This course should be suspected when purpuric pigmented dermatitis is extensive, longlasting 1 year, has a reticular arrangement and negative patchtesting. Mycosis fungoides presenting as pigmented purpuric dermatitis. 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. Mycosis fungoides pictures, staging, symptoms, treatment. 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. Mar 31, 2020 pigmented purpuric dermatitis affecting the trunk. Thrombocytopenia, hypergammaglobulinemic purpura of waldenstrom, and the pigmented purpuric dermatitislike variant of mycosis fungoides also need to be excluded. The pathology of cutaneous tcell lymphoma cancer network. The spectrum of pigmented purpuric dermatosis and mycosis. 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. Mycosis fungoides, a cutaneous tcell lymphoma, typically presents as indolent, progressive, and persistent erythematous patches or plaques with mild scaling. Hyperpigmented patch on the leg mdedge dermatology. A rare presentation of a great masquerader adel alabdulrazzaq, md, valid bagher zadeh, md, sultan alotaibi, md, nabeel najem, md. They are characterized by extravasation of erythrocytes in the skin with marked hemosiderin deposition.
Aug 12, 2019 the pigmented purpuric dermatoses are a group of chronic diseases of mostly unknown etiology that have a very distinctive clinical appearance. Prior biopsies were consistent with pigmented purpura. There are a number of known and proposed causes for the various forms of ppd. Mimics of cutaneous lymphoma american journal of clinical. 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. Mycosis fungoides presenting as a pigmented purpuric dermatosis. Mycosis fungoides is the most common example of cutaneous t cell lymphoma. 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. Aug 11, 2016 the pigmented purpuric dermatoses form a group of chronic skin diseases with a characteristic appearance. Mycosis fungoides mf can raise as pigmented purpuric dermatoses. In this situation, a longterm followup and treatments indicated in the early patch stage of mycosis fungoides puva therapy, chlormethine seem adequate. Mimics of mycosis fungoides uma sundram, md, phd professor of pathology oakland university william beaumont school of medicine. Mycosis fungoides causes, symptoms, diagnosis, stages. There are several different forms of pigmented purpuric dermatosis but all these forms share some common features.
There are reports of mycosis fungoides manifesting as pigmented purpura as well as progression. Mycoses fungoides is a rare cutaneous lymphoma that affects. 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. Very critical in the distinction between idiopathic pigmented purpuric dermatosis and purpuric mycosis fungoides is the clinical examination. 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. Pigmented purpuric dermatosis is a chronic condition characterized by reddishbrown skin lesions caused by leaky capillaries.
The prevalence has increased in some countries, but no descriptive studies of mf in the pediatric population have been done in colombia to date. Quality nursing care is necessary for effective diagnosis and treatment of patients with mfctcl. Pigmented purpuric dermatosis classification by phenotypic and. However, cases of mycosis fungoides with clinical features of pigmented purpuric eruptions including lichen aureus are well known20. 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. Figure 1 from dermoscopy of early stage mycosis fungoides. In early stages, its often treated with medicines or therapies that target just your skin. Pdf pigmented purpuric dermatosis or mycosis fungoides.
Pigmented purpuric dermatosis causes, symptoms, diagnosis. 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 most common sites are the legs, although lesions also may develop in other areas. Pigmented purpuric dermatosis of mf should be closely followed and likely treated as mf. 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. Mimics of mycosis fungoides uma sundram, md, phd professor of pathology. 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. Dermatologic examination revealed generalized purpuric patches with unclear borders primarily.
Given the clinical description this atypical epitheliotropic lymphocytic infiltrate is best categorized as purpuric mycosis fungoides arising in a background of pigmented purpuric dermatosis. Mycosis fungoides presenting as pigmented purpuric. Unusual presentation of mycosis fungoides as pigmented purpura with malignant thymoma. Treatment of pigmented purpuric dermatosis with topical photodynamic therapy.
Mycosis fungoides is an epidermotropic ctcl that evolves. Additional biopsies demonstrated changes consistent with mycosis fungoides mf. In some cases, the skin lesions cause severe itching. Pigmented purpuric dermatosis or mycosis fungoides. In our patient, histologic features were typical of mycosis fungoides presenting as pigmented purpuric dermatitis. Mycosis fungoides and cutaneous tcell lymphomas patient. Capillaritis as a potential harbinger to cutaneous tcell. Earlystage mfctcl stages ia and ib is most often managed in both. This is a rare presentation of a condition that is uncommon in the. Aug 07, 2016 in the differential diagnosis, allergic contact dermatitis may be hemorrhagic, mimicking capillaritis. Pigmented purpuric dermatoses ppd characterized a group of capillaritis of obscure aetiology, manifested by localized or generalized, pigmented, petechial eruption. 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. Pigmented purpuric dermatosis is a condition involving the skin on the lower limbs resulting from red blood cell extravasation from the superficial vascular plexus.
