small sample size is a limitation of the study. The lack of an objective scoring system in the diagnosis of Demodex infestation is another limitation. Conclusion: The findings of the present study emphasize that acne vulgaris, rosacea and seborrheic dermatitis are significantly associated with Demodex infestation. Standardized skin surface biopsy is a practical tool in the determination of Demodex infestation.

Reporte de caso de granuloma facial, una vasculitis cutánea infrecuente / A case report of granuloma faciale, an uncommon cutaneous vasculitis

El granuloma facial es una dermatosis benigna poco frecuente de etiología desconocida, generalmente asintomática, caracterizada por inflamación crónica y localizada habitualmente en zonas fotoexpuestas de la cara, con un patrón histológico característico. Si bien la respuesta al tratamiento es variable, existen múltiples opciones terapéuticas que han reportado ser efectivas en algunos pacientes. Entre estas alternativas se incluyen tratamientos sistémicos con corticoides y dapsona o tratamientos intralesionales con corticoides, crioterapia e inhibidores de calcineurina tópicos. Describimos el caso de un paciente adulto con una placa eritemato violácea asintomática en mejilla derecha, de crecimiento lentamente progresivo de dos años de evolución, clínica e histopatológicamente compatible con granuloma facial y respuesta favorable a corticoides intralesionales.

Granuloma faciale is an uncommon benign dermatosis, with unknown etiology, usually asymptomatic, characterized by chronic inflammation localized in sun-exposed areas of the face with a characteristic histological pattern. Although response to treatment is variable, there are multiple therapeutic alternatives that have been reported to be effective in some patients, including systemic treatments with steroids and dapsone or also topical treatments like intralesional corticosteroid, cryotherapy and calcineurin inhibitors, such as tacrolimus. We present the case of an adult patient with an asymptomatic erythematous-violet plaque on the right cheek, with progressive slow growth over two years, clinically and histologically and pathologically compatible with a facial granuloma. The patient responded well to intralesional corticosteroids.

Dermatosis neutrofílica de las manos y rostro posterior a mordedura de perro: reporte de caso y revisión de la literatura / Neutrophilic dermatosis of the hands and face after dog bite: case report and literature review

La Dermatosis neutrofílica de las manos es consi-derada una variante localizada acral del Síndrome de Sweet, más frecuente en mujeres y principal-mente asociada a enfermedades hematológicas. Las lesiones aparecen como pápulas, vesículas, nó-dulos, placas, úlceras y ampollas, principalmente en el dorso de las manos. Aproximadamente la mi-tad de los pacientes presenta fenómeno de patergia como factor desencadenante.En el presente caso clínico se describe una derma-tosis neutrofílica de las manos posterior a morde-dura de perro, asociado a mielofibrosis primaria y desarrollo de lesiones faciales.

Neutrophilic dermatosis of the hands is conside-red an acral localized variant of Sweet Syndrome, more frequent in women and mainly associated with hematological diseases. The lesions appear as papules, vesicles, nodules, plaques, ulcers, and blisters, mainly on the back of the hands. Appro-ximately half of the patients present a phenome-non of pathergy as a triggering factor. Herein we describe a case of neutrophilic dermatosis of the hands after a dog bite, associated with primary myelofibrosis and development of facial lesions.

PLECT or PPLECT? Granulomatous pyoderma gangrenosum in the differential diagnosis of the verrucous syndrome

Abstract: Pyoderma gangrenosum (PG) is an uncommon neutrophilic dermatosis, with variable clinical features, nonspecific histopathology and multifactorial pathogenesis, posing a challenging diagnosis for the dermatologist. Pyoderma gangrenosum is a diagnosis of exclusion and should be included in the differential diagnoses of the verrucous syndrome. We report a granulomatous variant affecting the face.

Tricodisplasia espinulosa asociada a síndrome mielodisplásico e inmunosupresión / Trichodysplasia spinulosa: report of one case

Trichodysplasia spinulosa is a rare disease that occurs in the setting of immunosuppression, associated with tolerogenic therapy used in allograft recipients or patients with hematologic malignancies. Clinically, it is characterized by a centrofacial cutaneous eruption of erythematous papules with a central keratinous spicule, often associated with variable degrees of alopecia. Histologic findings are characteristic, and electron microscopy reveals the presence of trichodysplasia spinulosa associated polyomavirus. We report a 47-year-old woman with idiopathic autoimmune pancytopenia refractory to diverse immunosuppressant regimens, with clinical and pathologic findings compatible with the disease, in whom complementary studies were required to exclude other differential diagnoses.

