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Abstract and Figures
We report a rare clinical case of hemosiderotic dermatofibroma in a 36-year-old female patient. The main dermatoscopic finding was represented by homogeneous blue-gray pigmentation. The aim of this report is to demonstrate the rarity of the lesion and the dermatoscopic importance it assumes by sharing a blue-gray homogeneous pattern with other benign and malignant lesions.
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... Hemosiderotic DF was first described by Diss in 1938 [7] . It is a rare clinical presentation that represents 2% to 5.7% of the overall DF [6,[8][9][10] . It is often seen in extremities as a single or multiple, firm, papular tumor with a smooth surface and homogeneously pigmented areas varying from light to dark brown, red-bluish or green-yellowish color. ...
... Histologically, the hemosiderotic DF is compose of capillaries, extravasated erythrocytes, hemosiderin deposits inside the histiocytes and accumulation of extracellular hemosiderin [9,12] . The mechanisms of extravasations is unknown; trauma and a decrease in the quantity of the stroma's reticulin in cellular rich areas are considered trigger factors for micro hemorrhages [1,7,10] . Many authors consider the hemosiderotic DF as an early stage in the development of an aneurismatic DF [1,7,13] . ...
... Many authors consider the hemosiderotic DF as an early stage in the development of an aneurismatic DF [1,7,13] . It has been proposed that the formation of cavities in the aneurismatic DF is due to a constant erythrocyte extravasation, which put pressure inside the stroma creating cavities lacking of endothelial tissue a characteristic of the aneurismatic DF [7,9,10] . The diagnosis of this variety is hardly clinical. ...
... [5] It is a rare though distinct variant, comprising 2-5.7% of all dermatofibromas. [1,6] It has been described at various anatomical sites like the abdomen, breast, and leg as a blue-gray, dark brown or black nodule or plaque, which may sometimes simulate melanoma. [5][6][7][8] Histopathologically, hemosiderotic dermatofibroma is composed of numerous small vessels, extravasated red blood cells, intra-and extracellular hemosiderin deposition, in addition to features of a classic dermatofibroma like storiform pattern. ...
... [1,6] It has been described at various anatomical sites like the abdomen, breast, and leg as a blue-gray, dark brown or black nodule or plaque, which may sometimes simulate melanoma. [5][6][7][8] Histopathologically, hemosiderotic dermatofibroma is composed of numerous small vessels, extravasated red blood cells, intra-and extracellular hemosiderin deposition, in addition to features of a classic dermatofibroma like storiform pattern. [1] It is hypothesized that slow extravasation of blood from the capillaries within the tumor results in the production of hemosiderin, which is engulfed by the tumor cells. ...
... Extravasation of blood may occur because of microhaemorrhages triggered by trivial trauma in a vascular dermatofibroma. [5,6] Aneurysmal dermatofibroma is another uncommon variant, comprising around 1.7% of dermatofibromas. [3] It presents as a bluish-brown nodule on the limbs, often clinically misdiagnosed as a vascular, melanocytic, adnexal tumor or a cyst. ...
... aneurismal. [2][3][4] El dermatofibroma hemosiderótico es una variante rara de dermatofibroma que representa sólo el 2% de estas lesiones, y que se necesita de una correlación clínico-patológica para llegar a su diagnóstico. 3 En cuanto al dermatofibroma aneurismático o aneurismal, se considera también una variante infrecuente de dermatofibroma, de etiología desconocida, los cuales predominan en mujeres jóvenes y pueden afectar cualquier región anatómica. ...
... [2][3][4] El dermatofibroma hemosiderótico es una variante rara de dermatofibroma que representa sólo el 2% de estas lesiones, y que se necesita de una correlación clínico-patológica para llegar a su diagnóstico. 3 En cuanto al dermatofibroma aneurismático o aneurismal, se considera también una variante infrecuente de dermatofibroma, de etiología desconocida, los cuales predominan en mujeres jóvenes y pueden afectar cualquier región anatómica. 4 ...
