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The Pigmented Purpuric Dermatoses: A Review












Members of the Division of Dermatology

Denis Sasseville, MD, Director Editor, Dermatology Rounds Alfred Balbul, MD Alain Brassard, MD Judith Cameron, MD Wayne D. Carey, MD Ari Demirjian, MD Anna Doellinger, MD John D. Elie, MD Odette Fournier-Blake, MD Roy R. Forsey, MD William Gerstein, MD David Gratton, MD Raynald Molinari, MD Brenda Moroz, MD Khue Huu Nguyen, MD Elizabeth A. O'Brien, MD Maria Rozenfeld, MD Wendy R. Sissons, MD Marie St-Jacques, MD Beatrice Wang, MD Ralph D. Wilkinson, MD

The pigmented purpuric dermatoses (PPD) are a group of chronic, benign diseases of unknown etiology that share similar clinical patterns and histological features. The hallmark of this group is a capillaritis with extravasation of erythrocytes and hemosiderin-laden macrophages into the skin that present as petechiae or ecchymoses, papules, or plaques of red, purple, yellow, or brown. The significance of identifying members of this group is in distinguishing them from more threatening causes of purpura, namely the cutaneous vasculitides and thrombocytopenia (Table 1).1,2 This issue of Dermatology Rounds presents a case that describes a 35-year-old white male with an eruptive petechial and purpuric rash in a linear array along the lateral aspect of his right extremity. A biopsy of the lesions confirmed the clinical diagnosis of segmental lichen aureus. It has been recently proposed that this form of lichen aureus is sufficiently clinically distinct to warrant its own designation within the pigmented purpuric dermatoses.3,4 This issue presents a review of all of the reported cases of segmental lichen aureus and discusses the salient clinical and histopathological features of the pigmented purpuric dermatoses.

A case history

A 35-year-old, white, HIV-positive, male presents with a 6-month history of an eruptive petechial and purpuric rash on his right lateral leg and right foot and loss of hair on his extremities. The patient notes that similar lesions appeared on his penis several months after the appearance of the rash. All were asymptomatic. There is no history of trauma or any coagulation abnormalities in the patient or his family. A review of his systems is unremarkable. The patient's medications include indinavir 800 mg b.i.d.; ritonavir 100 mg b.i.d.; lamivudine 150 mg b.i.d.; zidovudine 300 mg b.i.d.; paroxetine 25 mg q.d.; and clonazepam 0.5 mg q.h.s.. On physical exam, the patient has red-brown petechiae and macules ( 1 cm in diameter) grouped in a linear configuration along the lateral aspect of his right thigh to the anterolateral aspect of his right lower leg and foot. Several similar lesions are seen on the penis, sparing the glans. Patchy loss of hair on the extremities and a positive hair pull test are noted. The patient is given a prescription for betamethasone valerate 0.1% to be applied b.i.d. to affected areas, but there is no noticeable fading of lesions. In fact, the follow-up examination at one month reveals a darkening of older lesions, the appearance of several new lesions along the same axis, and several larger macules (up to 2 cm) on the right buttock and perineum, also in a linear array. Laboratory investigations reveal CD4+ 1500 cells/mm and normal hematological studies. Biopsy of the penis reveals nonspecific perivascular and focal interstitial lymphocytic infiltrates, without evidence of significant epidermal change. Iron staining is diffusely positive within the dermis. Biopsy of his right calf shows superficial perivascular and patchy bandlike lymphocytic infiltrates with focal lymphocytic vasculitis. No fluorescence is noted against IgG, IgA, IgM, or C3. Moderate granular fluorescence against fibrin is present at the superficial dermal capillaries.

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The editorial content of Dermatology Rounds is determined solely by the Division of Dermatology, McGill University Health Centre.

