The Pigmented Purpuric Dermatoses - Dermatologyrounds.ca

Table 1: Differential diagnosis of purpura• NonpalpablePetechia – Thrombocytopenia (ITP,TTP, DIC,drug-induced, marrow infiltration)– Thrombocytopathies (hereditary,drug-induced, thrombocytosis, renalor hepatic insufficiency, monoclonalgammopathy)– Non-platelet–related (PPD, Valsalvamaneuver, benign hypergammaglobulinemia)Ecchymotic – Procoagulant defect– Poor dermal support (actinic damage,corticosteroids, scurvy, amyloidosis,Ehlers-Danlos disease,pseudoxanthoma elasticum)– Benign hypergammaglobulinemicpurpura– Trauma• Palpable with early prominent erythema– Small-vessel leukocytoclastic vasculitis(hypersensitivity, Henoch- Schönlein purpura)– Small- and medium-sized–vessel leukocytoclasticor granulomatous vasculitis (SLE, RA, PAN, mixedcryoglobulinemia, Wegener’s granulomatosis)– Small-vessel injury (EM, PLEVA, PPD, benignhypergammaglobulinemic purpura)• Noninflammatory and retiformPlatelet- – Heparin necrosisrelated – Thrombotic thrombocytopenicpurpura– Myeloproliferative disease withthrombocytosisCold- – Cryoglobulinemiarelated – Cryofibrinogenemia– Cold agglutininsOrganism – Fungi (Mucormycosis)growing – Ecthyma gangrenosumin vessels (Pseudomonas sp)Coagulation – Disseminated intravascularanomalies coagulation (DIC)– Protein C or S deficiency (homozygous)– Acquired protein C deficiency– Coumarin necrosis– Antiphospholipid antibody(lupus anticoagulant)– Paroxysmal nocturnal hemoglobinuria– Calciphylaxis– Livedoid vasculitisEmbolization or – Cholesterolcrystal deposition – Oxalate• Mixed retiform and inflammatory– Henoch-Schönlein – Wegener’s granulomatosis– Livedoid vasculitis – Churg-Strauss syndrome– Septic vasculitis – Polyarteritis nodosa– Chilblain – syndromes– Pyoderma gangrenosumAdapted from Piette WW. 2Based on the clinical and histopathological impressions,a diagnosis of segmental lichen aureus is made.Clinical presentationThere is controversy about whether all the PPD havethe same pathological process. Generally, these eruptionspresent as red-brown to purpuric papules, macules, orplaques, either localized or diffuse, with little or no prurituson the lower extremities of middle-aged individuals.Lesions tend to be chronic, with the most persistentcourse seen in individuals with Schamberg’s disease andpigmented purpuric lichenoid dermatitis (PPLD) ofGougerot and Blum. 5 Typical examples of these entitiesare described below (Table 2)Schamberg’s diseaseAlso known as progressive pigmented purpura andprogressive pigmentary dermatoses, this disease was firstdescribed in 1901 by J.F. Schamberg in a 15-year-old boywith asymptomatic red-brown, hyperpigmented, irregularly-shapedpatches on the lower legs and arms, whoseborders comprised “pinhead-sized, reddish-brown,scarcely elevated puncta or cayenne-pepper spots.” 6 In1918, Kingery reported the first case in the American literatureand identified hemosiderin granules (thought tobe derived from red blood cells that had leaked from disruptedcapillaries) as the pigmentation in Schamberg’sdisease. 7 While later reports described the occurrence ofSchamberg’s disease as being 5 times more frequent inmales, 8 a more recent series showed a slight female predominance,5 with the average age of onset of the diseasein men and women being 48 and 55 years, respectively.Schamberg’s disease is insidious and chronic, beginningasymptomatically on the lower extremities (unilaterallyor bilaterally) as a hyperpigmented patch or as plaquesup to 3 cm in diameter, with red puncta at the periphery.The lesions may become progressively confluent andcommonly assume an annular configuration. Olderlesions may have central atrophy, telangiectasias at themargins, and a darker brown color. 1Majocchi’s diseaseAlso known as purpura annularis telangiectodes, thisdisease was originally described by Majocchi in 1896. 9He described a 21-year-old man who presented with aneruption on the lower legs consisting of 0.5 to 2.0 cmannular purpura with depigmented, atrophic centres andfollicular erythematous puncta. This rare dermatoses ismore common in females, the average age of onset is 30years, and it typically occurs on the lower extremities ina bilateral and symmetrical distribution. The lesions ofMajocchi’s disease evolve through three stages that maybe seen simultaneously on the same patient.

