Bullous Pemphigoid in a Patient with Longstanding Hailey-Hailey Disease Complicated by Eczema Herpeticum: Managing the Coexistence of Three Different Blistering Conditions.

Hailey-Hailey disease (HHD), or chronic benign familial pemphigus, is a rare inherited acantholytic dermatosis, characterized by chronic, recurrent vesicles, erosions, and maceration in intertriginous sites. We present a case of a male patient with longstanding HHD who presented with an acute exacerbation characterized by the worsening of pre-existing lesions but also with the appearance of new large, tense bullae on an erythematous base in the areas of the groin (i.e., inguinal region), trunk, and arms, associated with intense pruritus. Blood work revealed eosinophilia. Histopathology and direct immunofluorescence were compatible with the diagnosis of bullous pemphigoid (BP). Indirect immunofluorescence showed positivity for autoantibodies to BP antigen 180. We started oral methylprednisolone, oral antihistamines, and local care with potassium permanganate baths, a potent corticosteroid, and fusidic acid, with resulting improvement of the lesions. The case was further complicated by the occurrence of eczema herpeticum, which was successfully treated with acyclovir. At the time of discharge from the hospital, the patient was medicated with a low dose of oral steroid and oral doxycycline. During a later examination, the lesions had totally disappeared, but the skin had some residual hyperpigmented patches and excoriated papules. This case was a diagnostic challenge due to the simultaneous occurrence of three distinct bullous diseases with different etiopathogeneses. To our knowledge, there are no other reports of the coexistence of HHD and BP in the literature.

The clinical spectrum of dermatomyositis and the new autoantibodies - with regard to two clinical cases with distinct associations.

Dermatomyositis is a rare idiopathic inflammatory myopathy associated with different autoantibodies (anti-MDA5, anti-TIF1-γ) which are linked with typical and distinct phenotypes of dermatomyositis. We describe two cases that illustrate these diverse cutaneous and systemic manifestations.

Elephantiasis nostras verrucosa in the setting of lymphoedema tarda and neurofibromatosis type 1.

Elephantiasis nostras verrucosa (ENV) comprises an uncommon skin disease characterised by dermal fibrosis with hyperkeratotic, verrucous and papillomatous lesions that usually occur after chronic lymphoedema. We describe the case of a 56-year-old-man with neurofibromatosis type 1, no known surgical history, no chronic medication and no travel history, presenting with worsening non-pitting oedema and impressive foul-smelling mossy plaques and cobblestone-like nodules in both legs and feet, especially on the right, compatible with the diagnosis of ENV.

UVA1 for diffuse cutaneous systemic sclerosis in a Fitzpatrick skin type VI patient: outcomes in the modified Rodnan skin score.

INTRODUCTION: Cutaneous sclerosis can lead to important mobility impairment. Ultraviolet (UV) A1 phototherapy may improve skin sclerosis, although most of the studies have been with Caucasian patients. MATERIAL AND METHODS: A 44-year-old patient, Fitzpatrick skin type VI, was being followed up with the diagnosis of diffuse cutaneous systemic sclerosis. He had significant mobility impairment, especially of the right hand and arm. In 2015 he started UVA1 phototherapy daily, Monday until Friday (Waldmann® 7001 UVA cabin equipped with 40 Philips TL/10R lamps - spectral irradiation between 340 and 400 nm). The initial dose was 10 J/cm2, rapidly increased up to a steady dose of 35 J/cm2. RESULTS: After 40 sessions of UVA1, active fingers flexion and abduction of the right arm significantly improved and the modified Rodnan skin score changed from 26 to 11. CONCLUSION: The modified Rodnan skin score is a practical and useful tool during the follow-up of patients with systemic sclerosis. UVA1 phototherapy improves cutaneous sclerosis, and the related mobility impairment, and a dose of 35 J/cm2 is effective, even in higher phototypes, having a good safety profile.

Mycobacterium chelonae Is an Ubiquitous Atypical Mycobacterium.

The type of cutaneous infection varies mainly according to the patient's immune status, and the disseminated form is mostly found in the context of immunosuppression. We report the case of a 62-year-old male who was under long-term systemic corticosteroid therapy and presented with a 7-month history of multiple painless cutaneous lesions at various stages of development: papules, nodules, pustules and hemorrhagic crusts, as well as small erosions and ulcers distributed over the limbs and scalp. Cutaneous biopsy showed a suppurative granulomatous infiltrate with abscess formation. Fite stain revealed numerous extracellular bacilli, suggesting mycobacterial infection, particularly by atypical mycobacteria. Culture of a skin sample revealed Mycobacterium chelonae. The patient started multidrug therapy and showed clinical improvement despite of resistance to one of the antibiotics. This striking presentation underlines the role of immunosuppression with corticotherapy as a major risk factor for these infections. Multidrug therapy is advised and antibiogram is essential in directing treatment.

