Pigmented purpuric dermatosis may have tcell gene rearrangements that may or may not be associated with mf. Mycosis fungoides nord national organization for rare. Pdf pigmented purpuric dermatosis or mycosis fungoides. A rare presentation of a great masquerader adel alabdulrazzaq, md, valid bagher zadeh, md, sultan alotaibi, md, nabeel najem, md. Some of the lesions show the characteristic orangebrown, speckled, cayenne pepperlike discoloration that is the hallmark clinical sign of a. Finally, pigmented purpuric dermatosis ppd is considered by the authors as worthwhile because of the increasing evidence linking this group of disorders to cutaneous lymphoma. In individuals with mycosis fungoides, the skin becomes infiltrated with plaques and nodules that are composed of lymphocytes. Quality nursing care is necessary for effective diagnosis and treatment of patients with mfctcl. Capillaritis as a potential harbinger of cutaneous tcell lymphoma angela shen md a, brandie j metz md a, tosten ehrig md b, prasanna sinkre md b, and sylvia hsu md a dermatology online journal 10 2.
Symptoms include rash, tumors, skin lesions, and itchy skin. Mycosis fungoides mf is the most common primary cutaneous t cell lymphoma, which is characterised in its early stages by epidermotropism of small to mediumsized t lymphocytes with cerebriform nuclei. Mimics of mycosis fungoides uma sundram, md, phd professor of pathology oakland university william beaumont school of medicine. There are reports of mycosis fungoides manifesting as pigmented purpura as well as progression of ppd to cutaneous tcell lymphoma. Pigmented purpuric dermatosis is a condition involving the skin on the lower limbs resulting from red blood cell extravasation from the superficial vascular plexus.
A rare presentation of a great masquerader adel alabdulrazzaq, md, valid bagher zadeh, md, sultan alotaibi, md, nabeel najem, md department of dermatology, adan hospital, kuwait. Mycosis fungoides mf can raise as pigmented purpuric dermatoses. Earlystage mfctcl stages ia and ib is most often managed in both. Mycosis fungoides, pigmented purpuric dermatosis, t cell clonality.
Pigmented purpura like eruption as cutaneous sign of mycosis fungoides with autoimmune purpura. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma in adults and children. Originally described by alibert in 1806, mf is classically a disease of adults, although children and adolescents can be affected, and it typically has a protracted, indolent course. Two skin biopsies were performed, with the diagnosis of pigmented purpuric dermatosis. Mycosis fungoides causes, symptoms, diagnosis, stages. Aug 11, 2016 the pigmented purpuric dermatoses form a group of chronic skin diseases with a characteristic appearance. Pigmented purpuric dermatosis genetic and rare diseases. There are numerous clinical and histopathological variants of mycosis fungoides including a folliculotropic variant figure 6, a granulomatous variant granulomatous slack skin syndrome, and variants that mimic common dermatoses such as pigmented purpuric dermatitis and vitiligo.
Mycosis fungoides pictures, staging, prognosis, symptoms. Oct 05, 2016 the main features of pigmented purpuric dermatosis ppd are petechiae tiny red spots due to broken blood vessels or purpura purplecolored spots or patches due to broken blood vessels, and yellow to brown pigmented patches. Aug 26, 2016 mycosis fungoides, sezarys syndrome and primary cutaneous peripheral tcell lymphomas not otherwise specified are among the most important subtypes of the ctcls. Mycosis fungoides is rarely cured, but some people stay in remission for a long time. The diagnosis of cutaneous tcell lymphoma ctcl requires accurate histopathology, including immunocytochemistry, as well as careful clinical appraisal and analysis for tcell clonality. Mycosis fungoides is an epidermotropic ctcl that evolves through distinct disease stages of patch, plaque, and tumor, often leading to transformation in the final stages. The pigmented purpuric dermatoses are a group of chronic diseases of mostly unknown etiology that have a very distinctive clinical appearance. Persistent pigmented purpuric dermatitis and mycosis fungoides. It generally affects the skin, but may progress internally over time. This course should be suspected when purpuric pigmented dermatitis is extensive, longlasting 1 year, has a reticular arrangement and negative patch testing. Purpuric mycosis fungoides the gulf journal of dermatology and. There are a number of known and proposed causes for the various forms of ppd. Pigmented purpuric dermatoses ppd, a group of vascular disorders with variable clinical picture is reported in all races and age groups with a male predilection.
