The pigmented purpuric eruptions are a group of dermatoses characterized by petechiae, pigmentation, and, occasionally, telangiectasia in the absence of associated venous insufficiency or hematologic disorders table 1681. In this situation, a longterm followup and treatments indicated in the early patch stage of mycosis fungoides puva therapy, chlormethine seem. Pigmented purpuric dermatosis an overview sciencedirect. 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.
Pigmented purpuric dermatosis may have tcell gene rearrangements that may or may not be associated with mf. Differential diagnoses include mycosis fungoides, stasis pigmentation, and scurvy. Majocchi purpura purpura annularis telangiectodes in this condition there are dilated capillaries as well as brown patches and cayenne spots. Pathology outlines pigmented purpuric dermatosis ppd. Early stages of ctcl mycosis fungoides may resemble ppd clinically. Sep 28, 2007 pigmented purpuric dermatosis ppd is characterized by cutaneous petechiae and bronze discolorations located predominantly on the leg, with many clinical variations. Oct 05, 2016 pigmented purpuric dermatosis is a chronic condition characterized by reddishbrown skin lesions caused by leaky capillaries. Related venous stasis is to be treated with compressed socks. The key feature is the cayenne pepper coloured spots that can join up to create larger patches. Aug 11, 2016 the pigmented purpuric dermatoses form a group of chronic skin diseases with a characteristic appearance.
Mycoses fungoides is a rare cutaneous lymphoma that affects. There are reports of mycosis fungoides manifesting as pigmented purpura as well as progression of ppd to cutaneous tcell lymphoma. In case of children, this disease has been diagnosed who went on to expand cutaneous t cell lymphoma. The common pathologic pattern of ppd reveals a perivascular lymphocytic infiltrate with redcell extravasation and presence of hemosiderin. Pigmented purpuric dermatosis like variant of mycosis fungoides. Granulomatous changes associated with pigmented purpuric.
The diagnosis of cutaneous tcell lymphoma ctcl requires accurate histopathology, including immunocytochemistry, as well as careful clinical appraisal and analysis for tcell clonality. Although they can arise on any part of the body, they are most commonly located on the lower legs. Pigmented purpuric dermatosis or mycosis fungoides. Levin and oneals the diabetic foot seventh edition, 2008. 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. A drug associated reversible t cell dyscrasia mimicking purpuric mycosis fungoides most commonly triggered by antidepressant and statin drugs. Synonyms include persistent pigmented purpuric dermatitis, purpura simplex, and purpura pigmentosa chronica. Mycosis fungoides presenting as pigmented purpuric dermatitis. Pigmented purpuric dermatoses ppd are capillaritides of varying morphology and unknown etiology. Pigmented purpuric dermatosis refers to one of the three major classes of skin conditions characterized by purpuric skin eruptions pigmented purpuric dermatosis are distinguished from other purpura by size 0. The appearance of capillaritis should give a high index of suspicion for the diagnosis of cutaneous tcell lymphoma, and patients should undergo close followup and biopsies if. Capillaritis as a potential harbinger to cutaneous tcell lymphoma. Five different clinical types of pigmented purpuric. David weedon ao md frcpa fcaphon, in weedons skin pathology third edition, 2010.
Capillaritis as a potential harbinger to cutaneous tcell. Coexisting pityriasis lichenoides was histologically proven in three patients. Mycosis fungoides presenting as pigmented purpuric eruption. Idiopathic pigmented purpuric dermatosis as a well recognized form of prelymphomatous t cell dyscrasia that can presage purpuric mycosis fungoides and other variants of mycosis fungoides. Pigmented purpuric dermatosis may lie on a spectrum with mycosis fungoides mf. Mycosis fungoides mf, a form of cutaneous tcell lymphoma ctcl, is a. Pigmented purpuric dermatosis genetic and rare diseases. Pigmented purpuric dermatosis purpuric lesions, usually on the legs schambergs disease is the most common drugs and systemic diseases can be associated with ppd. What are the diagnostic considerations of pigmented purpuric.
