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Pigmented Purpuric Dermatosis
Introduction
Pigmented purpuric dermatosis (PPD) is the term used to describe a collection of numerous subtypes of generally benign, chronic, purpuric skin eruptions. Clinically, they are characterized by red to purple macules and patches as well as petechiae with erythrocyte extravasation and hemosiderin deposition in the skin, which can lead to a red-brown to golden-brown color as the hemosiderin is resorbed. These lesions most commonly occur on the lower extremities but can sometimes occur on the arms as well. Pigmented purpuric dermatitis is often asymptomatic but can be associated with mild pruritus. Treatment can be challenging, but patient reassurance on the benign nature of these diseases is crucial.
Etiology
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Etiology
Most cases of pigmented purpuric dermatosis are idiopathic. It is important to recognize that these disorders are not associated with coagulopathies or thrombocytopenia. However, since most cases present on the lower extremities, important underlying factors include venous stasis, exercise, and capillary fragility, which lead to erythrocyte extravasation into the dermis and the characteristic purpuric color.[1][2] Due to the inflammatory infiltrate of lymphocytes, macrophages, and Langerhans cells, which is often seen on skin biopsies as a capillaritis, cell-mediated immunity is also implicated in the pathogenesis of these disorders and may contribute to vascular fragility. Immunohistochemical studies of Schamberg disease, a subtype of pigmented purpuric dermatitis, have revealed a perivascular infiltrate of dendritic cells and lymphocytes, which interact with vascular endothelial cells and affect the permeability of the microvasculature.[3][4] Humoral immunity also may be involved in the pathogenesis as immunoglobulin and complement deposition around dermal vessels has been observed in some cases.[5][6][7][8][9]Many medications also have been reported as causing pigmented purpuric dermatitis, including acetaminophen, aspirin, carbamazepine, chlordiazepoxide, diltiazem, dipyridamole, furosemide, glipizide, hydralazine, infliximab, interferon-alpha, medroxyprogesterone acetate, meprobamate, pseudoephedrine, raloxifene, and thiamine.[10][11][12]
Epidemiology
Pigmented purpuric dermatoses are rare. There is not a racial predilection; although, they appear slightly more often in males. Children can also be affected.[1][2]
Pathophysiology
In pigmented purpuric dermatoses, capillaritis in the dermis with possible concomitant venous hypertension leads to endothelial cell dysfunction and extravasation of red blood cells. These erythrocytes deposit in the dermis, clinically manifesting as purpuric macules and patches with variable configurations. Over time, as the hemosiderin is resorbed, golden-brown pigmentation collects in the skin, which over time resolves. However, the disease can often flare and persist chronically.
Histopathology
Skin biopsies show perivascular lymphohistiocytic inflammation around small cutaneous blood vessels with endothelial cell swelling. Leukocytoclastic vasculitis is not observed.[5][13] Extravasated erythrocytes are often seen in the dermis with variable hemosiderin deposition in macrophages. The hemosiderin tends to occur in the superficial papillary dermis, in contrast to stasis dermatitis, in which the deposition occurs around deeper blood vessels. Mild dermatitis can be seen in the overlying epidermis, manifesting as mild spongiosis with lymphocyte exocytosis. Dermatitis is most pronounced in the pigmented purpuric dermatitis variants pigmented purpuric lichenoid dermatitis of Gougerot and Blum and the eczematoid-like purpura of Doucas and Kapetanakis. In contrast, in the lichen aureus variant of pigmented purpuric dermatosis, there is a band of lichenoid lymphocytic inflammation at the dermal-epidermal junction, with a Grenz zone of the uninvolved papillary dermis.[14] A rare variant of pigmented purpuric dermatitis has also been reported in which granulomatous inflammation was characteristic.[15]
History and Physical
There are many different subtypes of pigmented purpuric dermatosis. Their clinical presentation can mainly distinguish these, but some have unique histopathology as well.
