Granulomatous pigmented purpura: report of a case and review of the literature

Abstract

The pigmented purpuric dermatoses (PPD) are a group of diseases characterized by petechiae and bronze discoloration of the skin on the lower extremities. Histopathologically, extravasation of erythrocytes with hemosiderin deposition, a perivascular lymphocytic infiltrate centered on the superficial capillaries and endothelial cell swelling are seen. The granulomatous variant of PPD (GPPD) was described in 1996 and only 10 cases have been reported since in the literature, almost exclusively in patients of East Asian descent only involving the extremities. We present a case of GPPD in a Caucasian, North American Ashkenazi Jewish woman involving the thighs, back, forearms and wrists with concomitant non-granulomatous PPD of the shins. She presented with an asymptomatic, spreading, cayenne pepper-like rash. This rash intermittently involved the lower extremities and back for 15 years, but now involves the thighs with accompanying pink papules on the back, wrists and forearms. Histopathology of the thigh and back lesions revealed superficial lichenoid granulomatous dermatitis with palisading lymphocytes and focal interface changes. Extravasated erythrocytes were seen, but vasculitis was absent. No lymphocytic atypicality was noted and T-cell gene rearrangement studies were non-clonal. This is the second reported case of GPPD in a non-Asian patient and the first case involving sites other than the extremities.

Quiz Correct answer to the quiz. check your diagnosis Granulomatous pigmented purpura in an adolescent girl: a precursor of mycosis fungoides?

“…Since the first description of granulomatous variant of PPD in 1996 by Saito and Matsuoka in 1996, 12 additional cases have been published [1][2][3][4][5]. The majority of the patients, whose ages ranged from 22 to 71 years, were of East Asian descent.…”

Granulomatous Pigmented Purpuric Dermatitis Associated with Primary Sjögren’s Syndrome

“…To our knowledge, it has been described in only 14 patients in the English literature (1)(2)(3). In addition, it was reported that approximately 29% of patients (4/14) showed serological evidence of autoimmune dysregulation, including positive ANA and rheumatoid factor tests (2, 3).…”

“…These reports suggested significant relationships between GPPD and autoimmune disorders (2^). In addition, Kaplan et al (2) recently reported the immunological profiles of GPPD and described the infiltration of significant numbers of CD4+ cells in granulomatous tissues, suggesting that, like sarcoidosis, immunological mechanisms might be associated with the formation of granuloma.…”

Dermatosis purpúrica pigmentaria, variante granulomatosa, a propósito de un caso

“…Dependiendo de la extensión y de la morfología clínica, se reconocen distintas variantes 1,4,5,6 (Tabla 1).…”

“…Se debe hacer un análisis de la clínica, histología e inmunohistoquímica. 1 Hasta el momento no existe un tratamiento estándar. Si se identifica un agente etiológico, debe suspenderse y puede resolverse el cuadro.…”