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Table 1: Differential diagnosis of purpura

· Nonpalpable Petechia ­ Thrombocytopenia (ITP,TTP, DIC, drug-induced, marrow infiltration) ­ Thrombocytopathies (hereditary, drug-induced, thrombocytosis, renal or hepatic insufficiency, monoclonal gammopathy) ­ Non-platelet­related (PPD, Valsalva maneuver, benign hypergammaglobulinemia) Ecchymotic ­ Procoagulant defect ­ Poor dermal support (actinic damage, corticosteroids, scurvy, amyloidosis, Ehlers-Danlos disease, pseudoxanthoma elasticum) ­ Benign hypergammaglobulinemic purpura ­ Trauma · Palpable with early prominent erythema ­ Small-vessel leukocytoclastic vasculitis (hypersensitivity, Henoch- Schönlein purpura) ­ Small- and medium-sized­vessel leukocytoclastic or granulomatous vasculitis (SLE, RA, PAN, mixed cryoglobulinemia, Wegener's granulomatosis) ­ Small-vessel injury (EM, PLEVA, PPD, benign hypergammaglobulinemic purpura) · Noninflammatory and retiform Platelet­ Heparin necrosis related ­ Thrombotic thrombocytopenic purpura ­ Myeloproliferative disease with thrombocytosis Coldrelated Organism growing in vessels ­ Cryoglobulinemia ­ Cryofibrinogenemia ­ Cold agglutinins ­ Fungi (Mucormycosis) ­ Ecthyma gangrenosum (Pseudomonas sp)

Based on the clinical and histopathological impressions, a diagnosis of segmental lichen aureus is made.

Clinical presentation

There is controversy about whether all the PPD have the same pathological process. Generally, these eruptions present as red-brown to purpuric papules, macules, or plaques, either localized or diffuse, with little or no pruritus on the lower extremities of middle-aged individuals. Lesions tend to be chronic, with the most persistent course seen in individuals with Schamberg's disease and pigmented purpuric lichenoid dermatitis (PPLD) of Gougerot and Blum.5 Typical examples of these entities are described below (Table 2)

Schamberg's disease

Also known as progressive pigmented purpura and progressive pigmentary dermatoses, this disease was first described in 1901 by J.F. Schamberg in a 15-year-old boy with asymptomatic red-brown, hyperpigmented, irregularly-shaped patches on the lower legs and arms, whose borders comprised "pinhead-sized, reddish-brown, scarcely elevated puncta or cayenne-pepper spots."6 In 1918, Kingery reported the first case in the American literature and identified hemosiderin granules (thought to be derived from red blood cells that had leaked from disrupted capillaries) as the pigmentation in Schamberg's disease.7 While later reports described the occurrence of Schamberg's disease as being 5 times more frequent in males,8 a more recent series showed a slight female predominance,5 with the average age of onset of the disease in men and women being 48 and 55 years, respectively. Schamberg's disease is insidious and chronic, beginning asymptomatically on the lower extremities (unilaterally or bilaterally) as a hyperpigmented patch or as plaques up to 3 cm in diameter, with red puncta at the periphery. The lesions may become progressively confluent and commonly assume an annular configuration. Older lesions may have central atrophy, telangiectasias at the margins, and a darker brown color.1

Coagulation ­ Disseminated intravascular anomalies coagulation (DIC) ­ Protein C or S deficiency (homozygous) ­ Acquired protein C deficiency ­ Coumarin necrosis ­ Antiphospholipid antibody (lupus anticoagulant) ­ Paroxysmal nocturnal hemoglobinuria ­ Calciphylaxis ­ Livedoid vasculitis Embolization or crystal deposition ­ Cholesterol ­ Oxalate

Majocchi's disease

Also known as purpura annularis telangiectodes, this disease was originally described by Majocchi in 1896.9 He described a 21-year-old man who presented with an eruption on the lower legs consisting of 0.5 to 2.0 cm annular purpura with depigmented, atrophic centres and follicular erythematous puncta. This rare dermatoses is more common in females, the average age of onset is 30 years, and it typically occurs on the lower extremities in a bilateral and symmetrical distribution. The lesions of Majocchi's disease evolve through three stages that may be seen simultaneously on the same patient.

