Case Series of Frontal Fibrosing Alopecia and Fibrosing Alopecia in a Pattern Distribution: Is There a Familial Correlation?

Introduction: Frontal Fibrosing Alopecia (FFA) and Fibrosing Alopecia in a Pattern Distribution (FAPD) are two distinct entities of cicatricial pattern hair loss that share histological features of perifollicular lichenoid inflammation associated with concentric fibrosis. Although the pathophysiology of FFA and FAPD are still unknown, recently published reports of familial cases indicate a possible genetic correlation. Case Presentation: We report 6 cases of familial alopecia composed of mothers and daughters: five with FFA and one with FAPD. We describe clinical, trichoscopy and histological correlation in cases of familial alopecia. Conclusions: These cases of mother and daughter disease association suggest a potential benefit of and role for performing systematic scalp examinations of all first-degree relatives of patients with pattern cicatricial alopecia.

A pediatric case of disseminated American cutaneous leishmaniasis in Rio de Janeiro, Brazil.

American cutaneous leishmaniasis is an infectious disease caused by the protozoa of the genus Leishmania. Clinical manifestations vary according to the virulence of the parasite speciesand the host's immune response. We report a case of a 2-year-old girl vertically exposed to HIV who presented painful and itchy papules throughout her lower limbs with further dissemination of vegetative ulcers all over the body and scalp. The histopathological examination evidenced the amastigote form of Leishmania and the polymerase chain reaction was positive for Leishmania sp. in the tissue sample. The patient was treated with amphotericin B and demonstrated improvement of lesions. Despite successful treatment for American cutaneous leishmaniasis, she developed osteomyelitis related to a bacterial secondary infection over the site of a previous ulcer on the left ankle and required a 6-week course of intravenous antimicrobial treatment. Children with vertical exposure to HIV, even without seroconversion, are at greater risk of infections if compared to non-exposed children. This is perhaps the reason for such an exuberant and rare case of complicated eishmaniasis.

Comparison of clinical and histologic findings in daylight photodynamic therapy for skin field cancerization: A randomized controlled four-arm study on physical methods-assisted delivery of methyl aminolevulinate.

BACKGROUND: Daylight photodynamic therapy (DL-PDT) has similar efficacy to conventional photodynamic therapy in treating actinic keratosis (AKs). Good clinical outcomes have been reported when associated with physical methods such as microneedles, but a comparison of different methods and histologic studies is lacking. OBJECTIVES: To evaluate clinical and histologic modifications induced by standard DL-PDT and compare with DL-PDT associated with physical methods in treating skin field cancerization of the face. METHODS: Forty patients with photodamaged skin and at least one AK lesion on the face were randomly distributed into four groups, ten patients in each (I: Standard DL-PDT; II: DL-PDT + microneedles; III: DL-PDT + CO2 laser; IV: DL-PDT + microdermabrasion) and underwent two DL-PDT sessions with methyl aminolevulinate cream and 2-hour daylight exposure. Skin biopsies were performed on all patients before and 3 months after. All fragments were stained using the hematoxylin-eosin, orcein, and picrosirius. RESULTS: All 40 patients completed the study. Group III had a higher AK-clearance after 1 (p = 0,002) and 3 (p = 0,034) months, but it was similar in every group at 6 months (p = 0,441). Group III and IV had better clinical global improvement on texture, pigmentation and fine lines. In the groups associated with physical methods, the improvement of the keratinocytes' atypia and solar elastosis were remarkable. Only group III showed a significant reduction in solar elastosis (p = 0.034) and increased collagen type I (p = 0.028) after treatment. CONCLUSION: DL-PDT-associated with physical methods had better clinical and histologic results. AK-clearance were significantly higher after 1 and 3 months with pretreatment-CO2 laser. Photorejuvenation were more evident with pretreatment-CO2 laser and microdermabrasion. Pretreatment-CO2 laser showed a significant reduction in solar elastosis and increase of collagen type 1. These results pointed to the pretreatment with laser as a potentially better option for skin field cancerization of the face.

Pancreatic panniculitis in systemic lupus erythematosus and Crohn's disease: rare event / Paniculite pancreática no lúpus eritematoso sistêmico e na doença de Crohn: raro evento

ABSTRACT Pancreatic panniculitis (PP) is a rare manifestation of pancreatic disease, involving subcutaneous adipose tissue. We report two cases of this entity: a 37-year-old female patient with systemic lupus erythematosus (SLE) and an elderly woman with Crohn's disease. These are two chronic autoimmune inflammatory diseases that can be uncommonly related to acute pancreatitis and that culminates in PP. We also provide a brief review of the treatment, diagnosis and morphology of the lesions, as well as the pathophysiology of the disease. The importance of histopathological analysis of lesion biopsies is highlighted as an important diagnostic tool.