Bullous pemphigoid and comorbidities: a case-control study in Portuguese patients.

BACKGROUND: Although rare, bullous pemphigoid (BP) is the most common autoimmune blistering disease. Recent studies have shown that patients with bullous pemphigoid are more likely to have neurological and psychiatric diseases, particularly prior to the diagnosis of bullous pemphigoid. OBJECTIVE: The aims were: (i) to evaluate the demographic and clinical features of bullous pemphigoid from a database of patients at a Portuguese university hospital and (ii) to compare the prevalence of comorbid conditions before the diagnosis of bullous pemphigoid with a control group. METHODS: Seventy-seven patients with bullous pemphigoid were enrolled in the study. They were compared with 176 age- and gender-matched controls, which also had the same inpatient to outpatient ratio, but no history of bullous or cutaneous malignant disease. Univariate and multivariate analyses were used to calculate odds ratios for specific comorbid diseases. RESULTS: At least one neurologic diagnosis was present in 55.8% of BP patients compared with 20.5% controls (p<0.001). Comparing cases to controls, stroke was seen in 35.1 vs. 6.8%, OR 8.10 (3.80-17.25); dementia in 37.7 vs. 11.9%, OR 5.25 (2.71-10.16); and Parkinson's disease in 5.2 vs. 1.1%, OR 4.91 (0.88-27.44). Using multivariate analysis, all diseases except Parkinson's retained their association with BP. Patients under systemic treatment were eight times more likely to have complications than those treated with topical steroids (p< 0.017). CONCLUSIONS: The results of this study substantiate the association between BP and neurological diseases. In addition, they highlight the potential complications associated with the treatment of BP.

Bullous pemphigoid and comorbidities: a case-control study in Portuguese patients

BACKGROUND: aAlthough rare, bullous pemphigoid (BP) is the most common autoimmune blistering disease. Recent studies have shown that patients with bullous pemphigoid are more likely to have neurological and psychiatric diseases, particularly prior to the diagnosis of bullous pemphigoid. OBJECTIVE: The aims were: (i) to evaluate the demographic and clinical features of bullous pemphigoid from a database of patients at a Portuguese university hospital and (ii) to compare the prevalence of comorbid conditions before the diagnosis of bullous pemphigoid with a control group. METHODS: Seventy-seven patients with bullous pemphigoid were enrolled in the study. They were compared with 176 age- and gender-matched controls, which also had the same inpatient to outpatient ratio, but no history of bullous or cutaneous malignant disease. Univariate and multivariate analyses were used to calculate odds ratios for specific comorbid diseases. RESULTS: At least one neurologic diagnosis was present in 55.8% of BP patients compared with 20.5% controls (p<0.001). Comparing cases to controls, stroke was seen in 35.1 vs. 6.8%, OR 8.10 (3.80-17.25); dementia in 37.7 vs. 11.9%, OR 5.25 (2.71-10.16); and Parkinson's disease in 5.2 vs. 1.1%, OR 4.91 (0.88-27.44). Using multivariate analysis, all diseases except Parkinson's retained their association with BP. Patients under systemic treatment were eight times more likely to have complications than those treated with topical steroids (p< 0.017). CONCLUSIONS: The results of this study substantiate the association between BP and neurological diseases. In addition, they highlight the potential complications associated with the treatment of BP. .

Pyoderma gangrenosum--a review of 24 cases observed over 10 years.

BACKGROUND AND OBJECTIVES: Pyoderma gangrenosum (PG) is a disorder, included in the spectrum of neutrophilic and auto-inflammatory dermatoses, whose clinical aspects and outcome we intend to characterize. MATERIALS AND METHODS: In a retrospective study based on files of patients diagnosed during a 10-year period (2000-2009), we evaluated demographic data, anatomic locations, number of lesions, clinical variants, associated diseases, treatment regimens, healing time, and recurrence. RESULTS: A total of 24 patients were included, 19 women and five men (F/M = 3.8/1), aged between 17 and 89 years (mean 58.3 ± 24.6 years) with a diagnosis of PG. Lesions, single in 15 patients (62.5%) and multiple in nine (37.5%), were localized in the lower limbs in 19 patients (79.2%), upper limbs (4), abdomen (4), face (2) and genital area (1). Clinical variants observed were ulcerative (17 patients), pustular (4), bullous (2) and superficial granulomatous (1). Associated systemic diseases were observed in 18 patients (75%), gastrointestinal in seven patients (29.2%), hematological in seven (25%), autoimmune inflammatory in three (12%), and solid tumors in two (8.3%). Systemic steroids were used in the treatment, either alone in 10 patients (41.7%) or combined with cyclosporine in eight (33.3%). Complete healing was achieved in 20 patients, on average five months after diagnosis, but lesions recurred one or more times in four patients (16.7%). CONCLUSIONS: As reported in the literature, PG is a rare disorder, more common in females, frequently associated with systemic disease, which compromises the prognosis.

