Acral Pseudolymphomatous Angiokeratoma Research Articles

A Rare Case Report of T-Cell Rich Angiomatoid Pseudolymphoma.

Dear Editor, Cutaneous pseudolymphoma (PSL) is a reactive lymphoproliferation that histopathologically and/or clinically imitates cutaneous lymphomas. It can be either B-cell or T-cell predominant, and they are further divided into four main categories. Our case falls under the category of T-cell rich angiomatoid PSL.[1] An 86-year-old male presented with multiple, itchy skin lesions over his face, forearms, back, and legs for the past eight months. He was a known case of type 2 diabetes mellitus and right radical nephrectomy was done for Grade II right clear cell renal cell carcinoma six years ago. Cutaneous examination revealed multiple, erythematous infiltrated papules and plaques over the forehead [Figure 1] and lateral aspect of the face. A few hyperpigmented papules with central crusting were seen over bilateral legs and lower back. Angiolymphoid hyperplasia with eosinophilia (ALHE) and Jessner lymphocytic infiltrate of the skin were considered as differential diagnoses. Histopathology of an erythematous infiltrated papule over the forehead revealed a grenz zone and a fairly defined lesion in the dermis [Figure 2]. Many vascular channels lined by hyperplastic endothelial cells were seen in the lesion. Perifollicular mixed inflammatory cell infiltrates comprising histiocytes, lymphocytes, and a few neutrophils were also seen [Figure 3]. IHC showed lymphocytes with strong membranous staining for CD 3 [Figure 4a], and a few showed CD 20 staining [Figure 4b]. A diagnosis of T-cell-rich angiomatoid PSL was made based on histopathology and IHC.Figure 1: Multiple erythematous, few hyperpigmented infiltrated papules, and plaques seen over foreheadFigure 2: Grenz zone (black arrow) and a fairly defined lesion is seen in the dermis (H and E, 10×)Figure 3: The lesion in the dermis shows many vascular channels lined by hyperplastic endothelial cells (red stars). Perifollicular mixed inflammatory cell infiltrates comprising histiocytes, lymphocytes, and few neutrophils were also seen in the lesion (H and E, 100×)Figure 4: (a) Strong membranous staining of the lymphocytes for CD3. (b) Few lymphocytes show CD 20 staining (IHC)Cutaneous PSL is restricted to cases that histopathologically simulate cutaneous lymphomas but do not fit into any other diagnosis after clinical correlation. Some of the etiological factors of PSL include infections caused by bacteria, viruses, parasites, drugs such as anticonvulsants and antihypertensives, foreign agents such as tattoo dyes, injectable vaccinations, insect bites, and ultraviolet radiation. Cutaneous PSL is divided into four main groups based on histopathologic features and clinical data, which include nodular PSLs, PSLs as simulators of Mycosis fungoides (“pseudo-MF”) and of other CTCLs, other PSLs, and intravascular PSLs. Our case clinically and histopathologically fits into the other PSLs group, which comprises T-cell rich angiomatoid PSL and cutaneous plasmacytosis. Acral Pseudolymphomatous Angiokeratoma of Childhood (APACHE), T-cell-Rich Angiomatoid Polypoid Pseudolymphoma (TRAPP), primary cutaneous angioplasmocellular hyperplasia, and lymphoplasmacytoid plaque (LPP) are all forms of T-cell rich angiomatoid PSL.[1] Our case did not show any eosinophils in the inflammatory infiltrate which are characteristically observed in ALHE and the histopathological features observed in our case does not correlate with Jessner lymphocytic infiltrate of the skin. Histopathologically, APACHE and TRAPP were considered as differential diagnoses, but features were more suggestive of TRAPP. Microscopically, TRAPP shows numerous vessels with plump endothelia, a grenz zone, and a dense dermal infiltrate of lymphocytes with a predominance of CD3 over CD20 cells, as seen in our case. TRAPP usually presents as a solitary polypoidal lesion, but in our case it presented atypically as multiple grouped erythematous papules.[2,3] Review of a series of 19 cases showed that a variety of names have been suggested such as APACHE, TRAPP, angiolymphoid hyperplasia with high endothelial venules (ALH-HEV), etc., for lesions sharing similar features such as vascular proliferation with a dense lymphoid infiltrate. They constitute a spectrum of vascular lesions with considerable histopathological and clinical overlap.[4] A case of PSL of the liver in association with renal cell carcinoma occurring 14 months after right radical nephrectomy has been reported.[5] However, our patient developed PSL of the skin post-nephrectomy. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Adult-onset severe acral angiokeratoma-like pseudolymphoma: A case report and review of the literature.