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. Pigmented purpuric dermatoses capillaritis uptodate. 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. 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. Capillaritis pigmented purpuric dermatoses plastic. Although they can arise on any part of the body, they are most commonly located on the lower legs.
This condition may usually affect adults who are over 50 years of age but cases of children being affected have been reported 1, 2. An important consideration in the differential diagnosis of ppd is mycosis fungoides mf. In general, mycosis fungoides is described with patches or plaques coating the surface of the skin. Pigmented purpuric dermatosis may lie on a spectrum with mycosis fungoides mf. Mycosis fungoides mf can present with purpuric lesions, and rare patients who seemed to have persistent pigmented purpuric dermatitis pppd have developed mf. 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. Pigmented purpuralike eruption as cutaneous of mycosis fungoides with autoimmune purpura. 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. 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. Capillaritis pigmented purpuric dermatoses clinical gate. Mycosis fungoides is the most common type of ctcl, representing 4462% of cases. In the differential diagnosis, allergic contact dermatitis may be hemorrhagic, mimicking capillaritis. Some of the lesions show the characteristic orangebrown, speckled, cayenne pepperlike discoloration that is the hallmark clinical sign of a. There are reports of mycosis fungoides manifesting as pigmented purpura as well as progression of ppd to cutaneous tcell lymphoma.
It generally affects the skin, but may progress internally over time. Medline abstract for reference 41 of pigmented purpuric. Some of the lesions show the characteristic orangebrown, speckled, cayenne pepperlike discoloration that is. There are reports of mycosis fungoides manifesting as pigmented purpura as well.
The pigmented purpuric dermatoses are a group of chronic diseases of mostly unknown etiology that have a very distinctive clinical appearance. Other presentations include eczematous, psoriasiform, poikilodermatous, and hypopigmented patches. It is characterized by multiple tan to reddish small macules socalled cayenne pepper spots that coalesce into tan to orange patches. Outline mimics of mf lichen sclerosus hiv related dermatitis pityriasis lichenoides chronica.
Pigmented purpuric dermatosis genetic and rare diseases. A purpuric eruption may be an unusual early manifestation of mycosis fungoides mf, and also ppd may, occasionally, evolve to cutaneous tcell lymphoma. Persistent pigmented purpuric dermatitis and mycosis fungoides. Mycosis fungoides is the most common form of cutaneous tcell lymphoma mfctcl. 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.
In this situation, a longterm followup and treatments indicated in the early patch stage of mycosis fungoides puva therapy, chlormethine seem. Pigmented purpura like eruption as cutaneous sign of mycosis fungoides with autoimmune purpura. This course should be suspected when purpuric pigmented dermatitis is extensive, longlasting 1 year, has a reticular arrangement and negative patch testing. As such, ctcl includes both indolent and aggressive malignancies of varying clinical, histologic and immunophenotypic character.
A case of pigmented purpuric dermatosis similar to mycosis fungoides. Symptoms include rash, tumors, skin lesions, and itchy skin. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma in adults and children. Mycosis fungoides pictures, staging, prognosis, symptoms. What are the histologic findings of pigmented purpuric. Mycosis fungoides nord national organization for rare.
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. 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. 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. Mycosis fungoides is a rare form of tcell lymphoma of the skin cutaneous.
Their cytotoxic effects on keratinocytes may result in. The key feature is the cayenne pepper coloured spots that can join up to create larger patches. Mycosis fungoides, also known as alibertbazin syndrome or granuloma fungoides, is the most common form of cutaneous tcell lymphoma. Pigmented purpuric dermatosis may have tcell gene rearrangements that may or may not be associated with mf. Purpuric mycosis fungoides the gulf journal of dermatology and. This is a rare presentation of a condition that is uncommon in the pediatric population.
A case of pigmented purpuric dermatosis similar to mycosis. Pigmented purpuric dermatoses information and causes patient. 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 is rarely cured, but some people stay in remission for a long time.
Mycosis fungoides presenting with pigmented purpuric rash. Two skin biopsies were performed, with the diagnosis of pigmented purpuric dermatosis. Multiple annular purpuric patches on the abdomen, anterior thighs a, and right leg b. Mycosis fungoides, pigmented purpuric dermatosis, t cell clonality. The diagnosis of cutaneous tcell lymphoma ctcl requires accurate histopathology, including immunocytochemistry, as well as careful clinical appraisal and analysis for tcell clonality. In individuals with mycosis fungoides, the skin becomes infiltrated with plaques and nodules that are composed of lymphocytes.
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