· Mixed retiform and inflammatory ­ Henoch-Schönlein ­ Wegener's granulomatosis ­ Livedoid vasculitis ­ Churg-Strauss syndrome ­ Septic vasculitis ­ Polyarteritis nodosa ­ syndromes ­ Chilblain ­ Pyoderma gangrenosum

Adapted from Piette WW.2

Table 2: Clinical features of the pigmented purpuric dermatoses

Disease Schamberg's disease Distinguishing features Macular; patches and `cayenne pepper' puncta with pigmentation as prominent feature Scaly papular; eczematoid pupura with excoriations Purpura with lichenoid dermatitis Purpuric annular plaques with atrophic centres Golden lichenoid papules forming solitary plaque

Pigmented purpuric lichenoid dermatitis (PPLD) of Gougerot and Blum

In 1925, Gougerot and Blum described a dermatosis of 15 months duration on the lower extremity of a healthy 41-year-old male.12 Several years later, after encountering 4 similar cases,13 the authors termed the disease "dermatite lichénoide purpurique et pigmentée." The eruption is found predominantly in middle-aged men 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, and in some cases the thighs, trunk, and upper extremities. Punctate telangiectasias may be seen within the papules, and the papules frequently coalesce to form plaques with overlying scale. These lesions have been reported to show a striking similarity to Kaposi's sarcoma.14

Eczematid-like purpura of Doucas and Kapetanakis Pigmented purpuric lichenoid dermatosis of Gougerot and Blum Majocchi's disease Lichen aureus

Lichen aureus

· The first phase consists of a telangiectatic lesion comprised of a network of dilated capillaries. · The second phase, hemorrhagic and pigmentary, is composed of erythematous follicular puncta that enlarge in an annular configuration, with the centre becoming yellow to brown. · The last phase becomes apparent as the centre of the lesion becomes atrophic, giving the characteristic lesion of purpura annularis telangiectodes.10 The course of the disease is chronic and is often characterized by relapses and remissions over months to years. In 1958, Marten15 described the entity "lichen purpuricus," later termed "lichen aureus" by Calnan in 1960,16 highlighting the vibrant color of the lesion. Lichen aureus is characterized by the sudden appearance of grouped, copper-orange to purple, lichenoid papules that form an irregular, usually solitary, sharply demarcated plaque ranging from 1 to 20 cm in size, with or without a peripheral red zone. The lesion is often mistaken for a bruise. The average age of onset is in the mid-30s, with a slight male predominance.10 The eruption is usually asymptomatic, but occasionally, pruritus may be noted by the patient. Lichen aureus most commonly occurs on the lower extremities, typically in a unilateral distribution, although rarely, linear or `segmental' distributions of lichen aureus have been reported in the literature.3,4,17-22 Cases are summarized in Table 3. The segmental variant of lichen aureus has several distinct features. · First, multiple lesions are present in a striking linear configuration. The patterns described to date have all been linear, but they generally failed to correspond well with Blaschko's lines, loosely corresponding to dermatomes in 1 case,22 and approximating underlying channels of venous drainage in 2 others.18,19 · Second, while this small series of patients demonstrated no gender predilection and no association with systemic disease, it did exhibit a somewhat younger age distribution (mean age 21.5 years), with the youngest reported case in a 4-year-old child. · Finally, unlike the chronic and often permanent course typically described in lichen aureus,23 6 of 13 patients with segmental lichen aureus had spontaneous clearing of their lesions within 2 years.3,4,22

Eczematid-like purpura of Doucas and Kapetanakis

This disease was described in 1953 by Doucas and Kapetanakis, who followed a series of patients for at least one year.11 These patients experience a purpuric eruption that begins on the legs and leaves hemosiderin deposits. Lesions begin as red-to-orange (fawn) macules and develop mild scaliness later in the course of the disease. This dermatosis usually begins on the lower extremities of middle-aged adults and may eventually involve the arms and trunk. It is often bilateral and accentuated in areas of friction. The disease is noted to have a seasonal pattern, occurring more frequently in the spring and summer months.1 This condition is distinguished from Schamberg's disease by intense pruritus, a shorter course with spontaneous remission, and a higher predilection for the upper extremities. It has been suggested that this entity may simply be a pruritic variant of Shamberg's disease with eczematization of lesions secondary to excoriation.10

