Acute hemorrhagic edema in an infant]

The clinical and histological features of acute haemorrhagic oedema of childhood are discussed with reference to an actual case. Although closely related to Schoenlein-Henoch syndrome, it is considered a distinct entity, on the basis of the typical clinical picture and the excellent prognosis.     

Polychondritis recidivans et atrophicans

A typical case of chronic atrophic polychondritis with chondritis of the ear and episcleritis is presented. The inflammation subsided on treatment with colchicine. This entity is rare. It has an uncertain prognosis and causes a wide variety of symptoms. Accordingly, the spectrum of conditions that need to be considered in the differential diagnosis is also wide.     

Diagnóstico y tratamiento del síndrome de piel sensible: un algoritmo para la práctica clínica habitual

Sensitive skin has traditionally been viewed as a cosmetic problem or as a purely psychosomatic alteration with a major subjective component. Different studies of its pathophysiologic etiology, however, have shown it to be a complex entity that several authors now consider to be a neurodermatological syndrome. Because of this complexity, skin sensitivity can be difficult to diagnose and treat, particularly considering that it may present with another disease. Simple tools applicable to clinical practice are thus necessary to identify and manage this disease as an independent entity. In this study, we perform a practical review of the most recent scientific advances in the area of sensitive skin that justify it being considered an individual entity, and provide tools for its identification and treatment. We propose diagnostic and treatment algorithms based on evidence from the literature and our experience and expertise.     

Disseminated pagetoid reticulosis: plaques and tumoral lesions occurring simultaneously in the same patient

Disseminated pagetoid reticulosis is considered to have a poor prognosis. Some authors regard this disease as an epidermotropic type of mycosis fungoides rather than a distinct entity. We report the case of an 83-year-old woman with disseminated pagetoid reticulosis who subsequently developed tumoral lesions and died soon after the appearance of these tumours. Our case is in agreement with findings that disseminated pagetoid reticulosis has an aggressive clinical behaviour.     

Giant clear-cell acanthoma with keratoacanthoma-like changes: a case report

Clear cell acanthoma is a benign epidermal lesion with a variable clinical appearance and distinct histopathology features. Although, it is considered an entirely benign entity, few case reports describe unusual or atypical variants of clear cell acanthoma. We observed a case of a large clear cell acanthoma that also has features of a keratoacanthoma.     

Blastic NK-cell lymphoma

Blastic NK () cell lymphoma is a rare type of lymphoma, recognized as an independent entity in the new World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for cutaneous lymphomas. Its clinical, morphological and immunophenotypical characteristics are relatively well defined. It frequently starts with extranodal involvement (primarily cutaneous), often presents with splenomegaly and pancytopenia, and initially responds well to chemotherapy, although the medium-term prognosis is usually poor. It was first considered to originate from immature NK lymphocytes, but now its origin is thought to be plasmacytoid dendritic cell precursors. We present the clinicopathological characteristics of a new case of this entity in a 78-year-old male patient.     

Lymphomatoid papulosis--pseudolymphoma with uncertain prognosis

On account of its distinct clinical and histological picture, lymphomatoid papulosis (l.p.) is mostly considered an entity. A 70-year-old patient with l.p. is described. Histologically, there were similarities to the granulomatous type of pseudolymphoma. The oral treatment with penicillin for 4 weeks was successful, but we cannot exclude a spontaneous remission.     

Angiolymphoid hyperplasia with eosinophilia and Kimura's disease – a clinical and histopathological comparison

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular neoplasm mainly affecting middle‐aged women. Lesions typically affect the head and neck region. ALHE is considered a distinct disease entity different from Kimura's disease, a benign reactive lymphoid proliferation that is predominantly seen in young Asian men although it can affect all ethnic groups. In contrast to ALHE, Kimura's disease is typically associated with peripheral blood eosinophilia, increased serum IgE and lymphadenopathy. Several case reports suggest an overlap between ALHE and Kimura's disease. We review the current literature and discuss whether AHLE and Kimura's disease might represent two extreme variants of the same disease entity.     

Epitheloid Hemangioma: A Report of Two Cases

We report two cases of epitheloid hemangioma presented with multiple nodular lesions over head and neck region. One of them gave history of bleeding on minor trauma. Pyogenic granuloma was considered as a differential diagnosis from the morphological appearance and history of bleeding. Nodular leprosy, sarcoidosis, and secondary syphilis were also considered. Histopathological examination of both was typical of epitheloid hemangioma, an entity commonly overlooked clinically due to its rarity.     

Contact orofacial granulomatosis caused by delayed hypersensitivity to gold and mercury

Orofacial granulomatosis, an entity with characteristic clinicopathologic features, is thought to be a reactive process. The authors describe orofacial granulomatosis associated with contact allergy to gold in dental crowns in one patient and a possible allergic contact reaction to mercury from dental fillings in another one. Thus allergic contact dermatitis to the metals gold and mercury should be considered as a possible etiologic agent of orofacial granulomatosis.     

Actinic superficial folliculitis

Actinic superficial folliculitis was first described in 1985, and since then only three reports have been published. Clinically and histopathologically this disease is very particular and has been suggested to be considered as an entity. We report on a 30-year-old man who presented with an extensive superficial follicular pustulosis on his back, shoulders and upper chest after exposure to intense heat and subsequent sweating on a sunny day. The pustules arose within 24-36 h afterwards. Histology and immunohistochemistry revealed subcorneal pustules, suppurative folliculitis and an infiltrate consisting of T cells, macrophages and neutrophils around the hair follicle, sebaceous glands and small vessels. To the best of our knowledge this is the fourth report on actinic superficial folliculitis and the first on which a characterization of the inflammatory infiltrate has been performed. Because of the impressive, unique symptoms and the characteristic histology we agree with those who have suggested that actinic superficial folliculitis is a new entity.     

