International Journal of Dermatology 2004, 43, 482–488 © 2004 The International Society of Dermatology 482 Pigmented purpuric dermatoses are a spectrum of disorders characterized by a distinct purpuric rash, mainly localized to the lower limbs, which are morphologically dissimilar but histopathologically indistinguishable. Their etiology remains obscure. They are rather resistant to treatment. Although diagnosed quite straightforwardly, the disease entity remains an enigma and a therapeutic challenge. The current narration discusses the outline of the epidemiology, etiopathogenesis, clinical features, histopathology and management of these disorders. The current review, hopefully, may once again rekindle interest in the entity, for it has been sparingly reported or discussed for the benefit of under- and / or postgraduates and those in practice, including family physicians. Blackwell Publishing Ltd. Oxford, UK IJD International Journal of Dermatology 1365-4632 Blackwell Publishing Ltd, 2003 45 Review Progressive pigmentary dermatoses Sardana, Sarkar, and Sehgal REVIEW Pigmented purpuric dermatoses: An overview Kabir Sardana, MD, DNB, MNAMS, Rashmi Sarkar, MD, and Virendra N. Sehgal, MD From the Department of Dermatology and Sexually Transmitted Disease, Lady Hardinge Medical College & Associated SK Hospital, New Delhi, the Department of Dermatology and Venereology, Safdarjung Hospital, New Delhi, and the Dermato-Venereology (Skin/ VD) Center, Sehgal Nursing Home, Panchwati, Delhi, India Correspondence Prof. Virendra N. Sehgal, MD, FNASc, FAMS, FRAS (Lond) A/6 Panchwati Azadpur Delhi − 110 033 India E-mail: drsehgal@ndf.vsnl.net.in Introduction Progressive pigmentary dermatoses (PPD) are chronic and relapsing disorders characterized by a symmetrical rash of petechial and pigmentary macules confined to the lower limbs. Their etiology is largely unknown. Extravasations of erythrocytes in the skin and/or marked hemosiderin deposition are their cardinal clinical expressions. 1–3 A number of clinical patterns of pigmented purpuric dermatoses or capillaritis are recognized that may represent different features of the same disorder with a similar histopa- thology; however, this generally does not influence manage- ment or prognosis. 1,3 They have been traditionally divided into five clinical entities: Progressive pigmented purpuric dermatosis, Purpura annularis telangiectodes, Lichen aureus, Pigmented purpuric lichenoid dermatosis and eczematid- like purpura of Doucas and Kapetanakis. 1 The other rare and unusual presentations include the itching purpura of Loe- wenthal, linear, granulomatous, quadrantic, transitory and familial forms (Table 1). Brief Historical Overview More than 50 years ago, it was recognized that PPDs were purpuric conditions with no defect in platelets, and thus were noninflammatory purpura with no vasculitis. 3 The strik- ing golden-brown pigmentation also distinguished them from other purpuras. 1,3 In the early days, dermatology was an observational science with undue emphasis on the location and shape of the dermatosis. But by the mid 19th century, experience with diseases like psoriasis suggested that distribu- tion and morphology played little role in the pathogenesis of the disease. 3 Nevertheless, their localization to the legs sug- gested a gravitational role, while their annular arrangement suggested a central clearing with peripheral spread. 1,3 Local factors such as drugs and aniline dyes were proposed, but his- tology and capillary microscopy revealed dilated elongated capillaries with red blood cells inciting a mononuclear infil- trate. 1,3 It was suggested that the capillaries of the upper dermis elongate and coil as a result of a stimulus from the epi- dermis. 3 The Gougerot and Blum variant was presumed to be a variant of psoriasis in which, owing to the bleeding in the dermal papillae, the proliferating epidermis was cut off from the blood supply and the influence of the dermis. 3 Experiments Table 1 Classification of pigmented purpuric dermatosis 1. Majocchi’s disease (1896) – (Purpura annularis telangiectaticum) 2. Schamberg’s disease (1901) 3. Pigmented purpuric lichenoid dermatosis of Gougerot and Blum (1925) 4. Eczematoid-like purpura of Doucas and Kapetankis (1953) 5. Itching purpura of Lowenthal (1954) 6. Lichen purpuricus (Martin, 1958; Haber, 1958) Lichen aureus (Calnan, 1960) 7. Transitory PPD (Osment, 1960) 8. Linear PPD (Hersh and Schwayder, 1991) 9. Granuolmatous variant (Saito and Matsuoka, 1996) PPD = pigmented purpuric dermatoses.