ABSTRACT
La dermatosis purpúrica pigmentaria (DPP) corresponde a un grupo de enfermedades caracterizadas clínicamente por petequias y coloración bronce de la piel. Generalmente se localiza en las extremidades inferiores y se describen varios tipos, dentro de los cuales se encuentra la variante granulomatosa. Se trata de una dermatopatía benigna, poco frecuente, de etiología aún no precisada y de difícil manejo. Se presenta el caso de una paciente de 48 años, sexo femenino, sin antecedentes mórbidos, que es derivada a dermatología desde reumatología, con el diagnóstico clínico de vasculitis, por la aparición de lesiones maculares y papulares eritematovioláceas, confluentes y pruriginosas en extremidades. Presenta todos sus exámenes normales. Se planteó, entonces, una acroangiodermatitis o liquen plano. Sin embargo, fue necesario llegar al estudio histopatológico para llegar al diagnóstico de dermatosis purpúrica pigmentaria, variante granulomatosa. Si bien se considera que la DPP granulomatosa es una entidad poco frecuente, la literatura sugiere que es una entidad subdiagnosticada. Se debe plantear como diagnóstico diferencial de lesiones cutáneas en extremidades inferiores, lo que determina la importancia de realizar el estudio histopatológico. De esta forma, la DPP granulomatosa será cada vez más común y se plantearán alternativas de tratamiento mejores a las actuales.
The pigmented purpuric dermatoses are a group of diseases characterized clinically by petechiae and bronze skin color, usually affecting the lower extremities. Several types are described and one of them is the granulomatous variant. This is a still rare and benign skin disease of unknown etiology. We present the case of a 48 years old female, previously healthy, derived from rheumatology to dermatology, with the clinical diagnosis of vasculitis due to the presence of confluent erythematous macular and papular lesions, mildly pruritic in lower extremities. Her tests were all in normal ranges. Therefore, an acroangiodermatitis or lichen planus were considered. However, it was necessary to perform a histopathology study to reach the final diagnosis of granulomatous pigmented purpuric dermatosis. It is considered that Granulomatous DPP is a rare entity, but the literature suggests that it is underdiagnosed. It should always be considered as a differential diagnosis of cutaneous lesions located in lower extremities, which determines the importance of histopathology. Thus, the granulomatous DPP will become more common and will be posible to propose better treatment alternatives.
Subject(s)
Humans , Female , Middle Aged , Pigmentation Disorders/pathology , Purpura/pathology , Granuloma/pathology , Lower Extremity/pathology , Diagnosis, DifferentialABSTRACT
Pigmented purpuric dermatoses (PPD) are a group of diseases characterized by petechiae and bronze discoloration of the skin on the lower extremities. Histopathologically, superficial perivascular lymphocytic infiltration and hemosiderin deposition are seen. PPD can be subdivided into progressive pigmentary dermatosis of Schamberg, purpura annularis telangiectoides of Majocchi, pigmented purpuric dermatitis of Gougerot and Blum, eczematid-like purpura of Doucas and Kapetanakis, itching purpura, and lichen aureus. The granulomatous variant of PPD was described in 1996. Granulomatous PPD share common histopathologic features of PPD with granulomatous inflammation. We present the findings of a patient who presented with a clinical picture of PPD and histologic patterns of granulomatous inflammation.
Subject(s)
Humans , Dermatitis , Hemosiderin , Inflammation , Lichens , Lower Extremity , Pruritus , Purpura , Skin , Skin DiseasesABSTRACT
Granulomatous pigmented purpuric dermatosis was first described by Saito and Matsuoka in 1996. Clinically, the lesions manifest purpuric and petechial brown pigmented macules as a result of hemosiderin deposits. Histopathologically, it is characterized by lymphocytic perivascular infiltrate limited to the papillary dermis with extravasated red blood cells and hemosiderin deposition, accompanied by granulomatous infiltration. We report our experience of a case which presented a clinical picture of pigmented purpuric dermatosis and histologically granulomatous inflammation.