CORRESPONDENCIA D e r m a t o l o g í a C M Q 2 0 1 9 ; 1 7 (1) : 1 6-2 0 R E S U M E N El dermatofibroma es un tumor fibrohistiocítico benigno co-mún, en general observado en adultos, con un ligero predo-minio en el sexo femenino, habitualmente se localizan en los miembros inferiores. Su causa sigue siendo desconocida, sin embargo, es muy aceptado que el dermatofibroma puede ser resultado de un trauma, por ejemplo, tras la picadura de algún insecto, la ruptura de un folículo o un quiste folicular. Presentan múltiples variantes, algunas de ellas poco frecuentes en la prác-tica clínica. El dermatofibroma hemosiderótico es una variante rara, con patrones clínicos e histológicos bien definidos, pero poco reportado en la literatura o informado como sinónimo del dermatofibroma aneurismático. En este trabajo presenta-mos dos casos clínicos de dermatofibromas hemosideróticos en pacientes de sexo femenino de 25 y 30 años de edad, con tiempo de evolución de 12 y ocho meses, respectivamente; además se presenta el reporte de un dermatofibroma aneu-rismático en una paciente de 40 años de edad. Se realiza la re-visión de la literatura porque se trata de variantes infrecuentes de dermatofibromas. Palabras clave: dermatofibroma hemosiderótico, dermatofibroma aneurismático, hemosiderina, células multinucleadas. Características histopatológicas de los dermatofibromas hemosiderótico y aneurismático: reporte de tres casos y revisión de la literatura
... The physical presentation of HDF and melanoma is similar and can appear as a variably pigmented papule, plaque, or hardened nodule [12]. In cases where the HDF is not removed, it is hypothesized that the red blood cells would extravasate from small vessels, eventually producing hemosiderin, which would be taken up by the tumor cells [13]. Over time, the extravasation would create intravascular spaces, thereby increasing internal tumor pressure and creating endothelial damage [13]. ...
... In cases where the HDF is not removed, it is hypothesized that the red blood cells would extravasate from small vessels, eventually producing hemosiderin, which would be taken up by the tumor cells [13]. Over time, the extravasation would create intravascular spaces, thereby increasing internal tumor pressure and creating endothelial damage [13]. At this stage, the HDF could be considered an early ADF [14]. ...
A dermatofibroma (DF) is a common, benign tumor composed of fibroblastic and histiocytic cells. DF presents clinically with several different reported variants. One rare variant is hemosiderotic DF (HDF), which is made up of small blood vessels and hemosiderin deposits. HDF can be indistinguishable, clinically, from melanoma, making the use of other pathological tools crucial in the diagnosis.
We report the case of a 25-year-old male medical student from the Caribbean who presented to our clinic with a single asymptomatic pigmented cystic lesion on his left posterior calf. The cystic lesion was excised surgically. Histopathology examination of the excised mass revealed a moderately cellular, poorly demarcated, dermal, fibrohistiocytic proliferation. Pathology consultation confirmed a diagnosis of HDF.
... Majority of these cases raise concern for melanoma following initial dermatoscopic examination and excision was performed for a definite diagnosis. 11,[13][14][15][16][17][18][19][20][21][22][23][24][25][26][27] The area rich in poorly formed vessel-like structures can make the lesion look like a vascular tumor such as Kaposi's sarcoma both microscopically and clinically. 28 Given the patient having no clinical history of immunosuppressive diseases/conditions, the chance of Kaposi's sarcoma is likely to be very low. ...
Hemosiderotic dermatofibroma (HDF) often mimics melanoma clinically. A definite diagnosis relies on histopathological evaluation and immunohistochemistry. As it can progress to aneurysmal dermatofibroma (ADF), complete excision is recommended. Hemosiderotic dermatofibroma (HDF) often mimics melanoma clinically. A definite diagnosis relies on histopathological evaluation and immunohistochemistry. As it can progress to aneurysmal dermatofibroma (ADF), complete excision is recommended.
... Los resultados de nuestra serie revelan que un color azulado central o zona homogénea rojiza con estructuras blancas y una delicada red de pigmento periférica son las características dermatoscópicas más frecuentes de los DF hemosideróticos, hallazgos concordantes con lo descrito en la literatura 1,9,10 . ...
Objectives
Aneurysmal dermatofibroma (ADF) and hemosiderotic dermatofibroma (HDF) are rare variants of dermatofibroma (DF) characterized by distinct histologic features. While HDF is traditionally considered a precursor to ADF, supporting evidence is limited, and the etiology remains unclear. A retrospective analysis of 2128 DF cases (2016-2019) was conducted to investigate the clinicopathologic characteristics of ADF, HDF, and other DFs.
Methods
Histopathologically diagnosed DF cases were examined for ADF and HDF. Univariate analyses were performed to compare clinicopathologic features.
Results
Among the cases, 168 (7.9%) were ADF and 29 (1.4%) were HDF. Aneurysmal dermatofibroma and HDF shared several common characteristics, including lower occurrence in females, larger size, and increased cellularity (all P < .0001). Notably, 29% of ADFs lacked hemosiderin deposition. Aneurysmal dermatofibroma primarily manifested on exposed areas (face and forearm, both P < .001). In contrast, 41% of HDFs occurred on the lower leg (P = .018), and all lower leg HDFs exhibited signs of venous stasis, distinguishing them from other HDFs (P < .0001).
Conclusions
Our findings indicate a potential close relationship between ADF and HDF. Contrary to conventional beliefs, we also presented the possibility of ADF progressing into HDFs. Physical trauma may induce ADF, and HDFs may emerge from ADFs in conjunction with venous stasis in the lower extremities.
