Malignant syphilis in a patient with acquired human immunodeficiency virus (HIV) infection

Abstract Malignant syphilis is an uncommon variant of syphilis, most often (but not always) found in immunosuppressed individuals. This report describes the case of a 57-year-old man, infected with the acquired human immunodeficiency virus (HIV), with a generalized picture of erythematous-squamous papules that rapidly progressed to painful and ulcerated plaques and nodules, some covered with a black rupioid crust. The analytical study performed revealed positive VDRL (Venereal Disease Research Laboratory) and RPR (Rapid Plasma Reagin). The skin biopsy was nonspecific; however, the immunohistochemical analysis disclosed the presence of spirochetes. The patient was then treated with benzathine penicillin G 2.4 MU once a week IM for three weeks, with progressive resolution of the lesions. Considering its rarity, this atypical form of syphilis that needs to be known to better recognize its clinical presentation and provide more prompt treatment to patients.

Malignant syphilis in a patient with acquired human immunodeficiency virus (HIV) infection.

Malignant syphilis is an uncommon variant of syphilis, most often (but not always) found in immunosuppressed individuals. This report describes the case of a 57-year-old man, infected with the acquired human immunodeficiency virus (HIV), with a generalized picture of erythematous-squamous papules that rapidly progressed to painful and ulcerated plaques and nodules, some covered with a black rupioid crust. The analytical study performed revealed positive VDRL (Venereal Disease Research Laboratory) and RPR (Rapid Plasma Reagin). The skin biopsy was nonspecific; however, the immunohistochemical analysis disclosed the presence of spirochetes. The patient was then treated with benzathine penicillin G 2.4 MU once a week IM for three weeks, with progressive resolution of the lesions. Considering its rarity, this atypical form of syphilis that needs to be known to better recognize its clinical presentation and provide more prompt treatment to patients.

Nail Disease: Clinical Decisions among Portuguese Dermatologists and Family Physicians.

Onychomycosis is one of the most common nail disorders and may be difficult to distinguish from other causes of nail dystrophy, based on clinical grounds alone. With this study, we aimed to describe the use of fungal testing by dermatologists and family physicians in their daily current practice, analyze their respective familiarity with nail disease diagnosis, and ultimately treatment decision-making by both groups. An online survey was distributed among Portuguese dermatologists, trainees, and family physicians by email. The survey focused on the diagnostic impression, use of diagnostic methods to confirm a fungal infection, and the subsequent assessment of treatment. One hundred fifty-one responses were obtained, 60 (39.7%) from dermatologists and 91 (60.3%) from family physicians; 98.3% of dermatologists mentioned usually requesting a fungal testing at their local institution or outside, while this percentage was 50.5% among family physicians (p < 0.001). Regarding the diagnosis, the median of correct diagnosis by the dermatologist group was higher (10/15) than the family physicians (6/15). Considering the treatment strategy, we observed that in the dermatologists' group it would result in unnecessary treatment in a median of 2 cases, while in the family physicians' group, in a median of 4 cases.

Pediatric Kaposi's sarcoma associated with immune reconstitution inflammatory syndrome.

Immune reconstitution inflammatory syndrome (IRIS) represents paradoxical immune-mediated inflammation in response to an infecting pathogen, occurring after initiation of antiretroviral therapy (ART), concomitantly with immune system recovery. It has also been described in Kaposi's sarcoma (KS). We report a case of a 9-year-old Guinean girl, who developed Kaposi's sarcoma, following introduction of ART. KS associated with immune reconstitution inflammatory syndrome is rare, especially in children, but with the increased use of ART is becoming more prevalent.

Whole-Scalp Cobblestone Plica Neuropathica with Alopecia.

Plica neuropathica (PN) is an uncommon scalp condition that usually presents as a compact mass of scalp hair. We report a case of a 55-year-old woman presenting with PN. She presented with a 40-year history of thick plaques of yellow scale afflicting the entire scalp, progressive alopecia, and seeming lack of care of her own condition. Histology showed epidermal nevus-like features and severe miniaturization.

Case for diagnosis. Multinucleated cell angiohistiocytoma.