Table 2: Clinical features of the pigmentedpurpuric dermatosesDiseaseSchamberg’s diseaseEczematid-likepurpura of Doucasand KapetanakisPigmented purpuriclichenoid dermatosisof Gougerot and BlumMajocchi’s diseaseLichen aureusDistinguishing featuresMacular; patches and‘cayenne pepper’ puncta withpigmentation as prominentfeatureScaly papular; eczematoidpupura with excoriationsPurpura with lichenoiddermatitisPurpuric annular plaques withatrophic centresGolden lichenoid papulesforming solitary plaque• The first phase consists of a telangiectatic lesioncomprised of a network of dilated capillaries.• The second phase, hemorrhagic and pigmentary, iscomposed of erythematous follicular puncta that enlargein an annular configuration, with the centre becomingyellow to brown.• The last phase becomes apparent as the centre ofthe lesion becomes atrophic, giving the characteristiclesion of purpura annularis telangiectodes. 10The course of the disease is chronic and is oftencharacterized by relapses and remissions over monthsto years.Eczematid-like purpura of Doucas and KapetanakisThis disease was described in 1953 by Doucas andKapetanakis, who followed a series of patients for at leastone year. 11 These patients experience a purpuric eruptionthat begins on the legs and leaves hemosiderindeposits. Lesions begin as red-to-orange (fawn) maculesand develop mild scaliness later in the course of thedisease. This dermatosis usually begins on the lowerextremities of middle-aged adults and may eventuallyinvolve the arms and trunk. It is often bilateral andaccentuated in areas of friction. The disease is noted tohave a seasonal pattern, occurring more frequently in thespring and summer months. 1 This condition is distinguishedfrom Schamberg’s disease by intense pruritus, ashorter course with spontaneous remission, and a higherpredilection for the upper extremities. It has beensuggested that this entity may simply be a pruritic variantof Shamberg’s disease with eczematization of lesionssecondary to excoriation. 10Pigmented purpuric lichenoid dermatitis (PPLD) ofGougerot and BlumIn 1925, Gougerot and Blum described a dermatosisof 15 months duration on the lower extremity of ahealthy 41-year-old male. 12 Several years later, afterencountering 4 similar cases, 13 the authors termed thedisease “dermatite lichénoide purpurique et pigmentée.”The eruption is found predominantly in middle-agedmen and is characterized by smooth, slightly elevated,round or polygonal papules, 0.25 to 2.0 mm in diameter,orange-red in color, involving the lower legs bilaterally, andin some cases the thighs, trunk, and upper extremities.Punctate telangiectasias may be seen within the papules,and the papules frequently coalesce to form plaqueswith overlying scale. These lesions have been reported toshow a striking similarity to Kaposi’s sarcoma. 14Lichen aureusIn 1958, Marten 15 described the entity “lichen purpuricus,”later termed “lichen aureus” by Calnan in1960, 16 highlighting the vibrant color of the lesion.Lichen aureus is characterized by the sudden appearanceof grouped, copper-orange to purple, lichenoid papulesthat form an irregular, usually solitary, sharply demarcatedplaque ranging from 1 to 20 cm in size, with or withouta peripheral red zone. The lesion is often mistakenfor a bruise. The average age of onset is in the mid-30s,with a slight male predominance. 10 The eruption is usuallyasymptomatic, but occasionally, pruritus may benoted by the patient. Lichen aureus most commonlyoccurs on the lower extremities, typically in a unilateraldistribution, although rarely, linear or ‘segmental’ distributionsof lichen aureus have been reported in the literature.3,4,17-22 Cases are summarized in Table 3. Thesegmental variant of lichen aureus has several distinctfeatures.