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Chapter 130: Pigmented Purpuric Dermatoses

Sima Lynn Sharara; Farah Succaria

PIGMENTED PURPURIC DERMATOSES

Therapeutic Approach

Pigmented purpuric dermatoses (PPD) encompass subtypes of generally benign, chronic purpuric cutaneous eruptions, most commonly over the lower extremities, characterized by erythematous to violaceous macules, patches, and petechiae. PPD classifications include progressive pigmentary purpura or Schamberg’s disease (PPP), purpura annularis telangiectodes of Majocchi (PATM), lichen aureus (LA), pigmented purpuric lichenoid dermatitis of Gougerot and Blum (PPLD), eczematoid-like purpura of Doucas and Kapetanakis (EP), with other rare variants including disseminated pruriginous angiodermatitis, unilateral linear capillaritis (ULC), and granulomatous pigmented purpura (GPP). Treatment of PPD primarily focuses on managing the underlying aggravating factors including compression for venous insufficiency, discontinuation of suspected PPD-inducing drugs, as well as topical and systemic therapies outlined below. PPD are usually persistent and resistant to therapy. See Table 130-1.

Table 130-1Pigmented Purpuric Dermatoses Treatment Table
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Table 130-1 Pigmented Purpuric Dermatoses Treatment Table
MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE
Topical Therapy
Topical corticosteroids PPLD, LA, GPP Anti-inflammatory No standardized recommendations, medium to high potency QD-BIDa Skin atrophy None III1-7
Calcineurin inhibitors LA Anti-inflammatory Pimecrolimus 1% cream, tacrolimus 0.1% ointment twice daily Transient burning and erythema None III8,9
Systemic Therapy
Rutoside+Ascorbic acid PPP, EP, PPD Anti-oxidative, reduced vascular permeability, enhancement of endothelial barrier 50 mg BID+100 mg QD Rare palpitations, dizziness, abdominal pain None II10-13
Pentoxifylline PPD, PPLD, LA, PPD Reduce T-cell adherence to endothelial cells and keratinocytes 300 mg QD, 400 mg BID-TIDb GI side effects Renal function; hemoglobin/hematocrit (high-risk patients) III, Ib14-20
Venoactive agents with oral flavonoidsc and calcium dobesilate PPP Antioxidative, decreased capillary permeability Diosmin 450 mg QD, hesperidin 50 mg QD, euphorbia prostate extract 100 mg QD, calcium dobesilate 500 mg QD None reported, one case report None III21
Calcium dobesilate PPP Inhibition of prostaglandins and thromboxanes synthesis, downregulation of VEGF expression 500 mg QD Burning sensation in the epigastrium None III21,22
Colchicine PPP, PATM Immunomodulatory (reduce T-cell chemotaxis) 0.5 mg BID Contraindicated in the presence of any degree of renal or hepatic impairment in patients receiving a P-gp inhibitor or an agent that strongly reduces CYP3A4 availability None III23-25
Griseofulvin PPP, EP Immuno­modulatory 500-750 mg QD Not reported If long term, hemoglobin/hematocrit; renal and hepatic III26
Cyclosporine PPD (not specified) Immuno­modulatory (decreased T-cell function) 5 mg/kg/d, tapering to 1.5 mg/kg Hypertension Renal function, blood pressure, plasma levels III27
Methotrexate PATM Anti-inflammatory (decreases expression of skin-homing molecules) 15 mg/wk GI side effects CBC, CMP III28
Procedural Therapy
PUVA LA, PPP, PPLD, PATM, ULC Immuno­modulatory (impaired T cell chemotaxis and IL2 production) 7-20 treatments (cumulative dose 16-49 J/cm2)33, twice weekly (started at 0.5 J/cm2, increased 40% each visit),29 3 times weekly for 10 wk (cum dose 200 J/cm2),30 cumulative dose ...

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