RESUMEN La paniculitis pancreática (PP) es una manifestación rara de enfermedad pancreática que afecta el tejido graso subcutáneo. Reportamos dos casos de esa entidad: una paciente del sexo femenino, 37 años, con lupus eritematoso sistémico (LES), y una anciana con enfermedad de Crohn. Ambas manifestaciones son enfermedades crónicas autoinmunes raramente relacionadas con el cuadro de pancreatitis aguda y que culminaron en PP. También hicimos una breve revisión acerca de tratamiento, diagnóstico y morfología de las lesiones, así como fisiopatología de la enfermedad. Se resalta la importancia de la inmunohistoquímica y del análisis histopatológico de biopsias de la lesión como herramientas diagnósticas.

RESUMO A paniculite pancreática (PP) é uma manifestação rara de doença pancreática que acomete o tecido adiposo subcutâneo. Relatamos dois casos dessa entidade: uma paciente do sexo feminino, 37 anos, portadora de lúpus eritematoso sistêmico (LES) e uma idosa com doença de Crohn. Ambas manifestações são doenças inflamatórias crônicas autoimunes raramente relacionadas com o quadro de pancreatite aguda e que culminaram em PP. Também fizemos uma breve revisão sobre tratamento, diagnóstico e morfologia das lesões, bem como da fisiopatologia da doença. Destacamos a importância da imuno-histoquímica e da análise histopatológica de biópsias da lesão como ferramentas diagnósticas.

Sporotrichosis: A Clinicopathologic Study of 89 Consecutive Cases, Literature Review, and New Insights About Their Differential Diagnosis.

BACKGROUND: Sporotrichosis is the most common and least severe of deep mycoses. This disease has varied clinic presentation as well as several differential diagnosis. METHODS: A cross-sectional and retrospective, individually based, observational study, based on records from the dermatopathology service of a university hospital in Brazil. A total of 175 patients were identified with clinical suspicion of sporotrichosis, from 2009 to 2017. Statistical analysis using prevalence ratios was conducted to characterize the clinicopathologic and epidemiological aspects. RESULTS: The disease was suspected in 175 patients given the clinical presentation. Of these, 86 cases (49.14%) were sporotrichosis, 39 (22.29%) chronic granulomatous inflammatory processes of unidentified etiology, and 5 squamous cell carcinomas (2.86%). The majority of cases (52 or 60.46%) occurred in the upper limbs. A total of 40 samples presented a suppurative chronic granulomatous inflammatory process (46.51%). Only 5 samples (12.50%) showed positive staining (periodic-Schiff acid or GROCOTT). CONCLUSIONS: The sporotrichosis cases underwent both cumulative and linear growth, especially in the group above 60 years. Squamous cell carcinomas appeared several times as a misdiagnosis of sporotrichosis by dermatologists, so it should be considered as an important differential diagnose due to the current context of skin cancers.

Fibrosing Alopecia in a Pattern Distribution (FAPD) in 16 African-Descent and Hispanic Female Patients: A Challenging Diagnosis.

BACKGROUND: Fibrosing alopecia in a pattern distribution (FAPD) has only been described in Caucasian patients, and it is not clear whether it can develop in dark-skin ethnicities. MATERIALS AND METHODS: Sixteen Brazilian female patients, 12 of African descent and 4 Hispanic, with progressive scarring alopecia in a pattern distribution were analyzed. RESULTS: Dermatoscopic features showed perifollicular erythema and scaling (14/16), hair fiber diameter diversity (16/16), loss of follicular ostia (16/16), and follicular keratosis (3/16). Late stages showed a honeycomb pigmented network (12/16), a hyperpigmented perifollicular halo (12/16), and small white patches (12/16). Histopathological features showed lichenoid perifollicular infiltrate (14/16), follicular miniaturization (16/16), concentric fibrosis (16/16), perifollicular lymphocytic infiltrate (16/16), and vellus hair involvement (10/16). Premature desquamation of the inner root sheath was found in 11 patients. CONCLUSIONS: The concomitant findings of cicatricial pattern hair loss (with or without the recess of the front hair line), hair fiber diversity, perifollicular erythema and scaling, a whitish perifollicular halo, and histological findings of androgenetic alopecia, with vacuolar interface alteration of the upper portion of the follicular epithelium, are the main key features to suggest the diagnosis of FAPD. FAPD is a possible diagnosis in patients of color with cicatricial pattern hair loss. Clinical, dermatoscopic, and histopathological examination allow a proper final differential diagnosis.