• First, multiple lesions are present in a strikinglinear configuration. The patterns described to date haveall been linear, but they generally failed to correspondwell with Blaschko’s lines, loosely corresponding to dermatomesin 1 case, 22 and approximating underlyingchannels of venous drainage in 2 others. 18,19• Second, while this small series of patients demonstratedno gender predilection and no association withsystemic disease, it did exhibit a somewhat younger agedistribution (mean age 21.5 years), with the youngestreported case in a 4-year-old child.• Finally, unlike the chronic and often permanentcourse typically described in lichen aureus, 23 6 of 13patients with segmental lichen aureus had spontaneousclearing of their lesions within 2 years. 3,4,22

eruption may extend from the lower legs down tothe toes. Other changes due to venous insufficiencymay be present. Hypergammaglobulinemic purpurahas a predominantly petechial component andusually lacks pigmentary changes. Senile purpura,Henoch-Schönlein purpura, viral illness, Kaposi’ssarcoma, and certain conditions like diabetes andrheumatoid arthritis may resemble PPD.Mycosis fungoides (MF) can present with a premycoticeruption resembling PPD. Crowson 28 hasidentified 3 types of atypical pigmentary purpura(APP): typical PP lesions with MF, PP lesions precedingMF, and drug-related PP. 30 All had atypicallymphocytes. The drugs implicated were calciumchannelblockers, lipid-lowering agents, beta-blockers,ACE inhibitors, antihistamines, antidepressants andanalgesics.Work-up and etiological factorsA diagnosis of a pigmented purpuric eruption isbased largely on the clinical presentation. A skinbiopsy serves to confirm the diagnosis. Serial biopsiesmay be necessary to exclude cutaneous T-celllymphoma. The initial work-up should include athorough history, particularly any recent medicationchanges or environmental exposures, and a physicalexam. A full blood count is necessary (to excludethrombocytopenia), as well as a coagulation screen(to exclude other possible causes of purpura). Routinelaboratory studies are invariably normal as areinvestigations for underlying systemic disease.Induction tests of capillary fragility (quantificationof petechiae per unit area after application ofsphygmomanometer pressure on a patient’sappendage for a fixed period of time) are oftenequivocal, and generally unappreciated by patientsalready concerned with cosmesis. In the opinion ofthe authors, liver and renal function tests should beperformed. Immunoglobulins, ANA, RF, ANCA,hepatitis B and C screening, cryoglobulins, cryofibrinogen,and agglutinins are optional.TreatmentTreatment of these idiopathic dermatoses issymptomatic. Offending agents such as drugs andwool should be removed if possible. Topical and systemicsteroids, elastic stockings, antipruritic topicalpreparations, or systemic antihistamines can beused. Anecdotal reports of success in treating PPDwith PUVA, griseofulvin, cyclosporin A, bioflavonoids,and ascorbic acid have been described in avery limited number of patients.ConclusionThe prevailing view of the pigmented purpuricdermatoses is that they represent a group of clinicalpatterns of erythrocyte extravasation due to pericapillaryinflammation. Immunologic studies ofdrug-induced pigmented purpuras suggest that thisinflammatory reaction may be immune-mediated.While the PPD represent a spectrum of benign disease,it is important to differentiate them fromother causes of purpura. Knowledge of the distinctclinical features