Poikiloderma Atrophicans Vasculare Research Articles

LB936 Cutaneous T-cell lymphoma (CTCL) following B-cell lymphoma: A case series

Cutaneous T-cell lymphoma (CTCL) is associated with an increased risk of developing secondary malignancies, particularly lymphomas. However, the diagnosis of lymphoma prior to CTCL is very rare. Herein we present the cases of two patients diagnosed with lymphoma prior to a diagnosis of CTCL. The first patient was a female in her late 50s with a history of diffuse large B-cell lymphoma (DLBL) of her breast status post chemo plus radiation who developed poikilodermatous scaly patches on her arms and legs one year after her DLBL diagnosis. Her condition was initially managed as an eczema refractory to treatment until a biopsy performed at an outside clinic several years later revealed likely CTCL followed by confirmative biopsies demonstrating poikiloderma atrophicans vasculare. She was treated with acitretin 10 mg five times weekly and narrowband UVB twice weekly for 12 treatments, but self-discontinued both. She recently initiated monthly extracorporeal photopheresis. The second patient was a female in her 70s with history of melanoma who presented to outpatient dermatology for evaluation of erythematous macules on her groin pending radiation treatment for DLBL of her breast. The rash was refractory to topical therapies and a biopsy later demonstrated mycosis fungoides. The patient initially preferred conservative therapy with natural sunlight and topical hydrocortisone, but ultimately elected for monthly photopheresis as her disease progressed. The response of both patients to photopheresis is yet to be determined. These cases illustrate the importance of full workup for erythematous, scaly rashes especially in the setting of other malignancy and in rashes refractory to topical treatments for other conditions. Both cases had at least one biopsy unrevealing of the true underlying CTCL diagnosis, showing that multiple biopsies may be necessary to capture this diagnosis.

Erythroderma and extensive poikiloderma \u2013 a rare initial presentation of dermatomyositis: a case report

BackgroundDermatomyositis is a humoral-mediated inflammatory myopathy with symmetrical proximal muscle weakness and dermatological manifestations such as Gottron’s papules, heliotrope rash, periungual abnormalities, and flagellate erythema. Erythroderma is a severe and potentially life-threatening dermatological condition with diffuse erythema and scaling involving more than 90% of the skin surface area. Poikiloderma vasculare atrophicans refers to mottled hyperpigmentation and hypopigmentation of the skin with in-between telangiectases and areas of atrophy and is considered a variant of mycosis fungoides. Poikilodermatomyositis is the term given to the condition with poikiloderma and inflammatory myopathy. Only a few cases are reported on erythroderma in dermatomyositis and poikilodermatomyositis. Erythrodermal pattern of dermatomyositis transforming into poikilodermatomyositis is a recognized rare manifestation of dermatomyositis and we could find only one case report in the literature.Case presentationA 53-year-old Sri Lankan woman presented with intermittent fever of 5 months’ duration with erythroderma. Later she developed progressive, symmetrical proximal muscle weakness. Following a short course of small dose steroids, erythroderma settled but changed to extensive poikiloderma involving more than 90% of her skin with her face being relatively spared. She had an early heliotrope rash, shawl sign, and Gottron papules. Electromyography and muscle biopsy were supportive of inflammatory myositis and skin biopsy showed evidence of dermatomyositis. Inflammatory markers and muscle enzymes were also elevated. Autoimmune antibodies and myositis-specific autoantibodies were negative. She was started on orally administered prednisolone 1 mg/kg per day with methotrexate 10 mg once a week and had a good response to treatment with resolution of the skin condition and improvement of muscle power. Imaging studies, endoscopies, and tumor markers did not reveal any malignancy.ConclusionsThis case illustrates a rare presentation of dermatomyositis initially presenting as fever, erythroderma, and proximal muscle weakness and later developing poikiloderma involving more than 90% of the skin. It is important to be aware of this rare presentation to avoid misdiagnosis. With the currently available literature it is not possible to conclude that erythroderma is a bad prognostic factor in dermatomyositis or a predictive factor for a malignancy. Patients have a good response to steroids with a combination of immunosuppressants.

Usefulness of dermoscopy in poikiloderma vasculare atrophicans/parakeratosis variegata.

Poikiloderma vasculare atrophicans (PVA), also known as parakeratosis variegata, is considered a rare variant of early-stage mycosis fungoides or a premycotic condition usually presenting as asymptomatic or moderately pruritic, mildly scaling, reddish-brown papules, often coalescing into net-like patterned lesions, which mainly involve the breast, abdomen, buttocks, and flexures [1, 2]. Although the diagnosis of this condition is usually straightforward, sometimes the distinction from other similar [...]

Poikiloderma vasculare atrophicans: a distinct clinical entity?