Raynaud's phenomenon, anticentromere antibodies, and digital necrosis without sclerodactyly: an entity independent of scleroderma?

We describe 4 women of 43, 73, 84, and 86 years with Raynaud's phenomenon, severe digital necrosis, and high serum levels of anticentromere antibodies without skin thickening or internal organ sclerosis. Investigations revealed no diabetes or arterial vascular disease leading to arterial obstruction. Histologic examination did not show any dermal sclerosis or calcinosis. The intravenous infusions of prostaglandin reversed the ischemic lesions in 3 patients. These cases suggest that the triad Raynaud's phenomenon, anticentromere antibodies and necrosis of digits without sclerodactyly and sclerosis of internal organ should be considered as an entity distinct from scleroderma with sclerosis. For this entity we propose the name RACAND syndrome.     

Epithelial sheath neuroma: be aware of benign perineural invasion!

Perineural involvement by epithelial cells is usually considered as a sign of malignancy and is seen in a variety of malignant skin neoplasms. However, there are other benign conditions characterized by the presence of perineural involvement by epithelial cells. We present a case of epithelial sheath neuroma in a 43-year-old male. The clinicopathological features of this newly described entity are discussed together with the differential diagnosis and the different hypotheses of pathogenesis. Both pathologists and dermatologists should be aware of this entity to avoid misdiagnosis of malignancy.     

A case of argyria after colloidal silver ingestion

BACKGROUND: Argyria is often considered an entity of the past, one which has largely disappeared with the cessation of silver usage in oral medications. However, with the practice of colloidal silver ingestion in current "alternative health" treatments, argyria should be considered in the differential diagnosis of blue-gray hyperpigmentation. METHODS: A single case report with clinicopathological correlation. RESULTS: Histological examination of skin biopsy specimen, which showed perieccrine brown-black granules, verified that colloidal silver rather than a prescribed medication was the source of the patient's dyspigmentation.     

Reticular erythematous mucinosis syndrome in a patient with polyarthritis

A patient with seronegative oligoarthritis who developed the reticular erythematous mucinosis (REM) syndrome is described. This syndrome is considered to be a dermatological entity unrelated to systemic disorders. Aggravation of the rash by exposure to sunlight and a good response to anti-malarial agents suggest a relationship with rheumatological disorders, e.g. rheumatoid arthritis and systemic lupus erythematosus. Dermatologists consulted by a patient with the REM syndrome should be aware of the possibility of an associated rheumatological disease.     

Multiple cutaneous metastases of a scapular chondrosarcoma

The onset of metastatic chondrosarcoma in a young woman was heralded by the appearance of multiple firm, tender, cutaneous nodules two years after the primary tumor in the left scapula had been resected. Chondrosarcoma rarely metastasizes to the skin, but when it does, it may have an identical histological appearance to a cartilaginous tumor of the skin, an entity that is generally considered clinically benign. Although it is very unlikely that an occult chondrosarcoma will be first manifested clinically by a solitary cutaneous metastasis, the abrupt appearance of multiple cutaneous cartilaginous lesions would seem to warrant an investigation for primary chondrosarcoma.     

Palmar filiform parakeratotic hyperkeratosis without underlying malignancy

Filiform hyperkeratosis (FH) is a rare entity clinically characterized by keratotic spicules on the palms, soles or other areas of the body surface. Its association with several diseases, including neoplasms, has been extensively discussed but currently it is not considered a well-defined paraneoplastic disorder. We report a 72-year-old patient that referred lesions on both palms of three months duration. The rest of the body surface did not show similar lesions. Complementary exams did not reveal any abnormal findings. The histopathological exam showed parakeratotic columns with a slightly decreased granular layer and a mild dermal inflammatory infiltrate. In the last years different terms have been employed to refer to these hyperkeratotic lesions contributing to the lack of clarity that currently persists. Although FH might be classified close to the clinical spectrum of porokeratoses, it presents particular clinical and histological findings that allow it to be considered a separate entity. We report a new case of this rare disorder with exclusive palmar involvement in a patient without underlying malignancy and review the main characteristics of similar cases reported in the literature. Despite the obscure association between this entity and neoplasms, the majority of authors deem it necessary to rule out underlying diseases.     

Zosteriform connective-tissue nevus

Zosteriform connective-tissue nevus, because of its distribution and histopathologic characteristics, is considered to be a separate entity. The only previous case of zosteriform connective-tissue nevus to be reported in the American literature was in 1944. We report and discuss a similar case.     

Circumscribed palmar hypokeratosis associated with disseminated superficial actinic porokeratosis

Circumscribed palmar and plantar hypokeratosis (CPPH) is a recently described skin condition with particular clinical and histopathological findings. The etiopathogeny of CPPH is still unclear, but an abnormal clone of keratinocytes has been postulated as the possible origin. CPPH has been considered an entity with no potential malignant transformation, but recently, it has been recognized in association with signs of actinic keratosis in the same biopsy. CPPH has never been recognized in association with porokeratosis. Although these entities seem to be different, a possible relationship between them might be suggested.