Mycosis fungoides, also known as alibertbazin syndrome or granuloma fungoides, is the most common form of cutaneous tcell lymphoma. 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. However, cases of mycosis fungoides with clinical features of pigmented purpuric eruptions including lichen aureus are well known20. The average age of schamberg disease occurrence is amongst 40 years old patients. Jul 16, 2014 ultimately, 91% were diagnosed with a hypopigmented variant of mf. Dermatosis is a condition that affects the skin, nails, or hair. We present the case of an 82yearold male patient to emphasize the importance of this distinction. Pigmented purpuric dermatosis is a group of chronic skin diseases of mostly unknown cause characterized by a distinct purpuric rash, often confined to the lower limbs 1. Mycosis fungoides presenting with pigmented purpuric rash. There are a number of known and proposed causes for the various forms of ppd. Initial eruptions, resembling pigmented purpuric dermatitis both clinically and histologically, developed into histologically definite mycosis fungoides in followup period averaging 8. A purpuric eruption may be an unusual early manifestation of mycosis fungoides mf, and also ppd may, occasionally, evolve to cutaneous tcell lymphoma. Capillaritis pigmented purpuric dermatoses plastic. Pigmented purpuric dermatosis ppd is the term used to describe a collection.
Gradual progression over a number of years through three clinical stages. Schamberg disease is based on the feedback of a skin biopsy. Pigmented purpuric dermatosis is a condition involving the skin on the lower limbs resulting from red blood cell extravasation from the superficial vascular plexus. 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.
A case of pigmented purpuric dermatosis similar to mycosis. The spectrum of pigmented purpuric dermatosis and mycosis fungoides. Durmaz k 21 a case of pigmented purpuric dermatosis similar to mycosis fungoides glob dermatol, 21 doi. Usually scant perivascular, bandlike or periadnexal infiltrate of small lymphocytes, often with atypia described as cerebriform. There are several different forms of pigmented purpuric dermatosis but all these forms share some common features. Pigmented purpuric dermatoses ppd are a group of conditions characterised by extravasation of red cells andmarked haemosiderin pigmentation of the skin.
They are found most commonly on the lower extremities. Pigmented purpuric dermatosis ppd is a chronic skin disorder of unknown aetiology characterised by symmetrical petechial and pigmented macules, often confined to the lower limbs. What are the histologic findings of pigmented purpuric. Prior biopsies were consistent with pigmented purpura. Pigmented purpuric dermatoses are characterized by extravasation of red blood cells in the skin with marked hemosiderin deposition 2. In this situation, a longterm followup and treatments indicated in the early patch stage of mycosis fungoides puva therapy, chlormethine seem adequate. A case of pigmented purpuric dermatosis similar to mycosis fungoides. Pigmented purpuric dermatosis in mixed cryoglobulinaemia associated with rheumatoid arthritis and hepatitis c infection. The hallmark of a ppd is its characteristic orangebrown, speckled, cayenne pepperlike discoloration.
Mycosis fungoides is the most common type of ctcl, representing 4462% of cases. Thrombocytopenia, hypergammaglobulinemic purpura of waldenstrom, and the pigmented purpuric dermatitislike variant of mycosis fungoides also need to be excluded. Mycosis fungoides presenting with pigmented purpuric rash jaad. Pigmented purpuric dermatosis sometimes basal vacuolar alteration and necrotic keratinocytes can be seen mf. Pigmented purpuric dermatosis of mf should be closely followed and likely treated as mf. What are the diagnostic considerations of pigmented.
Mycosis fungoides presenting as pigmented purpuric. A, longstanding lesion of pigmented purpuric dermatosis involving the lower leg. Schamberg disease may occur at any age but is more common among males. The pathology of cutaneous tcell lymphoma cancer network. 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. Schamberg disease may also be known as progressive pigmentary dermatosis. Pigmented purpuric dermatosis and hepatitis profile.
It generally affects the skin, but may progress internally over time. 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. Another term for a dermatosis is a cutaneous condition. Mycosis fungoides mf can raise as pigmented purpuric dermatoses. A diagnostic dilemma najeeba riyaz, sarita sasidharanpillai, ettappurath n. Mycosis fungoides and cutaneous tcell lymphomas patient. It has also been called capillaritis, although there is no evidence of a true vasculitis. Mycosis fungoides, pigmented purpuric dermatosis, t cell clonality. Mimics of cutaneous lymphoma american journal of clinical. Aug 07, 2016 progression of pigmented purpuralike eruptions to mycosis fungoides. 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. Hyperpigmented patch on the leg mdedge dermatology.