This paper describes a typical case of poikiloderma vasculare atrophicans (PVA) in a 48-year-old female. Histologically, the features were suggestive of PVA with the absence of Pautrier's microabscess or atypical lymphoid cells. The biopsy specimen was positive for cluster of differentiation (CD) 8 on immunohistochemical staining. Its exact pathogenesis remains obscure, and it remains unclear whether PVA actually is mycosis fungoides (MF), a forme fruste of MF, or a distinct and benign dermatosis with CD8+ phenotype that can perhaps be labeled as PVA. However, it has a long benign clinical course without progression to tumor stage of MF in most cases, and its status within the spectrum of cutaneous T-cell lymphoma remains poorly understood. Yet it is imperative to distinguish PVA from poikilodermic MF.

Poikilodermatous mycosis fungoides

Poikilodermatous mycosis fungoides (MF) is a variant of MF, formerly referred to as poikiloderma vasculare atrophicans. The lesions are classically characterized by large plaques of hypopigmentation and hyperpigmentation with atrophy and telangiectases. The plaques may be asymptomatic or mildly pruritic and typically involve the major flexural areas and trunk. Poikilodermatous MF has an early stage (IA-IIA) at diagnosis and a male predominance. Poikilodermatous MF shows an atypical T-cell infiltrate in the papillary dermis with evidence of epidermotropism, epidermal atrophy, dilated blood vessels in the dermis, melanophages, and melanin incontinence. Recent studies suggest a predominance of a CD8+, CD4- immunophenotype. Treatment modalities are similar to classic MF with phototherapy being the most common first-line therapy. Poikilodermatous MF has an excellent prognosis.

A Case of Poikiloderma Vasculare Atrophicans

Poikiloderma vasculare atrophicans (PVA) is a rare variant of mycosis fungoides, and is characterized by generalized hyperkeratotic scaly papules in net-like, retiform, or zebra-like patterns. A 59-year-old Korean woman presented with asymptomatic, erythematous-to-violaceous, reticulated confluent papules on the trunk and extremities. Skin lesions were initially limited to both thighs 25 years ago, and then spread slowly over her body. Histopathological examination showed band-like inflammatory infiltrations and epidermotropism consisting of mostly CD8+ lymphocytes. Based on the clinical manifestations and histological findings, the diagnosis of PVA was made. We herein report on a case of PVA, which featured a long-benign course without progression into the tumor stage over a period of 30 years.

Poikilodermatous parapsoriasis - two cases with review of literature

Poikilodermic parapsoriasis is a rare varaint of poikiloderma where the chances of going into mycoses fungoides exists.But not all cases do go to mycoses. In the early stage of poikiloderma atrophicans vasculare, without respect to its course, shows moderate thinning of stratum malpigji, effacement of rete ridges, hydropic degeneration of basal cells. In the upper dermis-there is band like infiltrate which in places invades the epidermis. The infiltrate consists of a few histiocytes-so called initial 'lichenoid pattern'. In addition there is abundance of melanophages with pigment incontinence. In late stages there is thinning of is with hydropic degeneration of basal cells. In MF inflammatory infiltrate increases rather than decreases in time. Large hyperchromatic nuclei, so called 'mycoses cells' are likely to be present in this variant and often there is moderate to marked epidermotropism. It may result in Pautrier microabscess. formation.The diagnosis of Mycoses Fungoides is supported by presence of an aberrant phenotype, for example T cell lacking one or more pan T cell antigens. Most commonly absent antigen is CD-7 followed by CD-2, CD-3 and CD-5³ . Making a differential diagnosis between early MF and parapsoriasis is often difficult at clinical and histological level. We report two cases of poikilodermic parapsoriasis, both showing some degree of epidermotropism ,but one had panT cell marker CD 3, CD5, CD7 present and other absent.The lady with Pan T cell markers did not progress to frank mycoses fungoides while the patient with absent markers progressed rapidly and finally succumbed.

A case of poikiloderma vasculare atrophicans, a rare variant of cutaneous T-cell lymphoma, responding to extracorporeal photopheresis

A case of poikiloderma vasculare atrophicans, a rare variant of cutaneous T-cell lymphoma, responding to extracorporeal photopheresis

Mycosis fungoides: classic disease and variant presentations.

Mycosis fungoides is a peripheral non-Hodgkin's T-cell neoplastic process, representing the most common type of primary cutaneous malignant lymphoma. Neoplastic lesions classically show skin predilection and characteristic clinical and histologic features in patch, plaque, and tumor stages. In addition, several clinicopathologic variants of mycosis fungoides have been delineated, including poikiloderma atrophicans vasculare (parapsoriasis variegata), Sézary syndrome, granulomatous mycosis fungoides, hypopigmented mycosis fungoides, folliculocentric mycosis fungoides, syringotropic mycosis fungoides, and Woringer Kolopp disease. We will review the salient features of patch, plaque, and tumor stage mycosis fungoides in this article and follow with a discussion of these variant clinicopathologic presentations and of therapeutic modalities.