In the differential diagnosis, allergic contact dermatitis may be hemorrhagic, mimicking capillaritis. Clinical examination revealed multiple annular purpuric patches on the abdomen, buttocks, and legs covering approximately 20% of the body surface area without lymphadenopathy or hepatosplenomegaly. Mycosis fungoides and ppd are similar so this the clinician should be suspected when purpuric pigmented dermatitis is extensive, longlasting 1 year, has a reticular arrangement and negative patchtesting 15. Capillaritis as a potential harbinger to cutaneous tcell.
Mycosis fungoides mf can present with purpuric lesions, and rare patients who seemed to have persistent pigmented purpuric dermatitis pppd have developed mf. Although they can arise on any part of the body, they are most commonly located on the lower legs. Hyperpigmented patch on the leg mdedge dermatology. The pigmented purpuric dermatoses ppds, also known as capillaritis, purpura simplex, and inflammatory purpura without vasculitis, are a group of chronic, benign, cutaneous eruptions characterized by the presence of petechiae, purpura, and increased skin pigmentation. Pigmented purpuralike eruption as cutaneous of mycosis fungoides with autoimmune purpura. Mycosis fungoides is a cutaneous tcell lymphoma that clinically presents as a single or multiple hypopigmented or hyperpigmented patches or as erythematous scaly lesions in the patch or plaque stage. In this situation, a longterm followup and treatments indicated in the early patch stage of mycosis fungoides puva therapy, chlormethine seem. The term pigmented purpuric dermatoses includes schamberg disease ie, progressive pigmentary dermatosis, purpura annularis telangiectodes majocchi disease, 1 lichen aureus, itching purpura, eczematidlike purpura of doucas and kapetanakis, and the pigmented purpuric lichenoid dermatosis of gougerot and blum. The spectrum of pigmented purpuric dermatosis and mycosis. It is characterized by multiple tan to reddish small macules socalled cayenne pepper spots that coalesce into tan to orange patches. Pigmented purpuric dermatoses information and causes patient. Pigmented purpuric dermatosis causes, symptoms, diagnosis. As such, ctcl includes both indolent and aggressive malignancies of varying clinical, histologic and immunophenotypic character.
Mycosis fungoides is the most common example of cutaneous t cell lymphoma. Mar 31, 2020 pigmented purpuric dermatitis affecting the trunk. Their cytotoxic effects on keratinocytes may result in. Mycosis fungoides is the most common type of ctcl, representing 4462% of cases. Mycosis fungoides presenting with pigmented purpuric rash jaad. Mycosis fungoides is an epidermotropic ctcl that evolves. Dermatologic examination revealed generalized purpuric patches with unclear borders primarily. Pigmented purpuric dermatosis may lie on a spectrum with mycosis fungoides mf. This condition may usually affect adults who are over 50 years of age but cases of children being affected have been reported 1, 2.
Thrombocytopenia, hypergammaglobulinemic purpura of waldenstrom, and the pigmented purpuric dermatitislike variant of mycosis fungoides also need to be excluded. Figure 1 from dermoscopy of early stage mycosis fungoides. The pathology of cutaneous tcell lymphoma cancer network. There are reports of mycosis fungoides manifesting as pigmented purpura as well as progression. In early stages, its often treated with medicines or therapies that target just your skin. Capillaritis pigmented purpuric dermatoses clinical gate. This is a rare presentation of a condition that is uncommon in the. Mycosis fungoides presenting as pigmented purpuric.
Capillaritis pigmented purpuric dermatoses plastic. Mycosis fungoides presenting as a pigmented purpuric dermatosis. A case of pigmented purpuric dermatosis similar to mycosis. Mycosis fungoides is a rare form of tcell lymphoma of the skin cutaneous. Aug 12, 2019 the pigmented purpuric dermatoses are a group of chronic diseases of mostly unknown etiology that have a very distinctive clinical appearance. Multiple annular purpuric patches on the abdomen, anterior thighs a, and right leg b. Given the clinical description this atypical epitheliotropic lymphocytic infiltrate is best categorized as purpuric mycosis fungoides arising in a background of pigmented purpuric dermatosis. There are reports of mycosis fungoides manifesting as pigmented purpura as well.