Lichenoid folliculitis of the scalp in four patients with ichthyosiform skin disorders and cicatricial alopecia.

Ichthyosis is a heterogeneous group of inherited skin disorders characterized by a defect of keratinization. Patients diagnosed with lamellar ichthyosis (LI) and some ichthyosiform syndromes, such as the Conradi-Hünermann-Happle syndrome (CHHS), usually present with hair loss. Even though only few dermatologic complaints carry as many emotional overtones as hair loss, there are very few data available in the literature regarding scalp histopathological features in ichthyosis. A better understanding of scalp changes in such context may result in new therapeutic strategies that in turn would enhance patients' self-esteem and quality of life. The aim of this paper is to describe the scalp histopathological findings of four young patients with cicatricial alopecia: three diagnosed as having LI and the fourth with CHHS.

Brunsting-Perry Type Pemphigoid Causing Secondary Cicatricial Alopecia in 2 Patients.

Brunsting-Perry type pemphigoid (BPP) is a rare subepidermal blistering disease and a cause of secondary cicatricial alopecia. It was originally described by Brunsting and Perry in 1957 as a rare variant of cicatricial pemphigoid, characterized by bullous lesions limited to the head, neck, scalp, and upper trunk with mild or no mucosal involvement. We report 2 cases of BPP cicatricial alopecia with histopathology of subepidermal blister formation, different clinical presentation, and different salt-split test results. One patient had features of bullous pemphigoid (BP) with important oral mucosal involvement (not yet reported in the literature), and the second patient had typical features of epidermolysis bullosa acquisita (EBA). The secondary cicatricial alopecia may be due to different antigens associated with either BP or EBA. The phenomenon of epitope spreading could explain the association between 2 distinctive bullous diseases in the same patient, justifying the divergent findings of the immunofluorescence. The specific target antigen of BPP is yet to be defined.

Hyperpigmented Upper Eyelid: A Clue to the Diagnosis of Facial Lichen Planus Pigmentosus in a Patient with Frontal Fibrosing Alopecia.

Facial lichen planus pigmentosus (LPPig), a rare variant of classic lichen planus, was first described in patients with frontal fibrosing alopecia (FFA) by Dlova [Br J Dermatol 2013; 168: 439-442] in 2013. The diagnosis of facial LPPig is sometimes not easy, since clinical signs and histopathological features may frequently be confused with melasma or postinflammatory hyperpigmentation. We describe a case of a postmenopausal black woman diagnosed with FFA who presented with an identical brown-grayish pigmentation of the face and upper eyelids and typical dermoscopy analysis on both regions. We suggest that the hyperpigmentation of the upper eyelid with typical LLPig dermoscopy (upper eyelid sign) may be a clue for the diagnosis of LPPig and may avoid a scar-causing face biopsy.

Nevus lipomatosus cutaneous superficialis.

Nevus lipomatosus cutaneous superficialis is a rare benign hamartomatous skin tumor characterized by dermal deposition of mature adipose tissue. Two clinical forms have been described (classical and solitary types). We describe a case of nevus lipomatosus cutaneous superficialis with a 13-year history of growth in a young woman who had a solitary skin-colored tumoral mass on the right buttock. Histopathological findings were typical and confirmed the diagnosis. In this case, the lesion was a skin-colored isolated mass, as described in the solitary type, but its localization and age of appearance were compatible with the classical type. The combination of simultaneous clinical findings of both types had not been published before.

Nevus lipomatosus cutaneous superficialis

Abstract: Nevus lipomatosus cutaneous superficialis is a rare benign hamartomatous skin tumor characterized by dermal deposition of mature adipose tissue. Two clinical forms have been described (classical and solitary types). We describe a case of nevus lipomatosus cutaneous superficialis with a 13-year history of growth in a young woman who had a solitary skin-colored tumoral mass on the right buttock. Histopathological findings were typical and confirmed the diagnosis. In this case, the lesion was a skin-colored isolated mass, as described in the solitary type, but its localization and age of appearance were compatible with the classical type. The combination of simultaneous clinical findings of both types had not been published before.

Sweet's syndrome associated with Crohn's disease.

Sweet's syndrome is an uncommon benign skin disorder, whose pathogenesis remains unknown. Its classic form is more common in women and presents itself as papular-nodular, painful and erythematous or violaceous lesions. It mainly affects the face, neck, and upper limbs. Fever and neutrophilic leukocytosis are also common features. Although it is considered a systemic disease marker in more than half of patients, the association of this condition with Crohn's disease is rare, with few cases reported in the literature, of which, none in Brazil. We report the case of a patient with Crohn's disease who developed the classical features of Sweet's syndrome.