Background:
Haemosiderotic and aneurysmal dermatofibromas are uncommon and frequently misdiagnosed lesions, which can be considered as different histopathological stages of the same tumour. A dermoscopic diagnosis testing accuracy has not been performed for these tumours to date.
Objectives:
To determine the diagnostic significance of dermoscopic structures and patterns associated with haemosiderotic/ aneurysmal dermatofibromas in a large series.
Methods:
Dermoscopic images of histopathologically proven cases of 110 haemosiderotic/ aneurysmal dermatofibromas and 501 other tumours were collected. The frequency, sensitivity, specificity, positive predictive value, and negative predictive value of the dermoscopic structures and patterns associated with these lesions were calculated.
Results:
haemosiderotic/ aneurysmal dermatofibromas are mostly symmetric lesions (86.5%) and a prominent homogeneous area was present in 100% of them. The presence of vascular structures was very common (86.4%) and dotted vessels were predominant (58.2%). Shiny white structures were seen in 85.5% of lesions, while a peripheral delicate pigment network was present in 69.1%. The most significant pattern was the one composed of a prominent homogeneous area and peripheral delicate pigment network, which showed a specificity of 100% with a relatively good sensitivity (69.1%). All the patterns containing a peripheral delicate pigment network showed very good specificities, positive predictive values, and negative predictive values. Those patterns without a peripheral delicate pigment network showed the highest sensitivities, but they showed a significant overlap with other tumours, mainly with melanoma.
Conclusions:
Dermoscopy is helpful in improving the diagnostic accuracy of haemosiderotic/ aneurysmal dermatofibromas. However, there is a considerable dermoscopic overlap between these tumours and melanoma, specifically when the peripheral delicate pigment network is absent.
Apresenta-se caso clínico de variante rara de dermatofibroma (tipo aneurismático) em paciente do sexo feminino de 72 anos de idade, cuja lesão se localizava na dobra flexural do membro superior direito, resultante de proliferação mesenquimal associada à vasos sanguíneos e hemorragia tecidual, com características histológicas próprias e bem definidas. Os objetivos desta descrição foram a raridade da lesão e a importância que ela assume, do ponto de vista histopatológico, quando comparada no diagnóstico diferencial com outros tumores benignos e malignos e a terminologia utilizada atualmente.
To review recent dermoscopy studies that provide new insights into the evolution of nevi and their patterns of pigmentation as they contribute to the diagnosis of nevi and the management of pigmented melanocytic nevi.
Data for this article were identified by searching the English and German literature by Medline and Journals@Ovid search for the period 1950 to January 2009.
The following relevant terms were used: dermoscopy, dermatoscopy, epiluminescence microscopy (ELM), surface microscopy, digital dermoscopy, digital dermatoscopy, digital epiluminescence microscopy, digital surface microscopy, melanocytic skin lesion, nevi, and pigmented skin lesions. There were no exclusion criteria.
The dermoscopic diagnosis of nevi relies on the following 4 criteria (each of which is characterized by 4 variables): (1) color (black, brown, gray, and blue); (2) pattern (globular, reticular, starburst, and homogeneous blue pattern); (3) pigment distribution (multifocal, central, eccentric, and uniform); and (4) special sites (face, acral areas, nail, and mucosa). In addition, the following 6 factors related to the patient might influence the pattern of pigmentation of the individual nevi: age, skin type, history of melanoma, UV exposure, pregnancy, and growth dynamics.
The 4 x 4 x 6 "rule" may help clinicians remember the basic dermoscopic criteria of nevi and the patient-related factors influencing their patterns. Dermoscopy is a useful technique for diagnosing melanocytic nevi, but the clinician should take additional factors into consideration to optimize the management of cases of pigmented lesions.
Seventeen cases are reported of a variety of cutaneous fibrous histiocytoma, which we have designated as aneurysmal ("angiomatoid") fibrous histiocytoma. These lesions differ from the classical cutaneous fibrous histiocytoma in both their clinical presentation and pathologic features. Clinically, they may be larger than the usual cutaneous fibrous histiocytoma, are blue, black, or dark red, and have a cystic consistency. They are most commonly located on the extremities and may be associated with symptoms of pain and rapid growth. The clinical diagnosis of fibrous histiocytoma is seldom considered in the differential diagnosis, which may include malignant melanoma, hemangioma, neurofibroma, and nonspecific cyst. Histologically, the lesions are characterized by the presence of large, blood-filled tissue spaces, which, at times, account for up to one half their size. These spaces lack an endothelial lining, being surrounded and lined by histiocytes, many of which contain hemosiderin pigment, fibroblasts, and foam cells. The solid portions of the tumor have the usual features of a cutaneous fibrous histiocytoma. This "angiomatoid" lesion is closely allied to what has been termed "hemosiderin histiocytoma," which appears to be a precursor stage in its formation. The presence of extravasated erythrocytes in combination with a spindle-cell stroma may lead to an erroneous diagnosis of Kaposi's sarcoma. This cutaneous tumor has architectural and cytologic similarities to its malignant soft tissue counterpart recently described as angiomatoid malignant fibrous histiocytoma. However, unlike the latter, the cutaneous lesion is benign and lacks the prominent inflammatory infiltrate, pleomorphic appearance, and systemic manifestations of its soft tissue counterpart. The distinctive clinical and pathologic features of the cutaneous lesion serve to separate it as a specific variant of the cutaneous fibrous histiocytomas.