Table 3: Reported cases of segmental lichen aureus Case 11 12 13 14 15 16 17 18 19 10 11 12 Author Brassard et al Pock et al


Age 35 35 28

17 17

Sex M F F F F M M M F F M F

Clinical findings Right buttock and right lateral leg; penis Medial and dorsal aspect right arm; dorsum right hand Right lower abdomen Medial aspect left elbow flexure Flexor surface right lower leg Left knee dorsum; then left buttock, thigh and lower leg Right foot; then right buttock, thigh and lower leg Left buttock; posterolateral left leg to left foot Right ankle, popliteal fossa and buttock Dorsum of left lower leg, "overlying short saphenous vein" Left chest and inner arm overlying brachial and radial veins Medial right upper arm Palmar right index finger

Aoki et al22 Takeuchi et al Takeuchi et al Riordan et al3 Riordan et al Riordan et al

3 3

35 36 13 38 25 34 15 22 21

Mishra et al18 Ruiz-Esmenjaud et al19 Rudolf et al20 Abromovitz et al21


Like the clinical picture of the pigmented purpuras, there is considerable overlap in their histology. The basic process is a capillaritis of the upper dermal vessels that is manifested by a lymphocytic (mainly activated helper T-cells) and histiocytic perivascular infiltrate confined to the papillary dermis, capillary endothelial proliferation, and extravasation of erythrocytes.24 (It has been argued that the label of "capillaritis" is overly generous since fibrin is absent in vessel walls and thrombi are not present in the lumina.25) Hemosiderin may not be detected in early lesions and older, more pigmented lesions may show a less intense inflammatory infiltrate with hemosiderin deposits primarily within macrophages. Hemosiderin may also collect freely in the dermis or within basal epidermal cells. The pattern of the dermal infiltrate is variable and some features are seen more commonly in certain manifestations of this group of diseases. A perivascular bandlike or lichenoid pattern of infiltrate involving the reticular dermis is often seen in PPLD of Gougerot and Blum, while infiltration of the epidermis with mild spongiosis and patchy parakeratosis favours the diagnosis of eczematid-like purpura of Doucas and Kapetanakis. In lichen aureus, a clear (Grenz) zone separates the epidermis from a bandlike mononuclear infiltrate in the superficial dermis; epidermal change is not a significant feature.23 It has been suggested that delayed-type hypersensitivity plays a role in the fragility of capillaries in the pigmented purpuric dermatoses. Electron

microscopic studies of tissue from patients with pigmented purpuric eruptions have revealed Langerhans' cells and macrophages in apposition to lymphocytes and close membrane contacts within dermal infiltrates.26 Positive immunohistochemical staining of lesional keratinocytes for antigens, which are markers for effector and/or accessory cells of the immune system (HLA-DR, OKM 5, OKT 6, Leu-8 and Leu-11b), is evidence of this type IV reaction. In addition, the composition of the inflammatory infiltrate (Leu-4+, Leu-3a+, HLA-PR+, IL-2+) is the same as that occurring in late-phase hypersensitivity.27 The extent to which these signals participate in the localization of T lymphocytes and other inflammatory cells to the epidermis remains to be clarified.

Differential diagnosis

Often, the clinical presentation is adequate to diagnose PPD, but several closely resembling conditions must be excluded. Purpuric drug eruptions often resemble Shamberg's disease and have been most frequently associated with carbromal-containing medications (eg, acetyl carbromal, meprobamate, mephenesin), phenacetin, barbiturates, chlorthiazide, gold, isoniazide, quinidine, sulfonamides, ampicillin, and zomepirac sodium. Clothing purpura usually occurs as a result of contact with wool, usually in middle-aged men with a history of wearing woolen undergarments. It has also been reported from exposure to rubberized or elasticized underwear. Stasis purpura (venous) occurs mainly in men with varicose veins. The