Mitochondrial complex I and III mutations and neutral-lipid storage in activated mononuclear macrophages and neutrophils: A case presenting with necrotizing myopathy, poikiloderma atrophicans vasculare, and xanthogranulomatous bursitis

We report the case of a 57-year-old woman suffering from xanthogranulomatous bursitis, necrotizing myopathy, and poikiloderma atrophicans vasculare, which are associated with marked accumulation of neutral-lipid storage phagocytes. The observed lipid storage was restricted to activated phagocytes independent of the presence of tissue necrosis and was not seen either in circulating blood leukocytes or in muscle fibers. The patient's daughter disclosed xanthomatous inflammatory reaction with profound delay of wound healing secondary to pelviscopy. Examination of the mitochondrial DNAs of the patient, her daughter, and her two grandchildren revealed two homoplasmic mutations at positions 13708 and 15257 of the mitochondrial genome. We discuss the involvement of these mutations in the pathogenesis of xanthomatous and xanthogranulomatous inflammation. Further investigations are required to test whether impairment of aerobic energy production independent from mitochondrial DNA mutations (eg, by hypoxia or microbial toxins) similarly can cause the accumulation of lipid-laden macrophages and explain the persistency of xanthogranulomatous inflammation.

Cutaneous T-cell lymphoma (parapsoriasis en plaque). An association with pityriasis lichenoides et varioliformis acuta in young children

Pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC) are related benign disorders without recognized association with cutaneous T-cell lymphoma (CTCL). We report the cases of two children with documented PLEVA evolving into CTCL over several years. One child had the clinical lesions of PLC but the dermatopathologic findings of PLEVA at age 2 years. At age 12 years, he had skin changes of poikiloderma atrophicans vasculare and dermatopathologic findings consistent with parapsoriasis en plaque. The second child presented at age 7 years with scaling dermatitis and dermatopathologic findings of PLEVA. At age 12 years, the histologic diagnosis was parapsoriasis. Monoclonal antibody studies performed on biopsy specimens from both patients revealed 70% to 100% cells staining with CD5, 80% to 90% staining with CD4, 30% to 50% staining with CD8, and an increase in CD1-staining cells in the papillary dermis, indicating a predominantly helper T-cell infiltrate. We believe that PLC and PLEVA may be part of the spectrum of CTCL. Furthermore, CTCL may be more common in young children than once thought.

Diffuse, progressive hyperpigmentation: An unusual skin manifestation of mycosis fungoides

Pigmentary changes in mycosis fungoides usually occur in association with poikiloderma atrophicans vasculare or following therapy and regression of lesions. Several cases of hypopigmented mycosis fungoides have also been reported. We present the case report of a patient who developed pruritic, diffuse macular hyperpigmentation of the skin. Biopsy specimens from hyperpigmented skin revealed histologic and ultrastructural features typical of mycosis fungoides. Giant melanin granules were found in the tumor cells, as well as in keratinocytes and Langerhans cells. As far as we know, this is the first report of cutaneous hyperpigmentation as a single presenting sign of mycosis fungoides.

The Nature of Mycosis Fungoides

Thirty-four patients with a clinical diagnosis of plaque stage mycosis fungoides, poikiloderma atrophicans vasculare (poikiloderma) and parapsoriasis en plaques (parapsoriasis) were investigated for evidence of extracutaneous disease using a biochemical screen, blood films, bone marrow examination, chest radiograph, abdominal ultrasound, isotope liver scan, liver biopsy and lymphangiography. Skin biopsies were also taken from these patients and, in order to rule out non-specific histological change, from 29 controls with inflammatory skin disease; the slides were examined blind by two independent observers for evidence of mycosis fungoides or poikiloderma and graded accordingly. Both observers were able to differentiate significantly histological grades of mycosis fungoides corresponding to the clinical diagnosis although exact histological grades only corresponded on 35 per cent of slides. Features graded as classical or probable mycosis fungoides were found in between 22 and 67 per cent of patients with parapsoriasis and between 67 and 100 per cent of those with poikiloderma. We found no evidence of extracutaneous disease in any of our patients. Lymphangiograms were abnormal in 30 of 31 examinations but the changes were non-specific and did not correspond to disease type, duration or extent. Four patients had apparently unrelated co-existing disease including chronic lymphatic leukaemia in two, a monoclonal gammopathy and autoimmune haemolytic anaemia. This study shows that in its early stages mycosis fungoides is predominantly if not primarily a cutaneous disease. The findings also suggest that parapsoriasis and poikiloderma are part of the same disorder as mycosis fungoides or evolve into it. Aggressive treatment to prevent progression to mycosis fungoides plaque and tumour stage should therefore be tried. The findings support the idea that mycosis fungoides is a reactive rather than a neoplastic disorder.