Adult-onset severe acral angiokeratoma-like pseudolymphoma: A case report and review of the literature.

A Nodular GLUT1 Positive Verrucous Haemangioma Arising Over an Acral Pseudolymphomatous Angiokeratoma of Children (APACHE): A Diagnosis of Rarity.

A Nodular GLUT1 Positive Verrucous Haemangioma Arising Over an Acral Pseudolymphomatous Angiokeratoma of Children (APACHE): A Diagnosis of Rarity.

儿童肢端假性淋巴瘤样血管角皮瘤一例临床病理特征

儿童肢端假性淋巴瘤样血管角皮瘤一例临床病理特征

Inflammatory lobular hemangioma: A vascular proliferation with a prominent lymphoid component. Review of a series of 19 cases

In the last 30 years, there has been a strong interest in vascular proliferations. Pyogenic granuloma was not only renamed lobular capillary hemangioma, but also the conceptual interpretation was also changed from an overgrowth of granulation tissue to a genuine hemangioma (or benign vascular neoplasm). We describe 19 cases of patients who presented clinically with a vascular lesion, characteristically a pyogenic granuloma or lobular hemangioma, where the histopathological findings led to the pathologic concern for a lymphoma of the skin. These benign lesions with a dense lymphoid infiltrate were further defined on the basis of different vascular and lymphoid immunohistochemical markers as inflammatory lobular hemangiomas. We propose that given the considerable histopathological overlap between acral pseudolymphomatous angiokeratoma, T-cell rich angiomatoid polypoid pseudolymphoma of the skin, and other designations of some of these vascular proliferations with a rich and dense lymphoid infiltrate, they might constitute a spectrum of vascular lesions with varying clinical presentations.

A double dose of pseudolymphoma: Cutaneous intravascular CD30+ pseudolymphoma arising in acral pseudolymphomatous angiokeratoma – a case report

Cutaneous intravascular CD30+ pseudolymphoma is a rare entity with only a handful of cases reported thus far in the literature. This case study reports a patient who presented with a non-healing wound of the forearm, which on excision biopsy was found to have the histological features of an acral pseudolymphomatous angiokeratoma, also known as papular angiolymphoid hyperplasia. Distended lymphatics within the lesion were filled with a monotonous population of intermediate sized lymphocytes with distinct nucleoli and considerable associated apoptotic debris. Immunoperoxidase staining showed almost uniform staining for CD4 and around 50% staining for CD30. The occurrence of cutaneous intravascular CD30+ pseudolymphoma has previously been reported in the setting of a regressing keratoacanthoma of the cheek1 and has been associated with chronic inflammation after trauma.2 In this case the finding was interpreted as a florid reactive hyperplasia, with the recommendation for follow up and T-cell clonality studies should recurrence occur. To our knowledge, no recurrence has occurred 20 months post initial presentation. Awareness of this benign cutaneous lymphoproliferation as a reactive process is necessary in distinguishing it from malignant intravascular T-cell lymphomas.

Expression of Peripheral Node Addressins by Plasmacytic Plaque of Children, APACHE, TRAPP, and Primary Cutaneous Angioplasmacellular Hyperplasia.

High-endothelial venules are a common feature of 3 types of cutaneous pseudolymphomas: pretibial lymphoplasmacytic plaque (PLP) of children, acral pseudolymphomatous angiokeratoma of children (APACHE), and T-cell rich angiomatoid polypoid pseudolymphoma (TRAPP). In addition, primary cutaneous angioplasmacellular hyperplasia (PCAH) overlaps with these other 3 conditions. We intend to study the expression of peripheral node addressins in PLP, APACHE, TRAPP, and PCAH. We studied 1 case of PLP, 2 cases of APACHE, 2 cases of TRAPP, and 2 cases of PCAH. Immunostainings for MECA-79 and WT-1 were obtained in all cases. All cases showed a dense lymphohistiocytic dermal inflammatory infiltrate with abundant plasma cells. In addition, HEV were prominent in all cases. Cases of PLP, APACHE, and TRAPP expressed MECA-1. Cases of PCAH did not express MECA-1. Although PLP, APACHE, and TRAPP seem to fall under the same morphologic spectrum with different clinical representations, PCAH seems to be a different entity, with histopathologic peculiarities and a different immunophenotype.