Other presentations include eczematous, psoriasiform, poikilodermatous, and hypopigmented patches. Pigmented purpuric dermatosis classification by phenotypic and. Mycosis fungoides presenting with pigmented purpuric rash. Pigmented purpuric dermatoses ppd characterized a group of capillaritis of obscure aetiology, manifested by localized or generalized, pigmented, petechial eruption. This course should be suspected when purpuric pigmented dermatitis is extensive, longlasting 1 year, has a reticular arrangement and negative patchtesting. This is a rare presentation of a condition that is uncommon in the pediatric population. The prevalence has increased in some countries, but no descriptive studies of mf in the pediatric population have been done in colombia to date. Additional biopsies demonstrated changes consistent with mycosis fungoides mf. Aug 07, 2016 in the differential diagnosis, allergic contact dermatitis may be hemorrhagic, mimicking capillaritis. The most common sites are the legs, although lesions also may develop in other areas. Urmi khanna and sujay khandpur pigmented contact dermatitis is a noneczematous variant of contact dermatitis clinically characterised by reddishbrown to slate grey pigmentation in a reticulate pattern, usually without any active or preceding clinical dermatitis. Mycosis fungoides, also known as alibertbazin syndrome or granuloma fungoides, is the most common form of cutaneous tcell lymphoma.
They are characterized by extravasation of erythrocytes in the skin with marked hemosiderin deposition. Mycosis fungoides is the most common form of cutaneous tcell lymphoma mfctcl. Pigmented purpuric dermatoses capillaritis uptodate. A case of pigmented purpuric dermatosis similar to mycosis fungoides. In our patient, histologic features were typical of mycosis fungoides presenting as pigmented purpuric dermatitis. Pigmented purpuric dermatosis is a chronic condition characterized by reddishbrown skin lesions caused by leaky capillaries. Prior biopsies were consistent with pigmented purpura. Mimics of cutaneous lymphoma american journal of clinical. Mycosis fungoides pictures, staging, symptoms, treatment. Cutaneous t cell lymphoma and mycosis fungoides cutaneous t cell lymphoma ctcl is a nonspecific term that encompasses all extranodal t cell neoplasms that primarily involve the skin 25. Mycosis fungoides, a cutaneous tcell lymphoma, typically presents as indolent, progressive, and persistent erythematous patches or plaques with mild scaling.
Mycosis fungoides, a cutaneous tcell lymphoma, typically presents as indolent, progressive, and persistent erythematous patches or plaques with mild. Medline abstract for reference 41 of pigmented purpuric. Outline mimics of mf lichen sclerosus hiv related dermatitis pityriasis lichenoides chronica. The differential diagnosis of pigmented purpuric dermatosis includes hyperglobulinaemic purpura, early mycosis fungoides, purpuric clothing dermatitis, stasis pigmentation, scurvy, leukocytoclastic vasculitis, and drug hypersensitivity reactions 15. The aetiology is unknown but they tend to affect the lower limb and there is extravasation of erythrocytes in the skin with marked deposition of haemosiderin. Unusual presentation of mycosis fungoides as pigmented purpura with malignant thymoma. Mycoses fungoides is a rare cutaneous lymphoma that affects. In this situation, a longterm followup and treatments indicated in the early patch stage of mycosis fungoides puva therapy, chlormethine seem adequate. Very critical in the distinction between idiopathic pigmented purpuric dermatosis and purpuric mycosis fungoides is the clinical examination.
Patch phase mycosis fungoides there is a superficial lichenoid infiltrate, mainly lymphocytes and histiocytes and a few atypical cells infiltrating the epidermis without significant spongiosis a phenomenon which is known as exocytosis. An important consideration in the differential diagnosis of ppd is mycosis fungoides mf. Pigmented purpuric dermatosis or mycosis fungoides. Mycosis fungoides and cutaneous tcell lymphomas patient.
Mycosis fungoides is responsible for almost 50% of all cutaneous lymphomas that are primary and is more common in males than in females but rarely occurs in children. A purpuric eruption may be an unusual early manifestation of mycosis fungoides mf, and also ppd may, occasionally, evolve to cutaneous tcell lymphoma. Treatment of pigmented purpuric dermatosis with topical photodynamic therapy. Mimics of mycosis fungoides uma sundram, md, phd professor of pathology. Mycosis fungoides presenting as pigmented purpuric dermatitis. Because mf develops slowly over several years and may have a variety of clinical presentations, including itchy patches, plaques or tumors that may be confused with common benign conditions such as eczema and psoriasis, the disease presents a diagnostic challenge. The key feature is the cayenne pepper coloured spots that can join up to create larger patches. It exhibits a protracted clinical course with slow progression from slightly scaly skin lesions patches to infiltrated plaques. They are characterised by extravasation of erythrocytes in the skin with marked haemosiderin deposition, resulting in many tiny red lesions described as cayenne pepper spots, which group together to form brownred patches. What are the histologic findings of pigmented purpuric. In some cases, the skin lesions cause severe itching. Pigmented purpuric dermatosis an overview sciencedirect. However, cases of mycosis fungoides with clinical features of pigmented purpuric eruptions including lichen aureus are well known20.
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