Two first cousins with spondyloepiphyseal dysplasia tarda (X linked recessive form), one also with poikiloderma atrophicans vasculare progressing to lymphocytic lymphoma

Two male cousins with spondyloepiphyseal dysplasia tarda (X linked recessive form) are described. One presented with a poorly differentiated lymphocytic lymphoma of the skin in areas of poikiloderma atrophicans vasculare...

An ultrastructural study of the mononuclear cell infiltrate of mycosis fungoides and poikiloderma atrophicans vasculare.

Summary Tissue from twenty-five patients with mycosis fungoides and three patients with poikiloderma atrophicans vasculare was examined ultrastructurally. Characteristic abnormal lymphoid cells were conspicuous in all biopsies comprising 30–60% of the cutaneous infiltrate. These cells possessed irregular nuclear outlines presenting a spectrum from mild indentation to grossly cerebriform shapes. The other prominent cell type was the histiocyte which occasionally appeared abnormal. Bizarre histiocytic cells resembling the interdigitating reticulum cell of lymph node T cell areas were also observed. In the epidermal infiltrate, abnormal lymphoid cells were seen singly infiltrating between keratinocytes and less frequently in groups, forming Pautrier microabscesses, or in close apposition to histiocytes. Langerhans cells appeared in normal numbers and were usually isolated but were occasionally seen in contact with lymphoid cells.

Acid alpha-naphthyl acetate esterase staining of T lymphocytes in human skin

T lymphocytes were stained in order to disclose alpha-naphthyl acetate esterase activity (ANAE staining) by adopting the method described by Mueller et al. In ANAE-staining of frozen sections from human lymph nodes, more than 90% of the lymphocytes in paracortical areas (T cell areas) were ANAE-positive, but in cortical follicles (B cell areas) less than 5% of the cells were positive. Lymphocytic infiltrations in various dermatoses (lichen ruber planus, psoriasis, SLE, atropic dermatitis, erythema multiforme, poikiloderma atrophicans vasculare, and Sézary syndrome) were investigated. A high percentage of ANAE-positive lymphoid cells (greater than 80%) was found in most cases. One patient with chron. lymphatic leukaemia, however, had a smaller proportion of ANAE-positive lymphocytic cells in an erythema multiforme skin infiltrate. ANAE staining seems to be an easy method for the identification of T lymphocytes in skin sections. The results of this investigation support the hypothesis that T lymphocytes have an affinity to skin.

Clinical meeting of the St John's Hospital Dermatological Society: 3 February 1977 Poikiloderma atrophicans vasculare with cutaneous epitheliomata

Clinical meeting of the St John's Hospital Dermatological Society: 3 February 1977 Poikiloderma atrophicans vasculare with cutaneous epitheliomata

CELL‐MEDIATED IMMUNITY IN MYCOSIS FUNGOIDES*

ABSTRACTThe immunologic status of patients with mycosis fungoides in various stages was studied by testing skin reaction to PPD, by sensitization with topical application of 2,4 dinitrochlorbenzene, and by in vitro lymphocyte transformation test with PHA. Skin reaction to PPD was depressed in 7 of 8 patients and sensitization to dinitrochlorbenzene was impaired in 6 of 8 patients. In PHA‐induced lymphocyte transformation, when autologous serum was added to the culture medium, the transformation rate was below normal in 7 of 8 patients. When autologous serum was replaced by normal serum, the rate was usally improved and, in 3 patients, recovered to normal range. However, in the remaining 4 patients of whom 3 were at the infiltrative plaque stage, the rate was still below normal (21.5–36.6%). It is suggested that at least in 3 patients there was a serum factor which lymphocyte transformation by PHA. In 2 patients with poikiloderma atrophicans vasculare and parapsoriasis en plaques, no influence of serum was noted.

Poikiloderma atrophicans vasculare as a pointer to reticulosis of the skin.

Summary A case of reticulosis of the skin (mycosis fungoides) terminating in reticulum cell sarcoma is described where the features of poikiloderma atrophicans vasculare had been present for 40 years. The likelihood of reticulosis eventually developing after such a change in the skin is believed to be around 50%.

DYSKERATOSIS CONGENITA AND FAMILIAL PANCYTOPENIA.

Four or possibly five males in a kinship group had features of dyskeratosis congenita, a syndrome comprising poikiloderma atrophicans vasculare, dystrophia unguium, and leukoplakia oris. In addition, three members of the kinship demonstrated hematologic changes compatible with the hypoplastic anemia described by Fanconi. Based on the hemopoietic disturbance found in these three cases and that observed in seven similar cases from the literature, it is believed that the abnormalities associated with dyskeratosis congenita should be considered a further variant of the diverse congenital defects encompassed by the syndrome of Fanconi's familial pancytopenia.