Multinucleate cell angiohistiocytoma is a rare idiopathic benign fibrohistiocytic and vascular proliferation usually presenting as multiple asymptomatic papules, red to violaceous in colour, primarily located on the extremities of middle-aged females. This entity is probably underdiagnosed due to the lack of recognition by clinicians and pathologists. We describe a patient with a multinucleate cell angiohistiocytoma of the face, a less frequent localization, in order to increase awareness of this entity and elucidate its clinical, histopathological, and immunohistochemistry features.

Case for diagnosis. Multinucleated cell angiohistiocytoma

Abstract: Multinucleate cell angiohistiocytoma is a rare idiopathic benign fibrohistiocytic and vascular proliferation usually presenting as multiple asymptomatic papules, red to violaceous in colour, primarily located on the extremities of middle-aged females. This entity is probably underdiagnosed due to the lack of recognition by clinicians and pathologists. We describe a patient with a multinucleate cell angiohistiocytoma of the face, a less frequent localization, in order to increase awareness of this entity and elucidate its clinical, histopathological, and immunohistochemistry features.

Ashy dermatosis with involvement of mucous membranes.

Ashy dermatosis is a rare condition, of unknown aetiology, in which mucous membranes are typically spared. The authors report the case of a 57-year-old female with a history of asymptomatic gray-bluish macules located on the trunk and oral mucosa. There were no relief changes on examination. Skin biopsies from the oral mucosa and trunk were performed and both were compatible with ashy dermatosis. The patient started treatment with oral clofazimine but due to the absence of clinical improvement the drug was discontinued three months later. This case report illustrates an atypical case of ashy dermatosis owing to the involvement of mucous membranes, which is rarely described in the literature.

Birt-Hogg-Dubé Syndrome - report of two cases with two new mutations.

INTRODUCTION: Birt-Hogg-Dubé syndrome (BHDS) is a rare autosomal dominant genodermatosis characterized by cutaneous fibrofolliculomas and/or trichodiscomas, lung cysts, spontaneous pneumothorax and renal tumors. However, its clinical expression is highly variable. This syndrome is caused by germline mutations in the folliculin gene (FLCN) on chromosome 17p11.2. MAIN OBSERVATIONS: Two men, 60 and 39-year-old, presented with a several year history of asymptomatic whitish papules scattered over the face and neck. Skin biopsies revealed fibrofolliculomas. The clinical diagnosis of BHDS was corroborated by identification of new heterozygotic mutations in FLCN gene, in exon 6 (C.573_574delinsT) and in exon 9 (c.1015C>T), respectively. Computed tomography scan of the thorax and abdomen showed pulmonary cysts with no suspicious kidneys lesions, and, in the case of the second patient, a mass in left adrenal gland. Laparoscopic left adrenalectomy was performed and histopathological examination was compatible with a malignant perivascular epithelioid cell tumor. CONCLUSIONS: The presence of multiple fibrofolliculomas should raise the suspicion of BHDS. Patients with this syndrome, regardless of the detected mutation, should be carefully monitored to ensure that potentially serious disease-related conditions can be detected early.

Ashy dermatosis with involvement of mucous membranes

Abstract: Ashy dermatosis is a rare condition, of unknown aetiology, in which mucous membranes are typically spared. The authors report the case of a 57-year-old female with a history of asymptomatic gray-bluish macules located on the trunk and oral mucosa. There were no relief changes on examination. Skin biopsies from the oral mucosa and trunk were performed and both were compatible with ashy dermatosis. The patient started treatment with oral clofazimine but due to the absence of clinical improvement the drug was discontinued three months later. This case report illustrates an atypical case of ashy dermatosis owing to the involvement of mucous membranes, which is rarely described in the literature.

Drug-induced photosensitivity: Photoallergic and phototoxic reactions.

Drug-induced photosensitivity refers to the development of cutaneous disease due to the interaction between a given chemical agent and sunlight. Photosensitivity reactions can be classified as phototoxic or photoallergic. Sometimes, there is an overlap between these two patterns, making their distinction particularly difficult for the clinician. We review the drugs that have been implicated as photosensitizers, the involved mechanism, and their clinical presentations. The main topical agents that cause contact photosensitivity are the nonsteroidal antiinflammatory drugs, whereas the main systemic drugs inducing photosensitivity are antimicrobials, nonsteroidal antiinflammatory agents, and cardiovascular drugs. Drug-induced photosensitivity remains a common clinical problem and is often underdiagnosed.