In this study, dermatoscopic examination of 24 dermatofibromas was performed to evaluate specific dermoscopic criteria. A central white scarlike patch was appreciable in 22 of 24 lesions, whereas 20 of 24 dermatofibromas exhibited a delicate pigment network at the periphery. This stereotypical dermatoscopic finding allowed the diagnosis of dermatofibroma in most instances. (J Am Acad Dermatol 2000;43:1123-5.).
The lesions shown are from the shoulder of a 52-year-old white woman (Figure 1), the leg of a 34-year-old white woman (Figure 2), and the thigh of a 38-year-old white woman (Figure 3) (size bar, 2.5 mm). All 3 lesions reveal similar findings. White patchy areas in the center of the lesions, confluent or multiple, and peripheral pigment network are present in some lesions (Figure 1 and Figure 2), absent in others (Figure 3). This pigmented network corresponds to hyperplastic epidermis with hyperpigmentation of the basal layer and elongated rete ridges, whereas the whitish patch structure is caused by the presence of a clear (Grenz) zone from the spindle cell tumor in the dermis, and is composed of fibroblastlike spindle cells, histiocytes, and blood vessels in varying proportions. Capillaries may be plentiful in the stroma, giving a special appearance (Figure 3).
The clinical diagnosis of dermatofibroma is commonly easy. However, the differentiation of dermatofibroma from other cutaneous tumours is difficult in some instances, primarily in atypical cases and rare variants. Haemosiderotic dermatofibroma is a variant composed of numerous small vessels, extravasated erythrocytes and intra- and extracellular haemosiderin deposits. Aneurysmal dermatofibroma is a variant composed of large, blood-filled spaces without endothelial lining. Some authors consider that haemosiderotic dermatofibroma is an early stage in the development of aneurysmal dermatofibroma. The clinical differential diagnosis of haemosiderotic or aneurysmal dermatofibroma must include melanoma and other melanocytic tumours, vascular neoplasms, adnexal tumours and nonspecific cysts. Dermoscopy improves the diagnostic accuracy in pigmented and nonpigmented skin lesions.
To evaluate specific dermoscopic criteria.
Dermoscopic examination (using the DermLite Foto; 3Gen, LLC, Dana Point, CA, U.S.A.) of six patients with haemosiderotic or aneurysmal dermatofibromas was performed to evaluate specific dermoscopic criteria.
A multicomponent pattern with a central bluish or reddish homogeneous area in combination with white structures and a peripheral delicate pigment network along with vascular structures was noted in five of six lesions.
This dermoscopic pattern yielded the diagnosis of haemosiderotic or aneurysmal dermatofibroma in most cases. However, this multicomponent pattern may present in some melanomas and although it is useful in determining a clinical diagnosis of aneurysmal dermatofibroma, it may not be specific to this entity.
A case with a pigmented skin lesion that was diagnosed as a blue naevus on clinical and dermoscopic grounds and histopathologically confirmed as a dermatofibroma is presented. By means of this case, we define dermatofibroma as a new exception for 'homogeneous blue pigmentation' on dermoscopy.
Central white scarlike patch and a delicate pigment network at the periphery is the typical appearance of dermatofibromas on dermoscopy.
We aimed to analyze the different dermoscopic appearances of dermatofibromas.
Fifty-two dermatofibromas with dermoscopic features different from the classic type, which were detected between May 2003 and July 2005, were evaluated.
Globules in the scarlike area (38.5%), linear/irregular crypts (26.9%), lentigo-like reticular pigmentation (23%), homogeneous blue-gray pigmentation (5.9%), and erythematous homogeneous area surrounding the white patch (3.8%) were the patterns observed. In addition, dermoscopic features of an atrophic variant of dermatofibroma are described, which was seen in one lesion (1.9%).
None.
Dermatofibromas have various dermoscopic features. Among them, "linear, irregular crypts" is not unusual and is described in this study. Homogeneous bluish pigmentation on dermoscopy may be a possible clue for the diagnosis of hemosiderotic variants of dermatofibromas.