Progressive Pigmentary Dermatosis (Schamberg Disease)
This condition presents as an eruption on the legs that consists of red-brown macules and patches with pinpoint red puncta. These stippled puncta have been likened to grains of cayenne pepper as the skin lesions can often have orange-brown color due to hemosiderin deposition. Although initially described in adolescents, it can affect all ages and, on average, presents in the fifth decade of life.[16] The condition is most often asymptomatic, although mild pruritus can occur. The eruption is most common on the lower legs but can occur on the trunk or arms. A chronic relapsing and remitting course can happen over time.
Purpura Aannularis Telangiectodes of Majocchi
This variant presents with annular patches of punctate red-brown macules and patches on the legs with punctate petechiae at the border. The annular (ring-like) configuration of the patches with central clearing is unique, and annular plaques also can manifest. This eruption typically begins on the lower legs but can spread proximally and may also occur on the arms and trunk. This subtype is most common in females.[1]
Lichen Aureus
The hallmark of lichen aureus is the presentation of ovoid golden orange to mildly purpuric macules, patches, and plaques. They may be quite pruritic, although they are often asymptomatic. The lesions are often unilateral and localized to the legs but can involve the trunk and arms. It is most common in young adults and can have a chronic course that may persist for years. This eruption is also distinguished by a dense band of lichenoid dermal inflammation, in contrast to the other subtypes of pigmented purpuric dermatitis, which do not exhibit lichenoid histopathology.[17]
Pigmented Purpuric Lichenoid Dermatitis of Gougerot and Blum
This eruption is distinguished by red-brown to purpuric ovoid papules and plaques that occur on the lower extremities. Although it is named lichenoid dermatitis, it does not typically exhibit lichenoid histology. Clinically, this condition can mimic Kaposi sarcoma, and therefore, skin biopsies are helpful to make an accurate diagnosis.[18] This subtype is more common in males and can be pruritic.
Eczematoid-Like Purpura of Doucas and Kapetanakis
This condition is characterized by mild scale overlying purpuric and petechial macules, papules, and patches. It is often pruritic, and the lesions can be extensive and may involve the trunk and arms in addition to the lower extremities. It typically self-resolves over many months.[19]
Disseminated Pruriginous Angiodermatitis (Itching Purpura)
Itching purpura presents acutely on the legs with diffuse purpuric, orange to brown macules and patches, accompanied by severe pruritus. It can evolve to become widespread and often has a chronic course. It is most common in middle-aged men.[20][21]
Unilateral Linear Capillaritis (Linear Pigmented Purpura)
This eruption presents as a linear distribution of purpuric to red-brown macules, unilaterally on a lower extremity. It commonly self-resolves. Unilateral linear capillaritis has been reported as a segmental variant of Schamberg disease or lichen aureus.[22][23]
Granulomatous Pigmented Purpura
This subtype of pigmented purpuric dermatitis has been most often reported in patients of Asian descent. It occurs as red-brown macules, papules, and plaques on the feet and ankles but can occur on the hands and wrists. It is distinguished by dense granulomatous inflammation in the dermis with thickened capillaries and hemosiderin deposition.[16][24][25]
Evaluation
Pigmented purpuric dermatoses are largely diagnosed based on the clinical presentation. However, with atypical presentations or to rule out cutaneous vasculitis, dermatitis, or cutaneous t-cell lymphoma, skin biopsy by punch technique can be helpful. CBC and coagulation studies can be performed to rule out thrombocytopenia or clotting disorders. Medication history should also be obtained to screen for drug hypersensitivity as a cause. To rule out concomitant allergic contact dermatitis, patch testing can be performed.