Acral pseudolymphomatous angiokeratoma of children (APACHE) – a case report

Acral pseudolymphomatous angiokeratoma of children (APACHE) – a case report

Papular angiolymphoid hyperplasia and lymphoplasmacytic plaque: a clinical and histological spectrum

Acral pseudolymphomatous angiokeratoma of children (APACHE) is a rare form of cutaneouspseudolymphoma characterized byangiomatous papules with a predilection for the acral regions of children. Classically, a densedermal lymphocytic infiltrate composed of both T and B cells is seen in histological specimens,together with prominent vessels lined by plump endothelial cells. Increasing evidence suggests thatthis condition is neither necessarily acral, pseudolymphomatous, nor angiokeratomatous. It may notalways be a pediatric disease. Therefore, the correctness of its nomenclature has been questioned.Herein, we report threecases whose clinical and histological features were consistent with the diagnosis of APACHE. To ourknowledge, this is the first report of APACHE from Southeast Asia. We also discuss why we believe"APACHE" to be a misnomer and support "papular angiolymphoid hyperplasia" as a more accurate andencompassing term. In addition, we illustrate a case with significant overlapping features withlymphoplasmacytic plaque in children, suggesting that both entities may exist on a clinical andhistological spectrum.

Lymphoplasmacytic Plaque in Children.

Dear Editor: Fried et al.1 reported on a specific disease entity using the term Pretibial lymphoplasmacytic plaque in children. Herein, we describe a 3-year-old girl with a 6-month history of a solitary, asymptomatic, erythematous, scaly plaque with some papules over the right knee (Fig. 1A). She had a clean medical history with no history of travel abroad, injury, or insect bite. The biopsy specimen was characterized by focal parakeratosis, irregular acanthosis, and dense dermal lymphoid infiltrates, admixed with numerous plasma cells. The plasma cells extended into the deep dermis with a perivascular and periadnexal pattern, as previously reported by Ahn et al.2. The specimen also revealed a lichenoid reaction with basal vacuolization and lymphoid exocytosis, similar to those reported by Moulonguet et al.3 and Porto et al.4 (Fig. 1B, C). Fig. 1 (A) Solitary reddish scaly plaque on the right knee. (B) Punch biopsy shows dense inflammatory infiltrate in lichenoid arrangement with perivascular and periadenxal extension into the deep dermis (H&E, ×40). (C) Higher magnification revealing ... The immunohistochemical studies showed mixed inflammation of the B-cell and T-cell lymphocytes. Additionally, a polyclonal pattern was confirmed by the co-existence of kappa and lambda light chain-positive cells. No organisms were identified on the acid fast bacilli, Giemsa, and periodic acid-Schiff staining. A serologic test for syphilis and the QuantiFERON-TB Gold (Cellestis Limited, Carnegie, Australia) test both yielded negative results. Lymphoplasmacytic plaque (LPP) was originally termed isolated benign primary cutaneous plasmacytosis in as it was thought to be a part of the spectrum of primary cutaneous plasmacytosis (PCP)5. Several similar cases, which have been reported as LPP or under a different diagnosis, shared common clinical features including a solitary plaque with a predilection for the lower legs and prevalence in the pediatric age group4. These features were distinct from those of PCP, which is characterized by multiple red-brown plaques located on the trunk, mainly in adult Asian patients1. These previously reported cases, which we believe represent LPP, are described in Table 1. Histopathologically, LPP exhibited an irregular acanthosis with overlying parakeratosis and dermal lymphoplasmacytic infiltrates, with or without epitheloid granulomas. The localized inflammation and epidermal hyperplasia may contribute to the papulosquamous morphology of LPP. Clinically, the plaque seemed to be a benign chronic condition, although there was no effective treatment other than complete excision (Table 1). Table 1 Previously reported cases showing the same findings with lymphoplasmacytic plaque in children Moulonguet et al.3 differentiated LPP from acral pseudolymphomatous angiokeratoma of children, suggesting that these disease entities may reside on the spectrum of pseudolymphomas. Pseudolymphomas can develop after exposure to certain antigens. Interestingly, the histological pattern of a lichenoid inflammation with periadnexal infiltrate was very similar to those of lichen striatus, except for the heavy composition of plasma cells that is important for the diagnosis of LPP. Therefore, given the similarities in the age of onset and the histologic findings, we believe both conditions could be caused by a local reaction to an unknown, acquired stimulus in a specific age group. In conclusion, although the pathophysiology is still unknown, LPP is a unique disease entity with typical clinical features. When a child presents with this type of plaque on the lower extremities, several disease entities including primary lymphoproliferative disorders, infectious lesions, other reactive infiltrates, or pseudolymphomas need to be considered. A final punch biopsy is necessary to confirm the diagnosis.