of segmental lichen aureus will furtheraid the clinician in the diagnosis of PPD.References1. Sherertz. Pigmented purpuric eruptions. Semin ThrombHemost 1984;10(3):190-195.2. Piette WW. The differential diagnosis of purpura from a morphologicalperspective. Adv Dermatol 1994;9:3-23.3. Riordan CA, Darley C, Markey AC, Murphy G, Wilkinson JD.Unilateral linear capillaritis. Clin Exp Dermatol 1992;17:182-185.4. Pock L, Capkova S. Segmental pigmented purpura. PediatrDermatol 2002;19(6): 517-519.5. Ratnam KV, Su WPD, Peters MS. Purpura simplex (inflammatorypurpura without vasculitis); a clinicopathologic study of174 cases. J Am Acad Dermatol 1991;25:642-7.6. Shamberg JF.A peculiar progressive pigmentary disease of theskin. Br J Dermatol 1901;13:1-6.7. Kingery LB. Schamberg’s progressive pigmentary dermatoses:report of a case with histologic study. J Cutan Dis 1918;36:166-72.8. Randall SJ, Kierland RR, Montgomery H. Pigmented purpuriceruptions. Arch Dermatol 1951;64:177-191.9. Majocchi D. Spora una dermatosi telangiectode non ancoradescritta “purpura annularis.” G Ital Mal Vener Pelle 1896;311: 242.10. Lehman M. Benign pigmented purpura, In: Demis DJ, ed.Clinical Dermatology. 26th revision Philadelphia:Lippincott;1999, unit 7-27:1-11.11. Doucas C, Kapetanakis J. Eczematid-like purpura. Dermatologica1953;106: 86-95.12. Gougerot H, Blum P. Purpura angiosclereux prurigineux avecelements lichénoïdes. Bull Soc Fr Dermatol Syphil 1925;32:161.13. Gougerot H, Blum P. Dermatite lichénoïde purpurique et pigmentée:Comparision avec la maladie de Schamberg. ArchDermat Syph Hop St. Louis 1929;1:555-572.14. Wong RC, Soloman AR, Field SI, Anderson TF. Pigmentedpurpuric lichenoid dermatitis (PPLD) of Gougerot and Blummimicking Kaposi’s sarcoma. Cutis 1983;31:406-409.15. Marten R. Case for diagnosis. Trans St Johns Hosp DermatolSoc 1958;40:98.16. Calnan CD. Lichen aureus. Br J Dermatol 1960;72:373-374.17. Takeuchi Y, Chinen T, Ichikawa Y, Ito M. Two cases of unilateralpigmented purpuric dermatosis. J Dermatol 2001;28:493-498.18. Mishra D, Maheshwari V. Segmental lichen aureus in a child.Int J Dermatol 1991;30(9);654-655.19. Ruiz-Esmenjaud J, Dahl MV. Segmental lichen aureus. ArchDermatol 1988;124:1572-1573.20. Rudolf RI. Lichen aureus. J Am Acad Dermatol 1983;8:722-724.21. Abromovits W, Landau JW, Lowe NJ. A Report of two patientswith lichen aureus. Arch Dermatol 1980;116:1183-1184.22. Aoki M, Kawana S. Lichen aureus. Cutis 2002;69:145-148.23. Price ML, Wilson Jones E, Calnan CD, MacDonald DM.Lichen aureus: a localized persistent form of pigmented purpuricdermatitis. Br J Dermatol 1985;112:307-314.DERMATOLOGYRoundsT

24. Pigmented purpuric dermatitis. In: Elder D, ed. Lever’s Histopathologyof the Skin, 8th ed. Philadelphia: Lippincott Williams & Wilkins;1997:202-204.25. Ackerman AB. Persistent pigmented purpuric dermatitis. In: AckermanAB, ed. Histologic Diagnosis of Inflammatory Skin Diseases, 2nded. Philadelphia: Lea & Febiger;1997:609-613.26. Klug H, Haustein UF. Ultrastructure of macrophage-lymphocyteinteraction in purpura pigmentosa progressive. Dermatologica 1976;153:209-217.27. Simon M, Heese A, Gotz A. Immunopathological investigations inpurpura pigmentosa chronica. Acta Derm Venereol (Stockh) 1989;69:101-104.28. Crowson AN. Atypical pigmentary purpura. Hum Pathol 1999;30(9):1004-1012.Abstracts of InterestTreatment of progressive pigmented purpura with oralbioflavonoids and ascorbic acid: an open pilot study inthree patients.REINHOLD U, SEITER S, UGEREL S, TILGEN W., GERMANYBACKGROUND: Bioflavonoids and ascorbic acid have been shownto increase capillary resistance and to mediate potent antioxidativeradical