Treatment / Management
It can be challenging to treat pigmented purpuric dermatoses. If a medication reaction is causative, the eruption should clear with discontinuation of the drug. Due to the possible etiology of vascular hypertension and venous stasis of the lower extremities, compression stockings are recommended. A study of three patients with pigmented purpuric dermatitis revealed clearance of this eruption after 4 weeks of treatment with the bioflavonoid, rutoside 50 mg 2 times per day, and ascorbic acid 500 mg 2 times per day.[26] Given the safety of these supplements, this is a reasonable first-line treatment. Medium to high potency topical corticosteroids such as triamcinolone can also be used and may improve pruritus and the inflammatory component of this condition. Although systemic immunosuppression with corticosteroids or cyclosporine can be helpful, the side effect profile of these medications and the benign nature of pigmented purpuric dermatitis may not warrant their use.[27] However, the condition often recurs when immunosuppression is discontinued. Topical tacrolimus or pimecrolimus can be useful for chronic use as they limit the risk of cutaneous atrophy from topical corticosteroids.[28] Griseofulvin 500 mg to 750 mg daily improved 6 patients with pigmented purpuric dermatitis.[29] (B3)
Other case reports of patients with pigmented purpuric dermatitis showed improvement with colchicine 0.5 mg 2 times per day, minocycline, methotrexate, and pentoxifylline 400 mg 3 times per day.[30][31][32] Ultraviolet (UV) light therapy with PUVA or narrowband UVB also has been reported to be beneficial.[33][34][35][36] In some cases, the disease flared with discontinuation of treatment, but sustained remission was achieved in others.[37][38] Topical photodynamic therapy using 5-aminolevulinic acid or methyl aminolevulinic acid as photosensitizing agents also has been reported to improve pigmented purpuric dermatitis.[39](B3)
Differential Diagnosis
The differential diagnosis of pigmented purpuric dermatitis includes nummular dermatitis, allergic contact dermatitis, stasis dermatitis, cutaneous vasculitis, Kaposi sarcoma, and cutaneous t-cell lymphoma (CTCL). Cutaneous vasculitis can often be distinguished clinically as palpable purpura with variable cutaneous necrosis, whereas most pigmented purpuric dermatitis lesions are not palpable and tend to present most commonly as purpuric macules and patches, although papules and plaques may occur. The clinical history often is helpful as most lesions of pigmented purpuric dermatitis are not pruritic, in contrast to dermatitis, which is typically pruritic. The presence of lower extremity edema favors venous stasis changes.
Early stages of CTCL (mycosis fungoides) may resemble pigmented purpuric dermatitis clinically. There also have been reports of patients initially presenting with pigmented purpuric dermatitis who later declare as CTCL.[40] Numerous cases of CTCL have been reported in which patients initially present with the appearance of pigmented purpuric dermatitis.[41][42][43][44][45][46] Therefore, it is critical to follow patients with pigmented purpuric dermatitis and perform skin biopsies periodically with a lack of improvement or disease progression.
Prognosis
Pigmented purpuric dermatitis is often chronic with a relapsing and remitting course. However, it is a benign, often asymptomatic condition. Even if the capillaritis improves and the active inflammation ceases, the resulting hemosiderin deposition in the dermis can take months to years to slowly fade.
Complications
Complications of pigmented purpuric dermatitis usually revolve around the symptoms themselves, and patients are displeased with their appearance.
Deterrence and Patient Education
Patients should be reassured that pigmented purpuric dermatoses are benign. Treatment should focus on the relief of symptoms of itching. No treatment is required, however, and observation of the condition is a reasonable approach.
Enhancing Healthcare Team Outcomes
The diagnosis and management of pigmented purpuric dermatitis are difficult and is best done with an interprofessional team that includes the primary care provider, dermatologist, nurse practitioner, and pathologist. Pigmented purpuric dermatitis is often chronic with a relapsing and remitting course. However, it is a benign, often asymptomatic condition. Even if the capillaritis improves and the active inflammation ceases, the resulting hemosiderin deposition in the dermis can take months to years to slowly fade. Patients should be encouraged to wear compression stockings and keep the legs elevated at rest. The use of moisturizers may help relieve the itching. For most patients, the problem is poor cosmesis.