Morbihan disease: a therapeutic challenge

Abstract: Morbihan disease is a rare condition characterized by chronic and persistent erythematous solid edema localized on the face. It is believed to be a complication of rosacea and may occur at any stage of the disease. Features of this condition include variable therapeutic response and great refractoriness. We report a case of a 61-year-old man with rosacea history diagnosed with Morbihan disease, who showed excellent therapeutic response with the combination of deflazacort and oral isotretinoin but developed recurrence after corticosteroid discontinuation. We believe that in severe cases of lymphedema of the face this combination is effective and corticosteroid suspension should be done slowly and gradually.

Lupus miliaris disseminatus faciei

Abstract: Lupus miliaris disseminatus faciei is a rare inflammatory dermatosis of unknown etiology that primarily affects young adults. Clinically, it is characterized by an asymptomatic papular eruption mainly involving the central face, typically on and around the eyelids. Characteristic histopathological features include dermal epithelioid cell granulomas with central necrosis and surrounding lymphocytic infiltrate with multinucleate giant cells. Lupus miliaris disseminatus faciei has a spontaneously resolving course, yet can be cosmetically debilitating given the location and potential for scarring. Treatment is difficult and there is a lack of controlled studies. We report a new case of lupus miliaris disseminatus faciei successfully treated with minocycline and systemic steroids, and briefly discuss its nosology and therapeutic options.

Solitary ulcerated plaque on the face - an unusual presentation of cutaneous plasmacytosis?

Abstract Cutaneous and systemic plasmacytosis is a rare disorder characterized by cutaneous polyclonal plasma cell infiltration frequently associated with polyclonal hypergammaglobulinemia and lymphadenopathy. We report a case of a 67-year-old woman with an inflammatory ulcerated plaque in the left masseter region. A skin biopsy showed dense perivascular infiltrate of mature plasma cells in the dermis without atypia and immunoglobulin light chain restriction. After physical examination and further investigation, we ruled out systemic disease. Our patient was successfully treated only with hydrocortisone cream application. Few cases of isolated benign primary cutaneous plasmacytosis have been described, particularly in children. After excluding the diagnosis of a reactive process to an infection, which is unlikely in this case, we suspected of a rare manifestation of primary cutaneous plasmacytosis in adults with distinct presentation and clinical course.

Trigeminal trophic syndrome simulating rodent ulcer basal cell carcinoma: a new clinico-dermoscopic approach

Abstract Trigeminal trophic syndrome is an uncommon cause of facial ulcers, that affects the sensitive area of the trigeminal nerve. We present the case of an 84-year-old patient with ulcerated facial trigeminal trophic syndrome, and report the development of a clinico-dermoscopic approach for his clinical examination. The value of this model for the diagnosis of facial ulcers suspected to be a rodent ulcer basal cell carcinoma is suggested.

Red nodule on the face with "spontaneous" regression

Abstract Pseudolymphomatous folliculitis is a rare entity included among the cutaneous pseudolymphomas. A 32-year-old man, with an unremarkable medical history, presented with a two-month history of an asymptomatic solitary nodule on his left cheek. Histopathological examination demonstrated a dense nodular and diffuse dermal lymphocytic infiltrate with numerous histiocytes and dendritic cells that surrounded hypertrophic hair follicles. Pseudolymphomatous folliculitis commonly presents in the fourth decade of life, with no sex predominance, as an asymptomatic, rapidly growing and solitary red dome-shaped nodule on the face. It has a benign clinical course as the lesions usually resolve with surgical excision or regress spontaneously after incisional biopsy. Although there is no report of pseudolymphomatous folliculitis progressing into lymphoma in the literature, follow-up of these patients is recommended.