Prediction of sentinel node status and clinical outcome in a melanoma centre.

Background. Sentinel lymph node biopsy (SLNB) is a standard procedure for patients with localized cutaneous melanoma. The National Comprehensive Cancer Network (NCCN) Melanoma Panel has reinforced the status of the sentinel lymph node (SLN) as an important prognostic factor for melanoma survival. We sought to identify predictive factors associated with a positive SLNB and overall survival in our population. Methods. We performed a retrospective chart review of 221 patients who have done a successful SLNB for melanoma between 2004 and 2010 at our department. Univariate and multivariate analyses were done. Results. The SLNB was positive in 48 patients (21.7%). Univariate analysis showed that male gender, increasing Breslow thickness, tumor type, and absence of tumor-infiltrating lymphocytes were significantly associated with a positive SLNB. Multivariate analysis confirmed that Breslow thickness and the absence of tumor-infiltrating lymphocytes are independently predictive of SLN metastasis. The 5-year survival rates were 53.1% for SLN positive patients and 88.2% for SLN negative patients. Breslow thickness and the SLN status independently predict overall survival. Conclusions. The risk factors for a positive SLNB are consistent with those found in the previous literature. In addition, the SLN status is a major determinant of survival, which highlights its importance in melanoma management.

Synchronous pyoderma gangrenosum and inflammatory bowel disease, healing after total proctocolectomy.

We present a female patient observed with painful violaceous plaques with central bullae and pustules on the lower limbs, rapidly transformed into ulcers, associated with bloody diarrhea, recurrent oral erosions and hyperthermia in the previous 3 months. Cutaneous biopsy was consistent with pyoderma gangrenosum, and intestinal diagnostic procedures revealed a non-classifiable inflammatory bowel disease with high x-ANCA titers. Soon after admission the patient was submitted to total proctocolectomy following colonic perforation. Complete ulcer healing occurred three months after surgery, without recurrence. Pyoderma gangrenosum is a rare dermatosis frequently associated with inflammatory bowel disease. This case is particularly interesting for the synchronic clinical presentation of cutaneous and intestinal diseases, but also for the prompt regression of the former after total proctocolectomy.

Synchronous pyoderma gangrenosum and inflammatory bowel disease, healing after total proctocolectomy / Pioderma gangrenoso associado a doença inflamatória intestinal, com resolução após proctocolectomia total

We present a female patient observed with painful violaceous plaques with central bullae and pustules on the lower limbs, rapidly transformed into ulcers, associated with bloody diarrhea, recurrent oral erosions and hyperthermia in the previous 3 months. Cutaneous biopsy was consistent with pyoderma gangrenosum, and intestinal diagnostic procedures revealed a non-classifiable inflammatory bowel disease with high x-ANCA titers. Soon after admission the patient was submitted to total proctocolectomy following colonic perforation. Complete ulcer healing occurred three months after surgery, without recurrence. Pyoderma gangrenosum is a rare dermatosis frequently associated with inflammatory bowel disease. This case is particularly interesting for the synchronic clinical presentation of cutaneous and intestinal diseases, but also for the prompt regression of the former after total proctocolectomy.

Apresentamos uma paciente do sexo feminino observada com múltiplas placas violáceas dolorosas dos membros inferiores, com bolhas e pústulas evoluindo rapidamente para lesões ulceradas, surgindo no contexto de diarreia sanguinolenta, erosões orais recorrentes e febre com três meses de evolução. A biópsia cutânea foi compatível com pioderma gangrenoso; o estudo complementar revelou doença inflamatória intestinal inclassificável com títulos elevados de x-ANCA. Após perfuração cólica, a doente foi submetida a proctocolectomia total, com rápida cicatrização das lesões cutâneas ulceradas em três meses, sem recorrência. O pioderma gangrenoso é uma dermatose rara frequentemente associada a doença inflamatória intestinal. É interessante verificar neste caso a apresentação clínica sincrónica das doenças cutânea e intestinal, bem como a rápida resolução da primeira após proctocolectomia total.

Epidemiology of basal cell carcinomas and squamous cell carcinomas in a Department of Dermatology: a 5 year review.