References
Newton RC, Raimer SS. Pigmented purpuric eruptions. Dermatologic clinics. 1985 Jan:3(1):165-9 [PubMed PMID: 4092379]
Tristani-Firouzi P, Meadows KP, Vanderhooft S. Pigmented purpuric eruptions of childhood: a series of cases and review of literature. Pediatric dermatology. 2001 Jul-Aug:18(4):299-304 [PubMed PMID: 11576402]
Level 3 (low-level) evidencevon den Driesch P, Simon M Jr. Cellular adhesion antigen modulation in purpura pigmentosa chronica. Journal of the American Academy of Dermatology. 1994 Feb:30(2 Pt 1):193-200 [PubMed PMID: 7507134]
Aiba S, Tagami H. Immunohistologic studies in Schamberg's disease. Evidence for cellular immune reaction in lesional skin. Archives of dermatology. 1988 Jul:124(7):1058-62 [PubMed PMID: 3291778]
Ratnam KV, Su WP, Peters MS. Purpura simplex (inflammatory purpura without vasculitis): a clinicopathologic study of 174 cases. Journal of the American Academy of Dermatology. 1991 Oct:25(4):642-7 [PubMed PMID: 1791222]
Level 2 (mid-level) evidenceNishioka K, Katayama I, Masuzawa M, Yokozeki H, Nishiyama S. Drug-induced chronic pigmented purpura. The Journal of dermatology. 1989 Jun:16(3):220-2 [PubMed PMID: 2507607]
Level 3 (low-level) evidenceKwon SJ,Lee CW, Figurate purpuric eruptions on the trunk: acetaminophen-induced rashes. The Journal of dermatology. 1998 Nov [PubMed PMID: 9863291]
Level 3 (low-level) evidenceGupta G, Holmes SC, Spence E, Mills PR. Capillaritis associated with interferon-alfa treatment of chronic hepatitis C infection. Journal of the American Academy of Dermatology. 2000 Nov:43(5 Pt 2):937-8 [PubMed PMID: 11044828]
Level 3 (low-level) evidenceAdams BB, Gadenne AS. Glipizide-induced pigmented purpuric dermatosis. Journal of the American Academy of Dermatology. 1999 Nov:41(5 Pt 2):827-9 [PubMed PMID: 10534660]
Level 3 (low-level) evidenceSardana K, Sarkar R, Sehgal VN. Pigmented purpuric dermatoses: an overview. International journal of dermatology. 2004 Jul:43(7):482-8 [PubMed PMID: 15230884]
Level 3 (low-level) evidenceDíaz-Jara M,Tornero P,Barrio MD,Vicente ME,Fuentes V,Barranco R, Pigmented purpuric dermatosis due to pseudoephedrine. Contact dermatitis. 2002 May [PubMed PMID: 12084086]
Level 3 (low-level) evidenceTsao H, Lerner LH. Pigmented purpuric eruption associated with injection medroxyprogesterone acetate. Journal of the American Academy of Dermatology. 2000 Aug:43(2 Pt 1):308-10 [PubMed PMID: 10906656]
Level 3 (low-level) evidenceSmoller BR, Kamel OW. Pigmented purpuric eruptions: immunopathologic studies supportive of a common immunophenotype. Journal of cutaneous pathology. 1991 Dec:18(6):423-7 [PubMed PMID: 1723080]
Rudolph RI, Lichen aureus. Journal of the American Academy of Dermatology. 1983 May [PubMed PMID: 6863634]
Level 3 (low-level) evidenceSaito R, Matsuoka Y. Granulomatous pigmented purpuric dermatosis. The Journal of dermatology. 1996 Aug:23(8):551-5 [PubMed PMID: 8854588]
Level 3 (low-level) evidenceTorrelo A,Requena C,Mediero IG,Zambrano A, Schamberg's purpura in children: a review of 13 cases. Journal of the American Academy of Dermatology. 2003 Jan [PubMed PMID: 12522367]
Level 2 (mid-level) evidencePrice ML, Jones EW, Calnan CD, MacDonald DM. Lichen aureus: a localized persistent form of pigmented purpuric dermatitis. The British journal of dermatology. 1985 Mar:112(3):307-14 [PubMed PMID: 3978038]