Tinea faciei on the right eyebrow caused by Trichophyton interdigitale

Abstract Tinea faciei is a relatively uncommon dermatophyte infection entailing atypical clinical symptoms, usually misdiagnosed and treated with corticosteroids. The authors describe a case of tinea faciei on the right eyebrow caused by Trichophyton interdigitale. The patient was an 18-year-old girl, who had an inflammatory plaque with a scaly, pustular surface on the right eyebrow and upper eyelid, which had persisted for over 1 month. She was once misdiagnosed as having eczema and was treated using corticosteroid cream. A diagnosis of tinea faciei was made based on direct microscopy and culture. The sequencing of the nuclear ribosomal ITS region and β-tubulin gene of the isolate established its T. interdigitale lineage. The patient was cured by treatment with systemic terbinafine in combination with topical application of 1% naftifine-0.25% ketaconazole cream for 2 weeks.

Granuloma faciale: clinical, morphological and immunohistochemical aspects in a series of 10 patients

Abstract Granuloma faciale is a chronic, benign, cutaneous vasculitis with well-established clinical and morphological patterns, but with an unknown etiology. This study describes clinical and pathologic aspects of patients diagnosed with granuloma faciale. The authors analyzed demographic, clinical, morphological and immunohistochemical data from patients with a final diagnosis of granuloma faciale, confirmed between 1998 and 2012. There was a proportional and mixed inflammatory infiltrate, Grenz zones were present in almost all the samples. Immunophenotyping confirmed a higher intensity of T lymphocytes than B lymphocytes in thirteen samples, with a predominance of T CD8 lymphocytes in 64% of cases, in contrast to the literature, which indicates that the major component is T CD4 lymphocytes. All cases were positive for IgG4 but the majority (12/14) had less than 25% of stained cells. The pathogenesis of granuloma faciale remains poorly understood, making studies of morphological and immunohistochemical characterization important to better understand it.

Coexistence of nail lichen planus and lichen planus pigmentosus

Abstract We describe a fifty-six-year old, Afro-descendent female patient showing dystrophy of her twenty nails and hyperchromic, asymptomatic macule on her face. Histopathological examination of the macule showed vacuolization of the basal layer, melanophages in the superficial dermis and lymphoplasmocytic inflammatory infiltrate. Nail biopsy revealed orthokeratotic hyperkeratosis and lichenoid inflammatory infiltrate. Lichen planus pigmentosus is an uncommon variety of lichen planus. It is characterized by typical hyperpigmented macules on the face and neck. Nail changes might be present in 10% of lichen planus cases, but no associations with lichen planus pigmentosus have been described. We report a case of lichen planus in twenty nails associated with lichen planus pigmentosus on the patient's face.

Milia-like calcinosis cutis in a girl with Down syndrome

Abstract: Milia-like idiopathic calcinosis cutis (MICC) is a very rare dermatological disorder characterized by multiple whitish to skin colored, milia-like papules, mostly found on the hands. MICC can disappear spontaneously by adulthood; therefore, its early recognition is crucial to avoiding unnecessary interventions. Herein, we present a case of MICC in a 6-year-old girl with Down syndrome.

Histochemical and immunohistochemical differences between solitary oral fibroma and fibrous papule of the face

Abstract: Background: The morphological similarities between fibrous papules of the face and multiple sporadic oral fibromas were mentioned long ago and a relationship between them has been reported in the literature. Objective: The aim of this study was to evaluate the participation of mast cells, elastin and collagen in a series of oral fibromas and fibrous papules of the face in order to better understand the possible role of these factors in fibrosis and the formation of these lesions. Methods: Thirty cases of oral fibroma involving the buccal mucosa and 30 cases of fibrous papules of the face were selected. Tissue samples were submitted to picrosirius red staining and immunohistochemistry using anti-elastin and anti-tryptase antibodies. Results: The percentage of tryptase-positive mast cells and expression of elastin were higher in cases of fibrous papules of the face (p < 0.05). In contrast, a higher intensity of collagen deposition was observed in oral fibromas. The results showed mast cell accumulation and higher elastin synthesis in fibrous papules of the face, and mast cell accumulation with higher collagen fiber synthesis in oral fibromas. Conclusion: These findings support the hypothesis that mast cells influence the development and growth of these lesions through different mechanisms.