Sweet's syndrome associated with Crohn's disease

Abstract: Sweet's syndrome is an uncommon benign skin disorder, whose pathogenesis remains unknown. Its classic form is more common in women and presents itself as papular-nodular, painful and erythematous or violaceous lesions. It mainly affects the face, neck, and upper limbs. Fever and neutrophilic leukocytosis are also common features. Although it is considered a systemic disease marker in more than half of patients, the association of this condition with Crohn's disease is rare, with few cases reported in the literature, of which, none in Brazil. We report the case of a patient with Crohn's disease who developed the classical features of Sweet's syndrome.

Pruritic folliculitis of pregnancy

Abstract Pruritic folliculitis of pregnancy is a rare disease of unknown etiology. It occcurs primarily during pregnancy, usually with spontaneous resolution postpartum. It is characterized by a benign dermatosis, with papular and pustular follicular lesions that first appear on the torso and occasionally spread throughout the body. We report the case of a patient in the 27th week of pregnancy, with a two-month evolution of pruritic and papular erythematous lesions on her lower back. Differential diagnosis includes other pregnancy-specific dermatoses: gestational pemphigoid, pruritic urticarial papules and plaques of pregnancy (PUPPP), prurigo of pregnancy, and (PUPPP) and prurigo of pregancy. Histopathological tests showed changes consistent with pruritic folliculitis of pregnancy. This case is relevant due to its rare nature and its clinical and histopathological characteristics.

Lesões vesiculares e prurido intenso em paciente com sífilis secundária:manifestação clínica atípica.

Syphilis is an infectious disease with mucocutaneous lesions that are characteristic of their stage (primary, secondary, or tertiary). These are not always typical and may have other morphologies and unusual symptoms, making diagnosis difficult. The report herein is of a healthy woman with vesicular lesions associated with intense itching.  The diagnosis of secondary syphilis was confirmed with VDRL 1/512 and FTA Abs positive.  Histopathology showed spongiosis in the epidermis and infiltrated lymphocytes and plasma cell in the dermis.  Clinical improvement occurred after the first dose of benzathine penicillin. In the literature, few cases of vesicular lesions in syphilis have been found and itching is mentioned as uncharacteristic . Histology showing spongiosis is also unusual.

Scleredema of Buschke associated with difficult-to-control type 2 diabetes mellitus.

Scleredema of Buschke (SB) is a rare disorder of connective tissue characterized by diffuse non-pitting induration of the skin, mainly on the cervical, deltoid and dorsal regions. It is a cutaneous mucinosis of unknown etiology and is associated with bacterial or viral infections, hematological disorders and diabetes mellitus. Histopathological examination shows thickened dermis with wide collagen bundles separated by gaps that correspond to mucopolysaccharide deposits, visualized using special staining. Several treatments are reported in the literature without well-established results. We report a case of SB in a patient with type 2 diabetes mellitus.

Scleredema of Buschke associated with difficult-to-control type 2 diabetes mellitus / Escleredema de Buschke associado a diabetes mellitus tipo 2 de difícil controle

SUMMARY Scleredema of Buschke (SB) is a rare disorder of connective tissue characterized by diffuse non-pitting induration of the skin, mainly on the cervical, deltoid and dorsal regions. It is a cutaneous mucinosis of unknown etiology and is associated with bacterial or viral infections, hematological disorders and diabetes mellitus. Histopathological examination shows thickened dermis with wide collagen bundles separated by gaps that correspond to mucopolysaccharide deposits, visualized using special staining. Several treatments are reported in the literature without well-established results. We report a case of SB in a patient with type 2 diabetes mellitus.

RESUMO Escleredema de Buschke (EB) é doença rara do tecido conjuntivo caracterizada por endurecimento difuso e não depressível da pele, principalmente nas regiões cervical, deltoideanas e dorso. Enquadrado no grupo das mucinoses cutâneas, tem etiologia desconhecida e associação com: infecções bacterianas ou virais, alterações hematológicas e diabetes mellitus. O exame histopatológico evidencia derme espessada com fibras colágenas calibrosas separadas por fendas que correspondem a depósito de mucopolissacárides, observados por colorações especiais. Diversos tratamentos são relatados na literatura sem resultados bem definidos. Descrevemos caso de EB em paciente com diabetes mellitus tipo 2.