Wong RC, Solomon AR, Field SI, Anderson TF. Pigmented purpuric lichenoid dermatitis of Gougerot-Blum mimicking Kaposi's sarcoma. Cutis. 1983 Apr:31(4):406-8, 410 [PubMed PMID: 6851635]
Level 3 (low-level) evidenceDOUCAS C, KAPETANAKIS J. Eczematid-like purpura. Dermatologica. 1953:106(2):86-95 [PubMed PMID: 13060054]
Mosto SJ, Casala AM. Disseminated pruriginous angiodermatitis (itching purpura). Archives of dermatology. 1965 Apr:91(4):351-6 [PubMed PMID: 9626084]
Pravda DJ, Moynihan GD. Itching purpura. Cutis. 1980 Feb:25(2):147-51 [PubMed PMID: 7357881]
Level 3 (low-level) evidenceRiordan CA, Darley C, Markey AC, Murphy G, Wilkinson JD. Unilateral linear capillaritis. Clinical and experimental dermatology. 1992 May:17(3):182-5 [PubMed PMID: 1451296]
Level 3 (low-level) evidenceHiggins EM, Cox NH. A case of quadrantic capillaropathy. Dermatologica. 1990:180(2):93-5 [PubMed PMID: 2311802]
Level 3 (low-level) evidenceWong WR, Kuo TT, Chen MJ, Chan HL. Granulomatous variant of chronic pigmented purpuric dermatosis: report of two cases. The British journal of dermatology. 2001 Jul:145(1):162-4 [PubMed PMID: 11453929]
Level 3 (low-level) evidenceLin WL, Kuo TT, Shih PY, Lin WC, Wong WR, Hong HS. Granulomatous variant of chronic pigmented purpuric dermatoses: report of four new cases and an association with hyperlipidaemia. Clinical and experimental dermatology. 2007 Sep:32(5):513-5 [PubMed PMID: 17535280]
Level 3 (low-level) evidenceReinhold U, Seiter S, Ugurel S, Tilgen W. Treatment of progressive pigmented purpura with oral bioflavonoids and ascorbic acid: an open pilot study in 3 patients. Journal of the American Academy of Dermatology. 1999 Aug:41(2 Pt 1):207-8 [PubMed PMID: 10426890]
Level 3 (low-level) evidenceOkada K, Ishikawa O, Miyachi Y. Purpura pigmentosa chronica successfully treated with oral cyclosporin A. The British journal of dermatology. 1996 Jan:134(1):180-1 [PubMed PMID: 8745912]
Level 3 (low-level) evidenceBöhm M, Bonsmann G, Luger TA. Resolution of lichen aureus in a 10-year-old child after topical pimecrolimus. The British journal of dermatology. 2004 Aug:151(2):519-20 [PubMed PMID: 15327576]
Level 3 (low-level) evidenceTamaki K, Yasaka N, Osada A, Shibagaki N, Furue M. Successful treatment of pigmented purpuric dermatosis with griseofulvin. The British journal of dermatology. 1995 Jan:132(1):159-60 [PubMed PMID: 7756136]
Level 3 (low-level) evidenceGeller M. Benefit of colchicine in the treatment of Schamberg's disease. Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology. 2000 Sep:85(3):246 [PubMed PMID: 11030283]
Level 3 (low-level) evidenceHoesly FJ, Huerter CJ, Shehan JM. Purpura annularis telangiectodes of Majocchi: case report and review of the literature. International journal of dermatology. 2009 Oct:48(10):1129-33. doi: 10.1111/j.1365-4632.2009.04160.x. Epub [PubMed PMID: 19775410]
Level 3 (low-level) evidencePanda S, Malakar S, Lahiri K. Oral pentoxifylline vs topical betamethasone in Schamberg disease: a comparative randomized investigator-blinded parallel-group trial. Archives of dermatology. 2004 Apr:140(4):491-3 [PubMed PMID: 15096387]
Level 3 (low-level) evidenceLing TC, Goulden V, Goodfield MJ. PUVA therapy in lichen aureus. Journal of the American Academy of Dermatology. 2001 Jul:45(1):145-6 [PubMed PMID: 11423854]