eruption may extend from the lower legs down to the toes. Other changes due to venous insufficiency may be present. Hypergammaglobulinemic purpura has a predominantly petechial component and usually lacks pigmentary changes. Senile purpura, Henoch-Schönlein purpura, viral illness, Kaposi's sarcoma, and certain conditions like diabetes and rheumatoid arthritis may resemble PPD. Mycosis fungoides (MF) can present with a premycotic eruption resembling PPD. Crowson28 has identified 3 types of atypical pigmentary purpura (APP): typical PP lesions with MF, PP lesions preceding MF, and drug-related PP. 30 All had atypical lymphocytes. The drugs implicated were calciumchannel blockers, lipid-lowering agents, beta-blockers, ACE inhibitors, antihistamines, antidepressants and analgesics.


The prevailing view of the pigmented purpuric dermatoses is that they represent a group of clinical patterns of erythrocyte extravasation due to pericapillary inflammation. Immunologic studies of drug-induced pigmented purpuras suggest that this inflammatory reaction may be immune-mediated. While the PPD represent a spectrum of benign disease, it is important to differentiate them from other causes of purpura. Knowledge of the distinct clinical features of segmental lichen aureus will further aid the clinician in the diagnosis of PPD.


Work-up and etiological factors

A diagnosis of a pigmented purpuric eruption is based largely on the clinical presentation. A skin biopsy serves to confirm the diagnosis. Serial biopsies may be necessary to exclude cutaneous T-cell lymphoma. The initial work-up should include a thorough history, particularly any recent medication changes or environmental exposures, and a physical exam. A full blood count is necessary (to exclude thrombocytopenia), as well as a coagulation screen (to exclude other possible causes of purpura). Routine laboratory studies are invariably normal as are investigations for underlying systemic disease. Induction tests of capillary fragility (quantification of petechiae per unit area after application of sphygmomanometer pressure on a patient's appendage for a fixed period of time) are often equivocal, and generally unappreciated by patients already concerned with cosmesis. In the opinion of the authors, liver and renal function tests should be performed. Immunoglobulins, ANA, RF, ANCA, hepatitis B and C screening, cryoglobulins, cryofibrinogen, and agglutinins are optional.


Treatment of these idiopathic dermatoses is symptomatic. Offending agents such as drugs and wool should be removed if possible. Topical and systemic steroids, elastic stockings, antipruritic topical preparations, or systemic antihistamines can be used. Anecdotal reports of success in treating PPD with PUVA, griseofulvin, cyclosporin A, bioflavonoids, and ascorbic acid have been described in a very limited number of patients.

1. Sherertz. Pigmented purpuric eruptions. Semin Thromb Hemost 1984;10(3):190-195. 2. Piette WW. The differential diagnosis of purpura from a morphological perspective. Adv Dermatol 1994;9:3-23. 3. Riordan CA, Darley C, Markey AC, Murphy G, Wilkinson JD. Unilateral linear capillaritis. Clin Exp Dermatol 1992;17:182185. 4. Pock L, Capkova S. Segmental pigmented purpura. Pediatr Dermatol 2002;19(6): 517-519. 5. Ratnam KV, Su WPD, Peters MS. Purpura simplex (inflammatory purpura without vasculitis); a clinicopathologic study of 174 cases. J Am Acad Dermatol 1991;25:642-7. 6. Shamberg JF.A peculiar progressive pigmentary disease of the skin. 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 purpuric eruptions. Arch Dermatol 1951;64:177-191. 9. Majocchi D. Spora una dermatosi telangiectode non ancora descritta "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. Dermatologica 1953;106: 86-95. 12. Gougerot H, Blum P. Purpura angiosclereux prurigineux avec elements 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. Arch Dermat Syph Hop St. Louis 1929;1:555-572. 14. Wong RC, Soloman AR, Field SI, Anderson TF. Pigmented purpuric lichenoid dermatitis (PPLD) of Gougerot and Blum mimicking Kaposi's sarcoma. Cutis 1983;31:406-409. 15. Marten R. Case for diagnosis. Trans St Johns Hosp Dermatol Soc 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 unilateral pigmented purpuric dermatosis. J Dermatol 2001;28:493498. 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. Arch Dermatol 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 patients with 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 purpuric dermatitis. Br J Dermatol 1985;112:307-314.