scavenging activities.OBJECTIVE: We evaluated the clinical effect of oral bioflavonoidsand ascorbic acid in patients with chronic progressive pigmentedpurpura (PPP).METHODS: In an open pilot study, oral rutoside (50 mg twice aday) and ascorbic acid (500 mg twice a day) were administered to 3patients with chronic PPP.RESULTS: At the end of the 4-week treatment period, completeclearance of the skin lesions was achieved in all 3 patients. Noadverse reactions were noted. All patients remained free of lesionsat the end of 3 months after treatment.CONCLUSION: Our results suggest a beneficial effect ofbioflavonoids in combination with ascorbic acid on PPP. Because thedisease is mostly resistant to other treatment modalities, placebocontrolledstudies are necessary to determine the usefulness of thistherapy in PPP.J Am Acad Dermatol 1999;41:207-208.Persistent pigmented purpuric dermatitis and mycosis fungoides:simulant, precursor, or both? A study by lightmicroscopy and molecular methods.TORO JR, SANDER CA, LEBOIT PE, CALIFORNIAMycosis fungoides (MF) can present with purpuric lesions, and rarepatients who seemed to have persistent pigmented purpuric dermatitis(PPPD) have developed MF. We recently encountered twopatients referred to our cutaneous lymphoma clinic who had PPPDrather than MF and two others who appeared to have both conditions,leading us to explore the histologic similarities of these diseases.We examined specimens from 56 patients with PPPD todetermine the frequency of MF-like histologic configurations, namely,the psoriasiform lichenoid, psoriasiform spongiotic lichenoid, andatrophic lichenoid patterns. We also noted the degree of spongiosis,epidermotropism, papillary dermal fibrosis, lymphocytic atypia, andepidermal hyperplasia, the number of extravasated erythrocytes andsiderophages, and the distribution of lymphocytic infiltrate withinthe epidermis. In 29 of 56 patients, there were patterns typicallyseen in MF. PPPD can feature lymphocytes aligned along the epidermalside of the dermoepidermal junction, with few necrotic keratinocytes,as can MF. Papillary dermal edema occurred frequentlyin PPPD but not in MF, while lymphocytes in MF but not PPPD hadmarkedly atypical nuclei and had ascended into the upper spinouslayer. Given these similarities, we tested for clonality of the T-cellpopulation using a polymerase chain reaction assay for gammachainrearrangements. Clonal populations were present in three ofthree and one of two specimens from patients with both PPPD andMF, but also in 8 of 12 specimens typical of lichenoid patterns ofPPPD. These findings raise the possibility that the lichenoid variantsof PPPD are biologically related to MF.Am J Dermatopathol 1997;19(2):108-18.Upcoming Scientific Meetings16-19 October 2003Fall Clinical Dermatology ConferenceLuxor, Las VegasCONTACT: Center for Bio-Medical CommunicationTel: 201-883-5874 Fax: 201-342-7555www.cbcbiomed.com15-19 October 2003Dermatology ChicagoWyndham Chicago HotelCONTACT: Skin Disease Educationwww.sdefderm.comFax: 312-988-77596-8 November 2003Canadian Association of Wound Care9 th Annual MeetingToronto, OntarioCONTACT: Tel: 1-877-288-7018 Fax: 1-877-881-0713Website: cawc.net13-16 November 20034 th Annual Las Vegas Dermatology SeminarCONTACT: SDEFFax: 312-988-7759E-mail: sdef@sdefderm.comWebsite: www.sdefderm.com2-6 December 2003Journées Dermatologiques de ParisParis, FranceCONTACT: Tel: (33) 01 44 64 1515Fax: (33) 01 44 64 1516E-mail: p.fournier@colloquium.frChange of address notices and requests for subscriptionsfor Dermatology Rounds are to be sent by mail to P.O. 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