Progression of pigmented purpuralike eruptions to mycosis fungoides. Clinical examination revealed multiple annular purpuric patches on the. Two skin biopsies were performed, with the diagnosis of pigmented purpuric dermatosis. The skin lesions may spread over time, or clear up on their.
We report 3 cases of pigmented purpuric dermatosis ppd with granulomatous features in a 9yearold boy, a 49yearold woman, and a 75yearold woman. The spectrum of pigmented purpuric dermatosis and mycosis. Treatment of pigmented purpuric dermatosis with topical photodynamic therapy. Pigmented purpura like eruption as cutaneous sign of mycosis fungoides with autoimmune purpura. How to get rid of schambergs disease pigmented purpuric. Pigmented purpuric dermatoses information and causes patient. Gougerotblum purpura pigmented purpuric lichenoid dermatosis gougerotblum capillaritis is less common. Dermatoscopic findings of pigmented purpuric dermatosis. 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. All patients had stage ia or ib disease, except one with stage iia disease. 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. Biopsy taken with diagnoses of pigmented purpuric dermatosis, pityriasis rosea and mycosis fungoides. Dermatologic examination revealed generalized purpuric patches with.
Capillaritis pigmented purpuric dermatoses clinical gate. Apr 02, 2020 persistent pigmented purpuric dermatitis and mycosis fungoides. 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. 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. This is a rare presentation of a condition that is uncommon in the. Ultimately, 91% were diagnosed with a hypopigmented variant of mf.
Disease staging is made clinically, and diagnosis may be difficult during the early stages because several common dermatologic conditions share features with mf. Annular discrete cayennepepperlike redbrown patches on the thighs fig. Multiple annular purpuric patches on the abdomen, anterior thighs a, and right leg b. Additional biopsies demonstrated changes consistent with mycosis fungoides mf. Pdf pigmented purpuric dermatosis or mycosis fungoides. 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. Medline abstracts for references 3849 of pigmented. Persistent pigmented purpuric dermatitis and mycosis fungoides. The name mycosis fungoides is very misleadingit loosely means mushroomlike fungal disease.
The patches are thickened and itchy, rather like eczema, but pathology is of a lichenoid reaction. Although the majority of pigmented progressive purpuric dermatosis are benign and incidental, the potential for the evolution of pppds into cutaneous lymphomas exists. The early vascular lesions are subtle and must be differentiated from telangiectases, pigmented purpuric dermatosis, acroangiodermatitis, and lowgrade angiosarcoma. Their cytotoxic effects on keratinocytes may result in. 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. Schamberg disease can be controlled by a cortisone cream. A skin biopsy is the process of diagnosis of schamberg disease. The pigmented purpuric dermatoses ppd are a group of chronic skin disorders with overlapping clinical and histopathological features. This course should be suspected when purpuric pigmented dermatitis is extensive, longlasting 1 year, has a reticular arrangement and negative patch testing. Pigmented purpuric dermatosis is a chronic condition characterized by reddishbrown skin lesions caused by leaky capillaries. Symptoms include rash, tumors, skin lesions, and itchy skin. Granulomatous pigmented purpuric dermatosis gppd is a rare entity with few cases reported in the literature. With reports of ppd evolving into mycosis fungoides over the years it is recommended that atypical ppd, especially the widespread forms should be evaluated for tcell clonality with close monitoring of the monoclonal variants. It exhibits a protracted clinical course with slow progression from slightly scaly skin lesions patches to infiltrated plaques.
Mycosis fungoides presenting as a pigmented purpuric dermatosis. Mycosis fungoides is an epidermotropic ctcl that evolves. In the differential diagnosis, allergic contact dermatitis may be hemorrhagic, mimicking capillaritis. The vessels in ks are usually more irregular than in most developmental or acquired telangiectases, pigmented purpuric. The disease, however, is not a fungal infection but rather a type of nonhodgkins lymphoma. 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.
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