Purpura annularis telangiectoides
- Author(s): Miller, Kristen;
- Fischer, Max;
- Kamino, Hideko;
- Meehan, Shane;
- Cohen, David
- et al.
Published Web Locationhttps://doi.org/10.5070/D344m6d6k1
Purpura annularis telangiectoidesThe Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York
Kristen Miller MD, Max Fischer MD MPH, Hideko Kamino MD, Shane Meehan MD, David Cohen MD MPH
Dermatology Online Journal 18 (12): 5
Purpura annularis telangiectoides of Majocchi (PATM) is a clinical variant of pigmented purpuric dermatoses. It is characterized by asymptomatic or mildly pruritic, erythematous, annular patches that vary in size. The patches exhibit central clearing and peripheral cayenne pepper petechiae. It classically occurs on the lower legs but may be more generalized. Histopathologic features include a lymphocytic capillaritis with subsequent capillary leakage and extravasated erythrocytes, which is typical of all pigmented purpuric dermatoses. The pathogenesis of PATM is unknown, but it may uncommonly be associated with underlying diseases such as hematologic disorders and rarely cutaneous T-cell lymphoma. There is no standard effective treatment.
|Figure 1||Figure 2|
A 69-year-old woman presented to the Charles C. Harris Skin and Cancer Pavilion in October, 2011, for evaluation and possible patch testing for a three-year history of a widespread, asymptomatic eruption. This condition began on her lower legs as several, expanding, light-brown and pink patches with surrounding red dots. The eruption subsequently gradually spread to her trunk, upper legs, arms, and buttocks. The patient denied new medications in the last decade, frequent use of aspirin or nonsteroidal anti-inflammatory agents, recent travel, or hobbies. She did not note any temporal or spatial association between wearing certain articles of clothing and the eruption. Review of systems was negative.
Past medical history included leukopenia plus a decrease in all cell lineages, which was possibly suggestive of a myelodysplastic syndrome, and restless leg syndrome. Medications included trazodone. She denied tobacco, alcohol, or illicit drug use.
A punch biopsy and shave biopsy were obtained from a representative lesion on the right upper leg.
Annular, polycyclic, non-blanching, golden-brown and pink patches with central clearing and a peripheral rim of pinpoint cayenne pepper petechiae, which ranged in size from several to approximately 20 centimeters, were scattered on the arms, legs, abdomen, back, and buttocks. The head, palms, and soles were spared.
A white-cell count was 1.7 x 109/L. A basic metabolic panel, liver function tests, coagulation panel, iron studies, B12, folate, erythrocyte sedimentation rate, C-reactive protein, rheumatoid factor, immunoglobulins, and tumor markers (AFP, CEA, CA19-9) were normal. A bone-marrow biopsy showed a hypocellular marrow, with progressive tri-lineage hematopoiesis and no definitive evidence of acute leukemia or lymphoma.
There is a patchy, band-like, lymphocytic infiltrate with a few extravasated erythrocytes. Lymphocytes are present at the dermoepidermal junction where there are vacuolar changes with occasional necrotic keratinocytes. A Perls’s stain fails to show hemosiderin. A periodic acid-Schiff stain with diastase stain fails to show a thick basement membrane or evidence of fungi.
Purpura annularis telangiectoides of Majocchi (PATM) is a clinical subtype of pigmented purpuric dermatosis (PPD). PPD is an umbrella term for five clinical syndromes that are characterized by petechiae and golden-brown, pigmented macules, papules, and plaques that are typically localized to the lower extremities but may be more generalized. Although the condition is benign with no systemic involvement, the cutaneous manifestations tend to follow a chronic and relapsing course that may persist for years [1, 2]. Recognized subtypes include Schamberg disease, pigmented purpuric lichenoid dermatitis of Gougerot and Blum, lichen aureus, eczematoid purpura of Doucas and Kapetanakis, and purpura annularis telangiectoides of Majocchi. Some authors, however, consider this nomenclature to be superfluous because there is appreciable clinical overlap between these syndromes .
Majocchi first described PATM as an entity in 1896 . The eruption classically affects young women and begins symmetrically on the lower legs with gradual extension to the arms. Telangiectatic macules initially appear and progress to annular red patches of various sizes, which display central clearing with cayenne pepper petechiae at the periphery. Central atrophy occasionally may occur. Although the clinical course is typically of self-limited duration, some cases may persist for years [1, 3].
PPDs are unified by similar histopathologic features. They display a lymphocytic capillaritis that involves the superficial dermal vessels, without features of vasculitis. The degree of perivascular inflammation may vary widely and a lichenoid infiltrate also may be observed, which is common in lichen aureus and the Gougerot and Blum variants. Perivascular inflammation results in vessel leakage and extravasation of erythrocytes into the surrounding dermis; older lesions additionally demonstrate prominent hemosiderin-laden macrophages [1, 5].
The pathogenesis of PPDs is poorly understood. Although all variants are associated with some degree of perivascular inflammation that results in capillary leakage, the underlying cause for this inflammation is unknown. Venous hypertension, gravitational dependency, and medications may be inciting factors . Local cell-mediated immunity and Langerhans cell- mediated endothelial damage also may play an important role [1, 7].
The differential diagnosis of PPD includes several purpuric eruptions, such as leukocytoclastic vasculitis, thrombocytopenia, drug hypersensitivity reactions, purpuric allergic contact dermatitis to disperse blue 106 and 124 dyes, and purpuric cutaneous T-cell lymphoma (CTCL). Although there is no known relationship between myelodysplastic syndrome and PATM, PPDs have been reported in association with diabetes mellitus, rheumatoid arthritis, lupus erythematosus, hematologic disorders, and CTCL [2, 3, 8, 9].
The association between CTCL and PPD is uncertain. Several cases in the literature have documented either the coexistence of PPD and CTCL or the progression of PPD to CTCL, although this is extremely rare [9, 10, 11]. Furthermore, lesions of PPD with a lichenoid infiltrate may demonstrate populations of clonal T-cells. Based on these observations, some authors speculate that CTCL and lichenoid forms of PPD may be biologically related and certain variants of PPD may precede development of CTCL. This is controversial, however, as additional studies have found no association between lichenoid variants of PPD and progression to CTCL [12, 13, 14].
PATM does not require treatment because it is a self-limited condition, but associated symptoms, such as pruritus or appearance, may be distressing to patients. No medical intervention has been proven to be of benefit. Pruritus may be alleviated with topical glucocorticoids and venous compression stockings and leg elevation may counteract effects of venous stasis. Case reports have documented some success with pentoxifylline, PUVA photochemotherapy, ascorbic acid plus rutoside, and methotrexate. A recent case series demonstrated improvement with narrow-band ultraviolet B phototherapy [2, 14].
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