Level 3 (low-level) evidenceWong WK,Ratnam KV, A report of two cases of pigmented purpuric dermatoses treated with PUVA therapy. Acta dermato-venereologica. 1991 [PubMed PMID: 1676222]
Level 3 (low-level) evidenceKrizsa J, Hunyadi J, Dobozy A. PUVA treatment of pigmented purpuric lichenoid dermatitis (Gougerot-Blum). Journal of the American Academy of Dermatology. 1992 Nov:27(5 Pt 1):778-80 [PubMed PMID: 1430408]
Gudi VS, White MI. Progressive pigmented purpura (Schamberg's disease) responding to TL01 ultraviolet B therapy. Clinical and experimental dermatology. 2004 Nov:29(6):683-4 [PubMed PMID: 15550157]
Level 3 (low-level) evidenceLasocki AL,Kelly RI, Narrowband UVB therapy as an effective treatment for Schamberg's disease. The Australasian journal of dermatology. 2008 Feb [PubMed PMID: 18186840]
Level 3 (low-level) evidenceKocaturk E, Kavala M, Zindanci I, Zemheri E, Sarigul S, Sudogan S. Narrowband UVB treatment of pigmented purpuric lichenoid dermatitis (Gougerot-Blum). Photodermatology, photoimmunology & photomedicine. 2009 Feb:25(1):55-6. doi: 10.1111/j.1600-0781.2009.00398.x. Epub [PubMed PMID: 19152519]
Level 3 (low-level) evidenceKim SK, Kim EH, Kim YC. Treatment of pigmented purpuric dermatosis with topical photodynamic therapy. Dermatology (Basel, Switzerland). 2009:219(2):184-6. doi: 10.1159/000228327. Epub 2009 Jul 8 [PubMed PMID: 19590170]
Level 3 (low-level) evidenceBarnhill RL, Braverman IM. Progression of pigmented purpura-like eruptions to mycosis fungoides: report of three cases. Journal of the American Academy of Dermatology. 1988 Jul:19(1 Pt 1):25-31 [PubMed PMID: 3403742]
Level 3 (low-level) evidenceMartínez W,del Pozo J,Vázquez J,Yebra-Pimentel MT,Almagro M,García-Silva J,Fonseca E, Cutaneous T-cell lymphoma presenting as disseminated, pigmented, purpura-like eruption. International journal of dermatology. 2001 Feb [PubMed PMID: 11328399]
Level 3 (low-level) evidenceUgajin T, Satoh T, Yokozeki H, Nishioka K. Mycosis fungoides presenting as pigmented purpuric eruption. European journal of dermatology : EJD. 2005 Nov-Dec:15(6):489-91 [PubMed PMID: 16280306]
Level 3 (low-level) evidenceGeorgala S, Katoulis AC, Symeonidou S, Georgala C, Vayopoulos G. Persistent pigmented purpuric eruption associated with mycosis fungoides: a case report and review of the literature. Journal of the European Academy of Dermatology and Venereology : JEADV. 2001 Jan:15(1):62-4 [PubMed PMID: 11451328]
Level 3 (low-level) evidencePuddu P, Ferranti G, Frezzolini A, Colonna L, Cianchini G. Pigmented purpura-like eruption as cutaneous sign of mycosis fungoides with autoimmune purpura. Journal of the American Academy of Dermatology. 1999 Feb:40(2 Pt 2):298-9 [PubMed PMID: 10025852]
Level 3 (low-level) evidenceCather JC,Farmer A,Jackow C,Manning JT,Shin DM,Duvic M, Unusual presentation of mycosis fungoides as pigmented purpura with malignant thymoma. Journal of the American Academy of Dermatology. 1998 Nov [PubMed PMID: 9810916]
Level 3 (low-level) evidenceHanna S, Walsh N, D'Intino Y, Langley RG. Mycosis fungoides presenting as pigmented purpuric dermatitis. Pediatric dermatology. 2006 Jul-Aug:23(4):350-4 [PubMed PMID: 16918631]
Level 3 (low-level) evidence