BACKGROUND: Non-melanoma skin cancer, a common designation for both basal cell carcinomas and squamous cell carcinomas, is the most frequent malignant skin neoplasm. OBJECTIVE: Epidemiologic characterization of the population with Non-melanoma skin cancer. METHODS: Retrospective analysis of all patients diagnosed with Non-melanoma skin cancer based on histopathologic analysis of all incisional or excisional skin biopsies performed between 2004 and 2008 in a Department of Dermatology. RESULTS: A total of 3075 Non-melanoma skin cancers were identified, representing 88% of all malignant skin neoplasms (n=3493) diagnosed in the same period. Of those, 68,3% were basal cell carcinomas. Most Non-melanoma skin cancer patients were female and over 60 years old. Of all Non-melanoma skin cancer, 81,7% (n=1443) were located in sun-exposed skin, and represented 95,1% of malignant skin neoplasms in sun-exposed skin. Non-melanoma skin cancer was the most frequent malignant skin neoplasm in most topographic locations, except for abdomen and pelvis - over 95% of all malignant skin neoplasms in the face, neck and scalp were Non-melanoma skin cancer. Basal cell carcinomas were clearly predominant in all locations, except in upper and lower limbs, lower lip and genitals, where squamous cell carcinomas represented respectively 77,7%, 77,4%, 94,7% and 95,3% of the Non-melanoma skin cancers. CONCLUSION: Being the most common skin cancer, Non-melanoma skin cancer should be under constant surveillance, in order to monitor its epidemiologic dynamics, the efficiency of preventive measures and the adaptation of the healthcare resources.

Epidemiology of basal cell carcinomas and squamous cell carcinomas in a Department of Dermatology: a 5 year review / Epidemiologia dos carcinomas basocelulares e espinhocelulares em um Serviço de Dermatologia: revisão de 5 anos

BACKGROUND: Non-melanoma skin cancer, a common designation for both basal cell carcinomas and squamous cell carcinomas, is the most frequent malignant skin neoplasm. OBJECTIVE: Epidemiologic characterization of the population with Non-melanoma skin cancer. METHODS: Retrospective analysis of all patients diagnosed with Non-melanoma skin cancer based on histopathologic analysis of all incisional or excisional skin biopsies performed between 2004 and 2008 in a Department of Dermatology. RESULTS: A total of 3075 Non-melanoma skin cancers were identified, representing 88% of all malignant skin neoplasms (n=3493) diagnosed in the same period. Of those, 68,3% were basal cell carcinomas. Most Non-melanoma skin cancer patients were female and over 60 years old. Of all Non-melanoma skin cancer, 81,7% (n=1443) were located in sun-exposed skin, and represented 95,1% of malignant skin neoplasms in sun-exposed skin. Non-melanoma skin cancer was the most frequent malignant skin neoplasm in most topographic locations, except for abdomen and pelvis - over 95% of all malignant skin neoplasms in the face, neck and scalp were Non-melanoma skin cancer. Basal cell carcinomas were clearly predominant in all locations, except in upper and lower limbs, lower lip and genitals, where squamous cell carcinomas represented respectively 77,7%, 77,4%, 94,7% and 95,3% of the Non-melanoma skin cancers. CONCLUSION: Being the most common skin cancer, Non-melanoma skin cancer should be under constant surveillance, in order to monitor its epidemiologic dynamics, the efficiency of preventive measures and the adaptation of the healthcare resources.

FUNDAMENTOS: O cancro cutâneo não-melanoma, designação conjunta para os carcinomas basocelulares e espinhocelulares, é o tipo de neoplasia cutânea maligna mais frequente. OBJETIVOS: Caracterização epidemiológica da população diagnosticada com cancro cutâneo não-melanoma. MÉTODOS: Foi realizada uma análise retrospectiva dos doentes portadores de cancro cutâneo não-melanoma identificados por análise histológica de todas as biopsias cutâneas incisionais ou excisionais ao longo de 5 anos (2004-2008) num serviço de Dermatologia. RESULTADOS: foram identificados 3075 cancros cutâneos não-melanoma, representando 88% do total de neoplasias malignas diagnosticadas no mesmo período (n=3493). Destes, 68,3% eram carcinomas basocelulares. No seu conjunto, a população de cancro cutâneo nãomelanoma era predominantemente constituída por indivíduos idosos e do sexo feminino, tendo sido observado um aumento consistente da freqüência ao longo do período avaliado (5,25%/ano). A maioria dos cancros cutâneos não-melanoma (n=1443, 81,7%) foi identificada nas áreas de pele foto-exposta, representando 95,1% de todas as neoplasias malignas em áreas foto-expostas. O cancro cutâneo não-melanoma foi a neoplasia mais representativa na generalidade das áreas topográficas, à exceção do abdômen e da pélvis, representando, em particular, mais de 95% das neoplasias malignas da face, da região cervical e do couro cabeludo. O carcinoma basocelular foi o cancro cutâneo não-melanoma predominante em todas as localizações, à exceção dos membros inferiores e superiores, lábio inferior e da área genital, onde o carcinoma espinhocelular representou, respectivamente, 77,7%, 77,4%, 94,7% e 95,3% dos casos. CONCLUSÕES: O cancro cutâneo não-melanoma, como neoplasia maligna cutânea mais freqüente, deverá ser alvo de uma monitorização regular, com vista à determinação da sua dinâmica epidemiológica, da eficácia das medidas preventivas e adequação dos recursos de saúde.