Papular pseudolymphoma of adults as a variant of acral pseudolymphomatous angiokeratoma of children (APACHE)

JDDG: Journal der Deutschen Dermatologischen GesellschaftVolume 12, Issue 5 p. 423-424 Clinical Letter Papular pseudolymphoma of adults as a variant of acral pseudolymphomatous angiokeratoma of children (APACHE) Gunnar Wagner, Corresponding Author Gunnar Wagner Department of Dermatology, Klinikum Bremerhaven Reinkenheide, Bremerhaven Correspondence to Dr. med. Gunnar Wagner Department of Dermatology Klinikum Bremerhaven Reinkenheide Postbrookstraße 103 27574 Bremerhaven, Germany E-mail: gunnar.wagner@klinikum-bremerhaven.deSearch for more papers by this authorChristian Rose, Christian Rose Histological Laboratory, LübeckSearch for more papers by this authorMichael Max Sachse, Michael Max Sachse Department of Dermatology, Klinikum Bremerhaven Reinkenheide, BremerhavenSearch for more papers by this author Gunnar Wagner, Corresponding Author Gunnar Wagner Department of Dermatology, Klinikum Bremerhaven Reinkenheide, Bremerhaven Correspondence to Dr. med. Gunnar Wagner Department of Dermatology Klinikum Bremerhaven Reinkenheide Postbrookstraße 103 27574 Bremerhaven, Germany E-mail: gunnar.wagner@klinikum-bremerhaven.deSearch for more papers by this authorChristian Rose, Christian Rose Histological Laboratory, LübeckSearch for more papers by this authorMichael Max Sachse, Michael Max Sachse Department of Dermatology, Klinikum Bremerhaven Reinkenheide, BremerhavenSearch for more papers by this author First published: 14 March 2014 https://doi.org/10.1111/ddg.12291Citations: 2AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume12, Issue5May 2014Pages 423-424 RelatedInformation

Acral pseudolymphomatous angiokeratoma: case report and literature review

The authors describe a case of a female patient with Acral Pseudolymphomatous Angiokeratoma of Children, known as APACHE. It is a rare benign cutaneous disease, of unknown etiology, characterized by multiple, asymptomatic erythematous-violaceous papules and nodules, usually located unilaterally with acral distribution. Today, this denomination is questionable, since there are published reports of this disease in adults and in different locations. Clinically, it is similar to an angiokeratoma, whereas hystologically, it corresponds to a distinct type of pseudolymphoma. The immunohistochemical study is required to distinguish APACHE from cutaneous lymphoma.

Acral pseudolymphomatous angiokeratoma of childhood: A case report and review of the literature

Acral pseudolymphomatous angiokeratoma of childhood: A case report and review of the literature

Acral Pseudolymphomatous Angiokeratoma of Children

Acral pseudolymphomatous angiokeratoma of children (APACHE) is characterized by multiple angiomatous papules on the hands and feet in children. Here, we report a case of APACHE in a female patient followed up from 13 to 19 years of age with a dark red lesion on the center of the dorsum of the right thigh. Histologically, vacuolar alteration and exocytosis of lymphocytes, and specific dense cellular infiltration beneath the epidermis to the reticular dermis were found. On immunolabeling study, the lesion vessels were found to be positive for both the lymphatic endothelium-specific marker podoplanin and blood vessel-specific marker CD34. These findings suggested that APACHE is a type of vascular malformation.

T-cell-rich angiomatoid polypoid pseudolymphoma of the skin: a clinicopathologic study of 17 cases and a proposed nomenclature

We describe a series of previously unreported, distinctive, polypoid solitary T-cell-rich cutaneous pseudolymphomas. The clinicopathologic features were examined in 17 cases. Patient ages ranged from 16 to 71 years (mean = 38.5) with a female predominance (female : male = 14 : 3). All lesions, clinically diagnosed most often as pyogenic granuloma, presented as a solitary, polypoid, erythematous, papule ranging in size from 2.5 to 7.5 mm (mean = 5.8). Most occurred on the head and neck (7) and trunk (6) with other sites including the thigh (1), shoulder (1) and knee (1). A dense dermal infiltrate composed of mildly atypical lymphocytes with variable numbers of admixed plasma cells and histiocytes was prototypical. Commonly, there was an associated epidermal collarette (16/17), Grenz zone (11/17) or admixed eosinophils (8/17). Prominent vessels lined by plump endothelial cells, reminiscent of high endothelial venules of lymph nodes, were universal and some degree of telangiectasia was also common (12/17). CD3-positive T-cells consisted of an admixture of CD4-positive and CD8-positive forms (15/16). Multiple studies suggested polyclonality (seven cases). No recurrences after lesional excision were noted in the 17 patients with a follow-up range from 24 to 120 months (mean = 46.6). Although these lesions share histopathologic features of the so-called acral pseudolymphomatous angiokeratoma of children (APACHE), they occur in a completely different clinical setting, present in solitary and polypoid fashion and are T-cell rich. We propose the diagnostic label T-cell-rich angiomatoid polypoid pseudolymphoma for this distinctive but presumably reactive lesion.