24. Pigmented purpuric dermatitis. In: Elder D, ed. Lever's Histopathology of the Skin, 8th ed. Philadelphia: Lippincott Williams & Wilkins; 1997:202-204. 25. Ackerman AB. Persistent pigmented purpuric dermatitis. In: Ackerman AB, ed. Histologic Diagnosis of Inflammatory Skin Diseases, 2nd ed. Philadelphia: Lea & Febiger;1997:609-613. 26. Klug H, Haustein UF. Ultrastructure of macrophage-lymphocyte interaction in purpura pigmentosa progressive. Dermatologica 1976; 153:209-217. 27. Simon M, Heese A, Gotz A. Immunopathological investigations in purpura pigmentosa chronica. Acta Derm Venereol (Stockh) 1989; 69:101-104. 28. Crowson AN. Atypical pigmentary purpura. Hum Pathol 1999;30 (9):1004-1012.

atinocytes, as can MF. Papillary dermal edema occurred frequently in PPPD but not in MF, while lymphocytes in MF but not PPPD had markedly atypical nuclei and had ascended into the upper spinous layer. Given these similarities, we tested for clonality of the T-cell population using a polymerase chain reaction assay for gammachain rearrangements. Clonal populations were present in three of three and one of two specimens from patients with both PPPD and MF, but also in 8 of 12 specimens typical of lichenoid patterns of PPPD. These findings raise the possibility that the lichenoid variants of PPPD are biologically related to MF. Am J Dermatopathol 1997;19(2):108-18.

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Abstracts of Interest

Treatment of progressive pigmented purpura with oral bioflavonoids and ascorbic acid: an open pilot study in three patients.

R EINHOLD U, S EITER S, U GEREL S, T ILGEN W., G ERMANY BACKGROUND: Bioflavonoids and ascorbic acid have been shown to increase capillary resistance and to mediate potent antioxidative radical scavenging activities. OBJECTIVE: We evaluated the clinical effect of oral bioflavonoids and ascorbic acid in patients with chronic progressive pigmented purpura (PPP). METHODS: In an open pilot study, oral rutoside (50 mg twice a day) and ascorbic acid (500 mg twice a day) were administered to 3 patients with chronic PPP. RESULTS: At the end of the 4-week treatment period, complete clearance of the skin lesions was achieved in all 3 patients. No adverse reactions were noted. All patients remained free of lesions at the end of 3 months after treatment. CONCLUSION: Our results suggest a beneficial effect of bioflavonoids in combination with ascorbic acid on PPP. Because the disease is mostly resistant to other treatment modalities, placebocontrolled studies are necessary to determine the usefulness of this therapy in PPP. J Am Acad Dermatol 1999;41:207-208.

Persistent pigmented purpuric dermatitis and mycosis fungoides: simulant, precursor, or both? A study by light microscopy and molecular methods.

TORO JR, S ANDER CA, L E B OIT PE, CALIFORNIA Mycosis fungoides (MF) can present with purpuric lesions, and rare patients who seemed to have persistent pigmented purpuric dermatitis (PPPD) have developed MF. We recently encountered two patients referred to our cutaneous lymphoma clinic who had PPPD rather 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 to determine the frequency of MF-like histologic configurations, namely, the psoriasiform lichenoid, psoriasiform spongiotic lichenoid, and atrophic lichenoid patterns. We also noted the degree of spongiosis, epidermotropism, papillary dermal fibrosis, lymphocytic atypia, and epidermal hyperplasia, the number of extravasated erythrocytes and siderophages, and the distribution of lymphocytic infiltrate within the epidermis. In 29 of 56 patients, there were patterns typically seen in MF. PPPD can feature lymphocytes aligned along the epidermal side of the dermoepidermal junction, with few necrotic ker-

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