Florid cutaneous papillomatosis and acanthosis nigricans maligna revealing gastric adenocarcinoma.

This paper reports the case of a 57-year-old, previously healthy male with no systemic symptoms who over a short period of time developed multiple wart-like lesions on his trunk, limbs and face, typical lesions of acanthosis nigricans in the major body folds and tripe palms. Diagnostic tests revealed a metastatic gastric adenocarcinoma. Despite the implementation of therapy, which had a transient effect on the tumor and skin lesions, the patient died in 14 months. The association of these three paraneoplastic dermatoses (florid cutaneous papillomatosis, acanthosis nigricans maligna and tripe palms) in the same patient, apparently with a common pathogenic mechanism, is noteworthy.

Value of patch tests in clindamycin-related drug eruptions.

BACKGROUND: Patch tests help to confirm the aetiology of the cutaneous adverse drug reactions involving delayed hypersensitivity mechanisms, but the results vary with the pattern of skin reaction and the culprit drug. OBJECTIVES: To analyse the results of patch tests in patients with cutaneous adverse drug reactions imputable to clindamycin and assess their contribution to the diagnosis. PATIENTS AND METHODS: Between 2005 and 2009, we studied patients with delayed cutaneous adverse drug reactions following administration of clindamycin, usually associated with other drugs. After resolution of the cutaneous adverse drug reaction, patch tests were performed with a series of antibiotics, including pure clindamycin 10% in petrolatum. RESULTS: We studied 30 patients (23 females and 7 males) aged 33-86 years (mean 59.97 years) with generalized maculopapular exanthema where clindamycin was among the highly suspected drugs. Two patients had a previous positive involuntary rechallenge. Patch tests with clindamycin were positive in 9 of 30 patients (30%). More than 50 control patients patch tested with clindamycin were negative. DISCUSSION: We considered the positive patch tests results with clindamycin, in the 9 patients with maculopapular exantema, to be specific, versus the negative results observed in the control group. Although the sensitivity is low (30%), they confirmed the responsibility of this antibiotic in cutaneous adverse drug reactions in which, with only chronological criteria, it was not possible to conclude on the culprit drug.

Papilomatose cutânea florida e acantose nigricante maligna reveladoras de neoplasia gástrica / Florid cutaneous papillomatosis and acanthosis nigricans maligna revealing gastric adenocarcinoma

Apresenta-se o caso clínico de um doente de 57 anos, previamente saudável, sem sintomatologia sistêmica, que, num curto intervalo de tempo, desenvolve múltiplas lesões semelhantes a verrugas virais no tronco, membros e face, lesões típicas de acantose nigricante nas grandes pregas e uma queratodermia difusa palmar com paquidermatoglifia. Os exames complementares de diagnóstico revelaram uma neoplasia gástrica metastizada. Apesar da instituição da terapêutica, com efeito transitório na neoplasia e nas lesões cutâneas, o doente viria a falecer em 14 meses. Salientamos a associação destas três dermatoses paraneoplásicas num mesmo paciente: papilomatose cutânea florida, acantose nigricante maligna e tripe palms que parecem ter um mecanismo patogênico comum.

This paper reports the case of a 57-year-old, previously healthy male with no systemic symptoms who over a short period of time developed multiple wart-like lesions on his trunk, limbs and face, typical lesions of acanthosis nigricans in the major body folds and tripe palms. Diagnostic tests revealed a metastatic gastric adenocarcinoma. Despite the implementation of therapy, which had a transient effect on the tumor and skin lesions, the patient died in 14 months. The association of these three paraneoplastic dermatoses (florid cutaneous papillomatosis, acanthosis nigricans maligna and tripe palms) in the same patient, apparently with a common pathogenic mechanism, is noteworthy.