Acral pseudolymphomatous angiokeratoma of children in an elderly man: report of a case and review of the literature

Acral pseudolymphomatous angiokeratoma of children (APACHE) is clinically characterized by angiomatous papules that preferentially affect acral areas of children. It is currently believed to be a variant of pseudolymphoma rather than angiokeratoma. Histopathologically, a mixed inflammatory cell infiltrate comprising B- and T-lymphocytes and a proliferation of thick-walled blood vessels are the hallmark. In the literature, although more adult cases are being described, the clinical, epidemiologic and histopathologic data of this presentation is scant. We report a 76-year-old man who presented with a 5-year history of progressively increasing asymptomatic dusky dome-shaped papules and plaques on his upper and lower extremities. Histology was consistent with APACHE. After reviewing all the adult cases in the literature and comparing them to the classical variant in children, we found no significant difference warranting separating these two variants. We prefer the designation "papular angiolymphoid hyperplasia" to APACHE as it encompasses all the childhood and adult cases involving acral and nonacral sites and highlights the histological and sometimes clinical similarities this entity shares with angiolymphoid hyperplasia with eosinophilia.

Pseudolymphomatous angiokeratoma: report of three cases and an immunohistological study

Pseudolymphomatous angiokeratoma (PA), originally termed 'acral pseudolymphomatous angiokeratoma of children', is a disorder characterized clinically by development of red nodules on the extremities and histologically by a subepidermal dense lymphocyte infiltrate. We report three cases of PA, with characteristically dense, nodular infiltrate composed predominantly of small lymphocytes, and thick-walled vessels. Immunohistochemical investigation revealed a dense accumulation of CD20+ cells with CD3+ cells in one case. Infiltrate in the other two cases was mainly composed of CD3+ cells and a mixture of CD4+ and CD8+ cells, with a few cells expressing CD20. Our immunohistological results reveal a wide spectrum of cellular infiltrate compositions ranging from T-cell to B-cell predominance.

Acral pseudolymphomatous angiokeratoma of children (APACHE)

13-year-old girl presented with a 10-year history of multiple discrete red firm papules situated on her right upper arm. The histopathology examination revealed a massive subepidermal lymphohistiocytic infiltrate, consistent with acral pseudolymphomatous angiokeratoma of children. Treatment with topical application of mometasone furoate 0.1% cream once daily for 6 months improved the lesions partially. However, these papules returned to their pretreatment appearance and consistency within 6 months after the cessation of the topical corticosteroid. The authors prefer the term 'papular angiolymphoid hyperplasia' for this entity, based on the absence of histological angiokeratomatous features, the benign nature of the lesion and the previously reported cases from adults and non-acral sites.

Clinicopathologic Study of Cutaneous Pseudolymphomas

A study was conducted to determine the clinical and histopathologic findings of cutaneous pseudolymphomas. In the period between July of 1989 and December of 2002, 42 cases were collected. Clinical records and pathology slides of the patients were reviewed retrospectively. A striking common clinical feature was a pruritic papule, nodule, or plaque with a smooth surface on the head and neck. The histologic patterns were diverse, MF-like, perivascular/periadnexal, nodular, diffuse, pseudolymphomatous folliculitis, and acral pseudolymphomatous angiokeratoma of children (APACHE) patterns. The most common pattern was a multinodular pattern with an extension into the deep dermis and subcutaneous adipose tissue. The differentiation between cutaneous lymphomas and pseudolymphomas must depend on a constellation of criteria, never on a single criterion.

Akrale pseudolymphomat�se Angiokeratome der Kindheit (APACHE)

Acral pseudolymphomatous angiokeratoma of children (APACHE) is a pseudolymphoma of posttraumatic origin which was first described in 1988. We report on a female patient with APACHE following a traumatic abrasion on her foot. Reddish papules developed that histologically were characterized by a wedge-shaped inflammatory infiltrate rich in capillaries. Conservative treatment was not successful.