ABSTRACT
Unilateral linear capillaritis (ULC) is a rare variant of pigmented purpuric dermatosis (PPD). ULC is clinically characterized by unilateral linear macules on the lower extremities, which fade spontaneously. We present three patients with linearly distributed purpuric macules on only one side of the lower extremities. Skin biopsy showed histopathological features of PPDs. We diagnosed ULC based on these clinicopathological findings. For all patients, the skin lesions began to fade spontaneously within several months to a few years after onset. ULC has been reported rarely, with only about 20 cases worldwide and no cases described in the Korean literature.
Subject(s)
Humans , Biopsy , Lower Extremity , Skin , Skin DiseasesABSTRACT
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
El liquen aureus es una dermatosis purpúrica pigmentaria crónica. Se caracteriza por la presencia de máculas, pápulas y placas de color marrón dorado, localizadas en los miembros inferiores. A nivel histológico se observaz un infiltrado linfocítico liquenoide y perivascular, con extravasación de eritrocitos en la dermis. La dermatoscopia evidencia un patrón purpúrico moteado sobre un fondo amarillento. Se presenta el caso de un paciente de 19 años que consultó por máculas, pápulas y placas de color marrón dorado, asintomáticas, ubicadas en la cara externa del muslo izquierdo, de 7 meses de evolución. Se realizó dermatoscopia y estudio histopatológico, e inició tratamiento con tacrolimus ungüento al 0,1%, con mejoría clínica sin progresión del cuadro (AU)
Lichen aureus is a chronic pigmentary purpuric dermatosis. Clinically is characterized by macules, papules and plaques of golden-brown coloration, located in lower extremities. Histologically by a lichenoid and perivascular lymphocytic infiltrate with extravasation of erythrocytes in the dermis. Dermoscopy show a speckled purpura pattern on a yellowish background. We report a 19 year old man who presented asymptomatic macules, isolated papules and golden-brown plaques on his left thigh for 7 months. Dermoscopy and histopathology study confirmed the diagnosis and treatment with tacrolimus 0.1% ointment was indicated, achiving clinic improvement and no progression of previous lesions (AU)
Subject(s)
Humans , Male , Adult , Pigmentation Disorders/diagnosis , Skin Diseases/diagnosis , Signs and SymptomsABSTRACT
Pigmented purpuric dermatosis (PPD) represents a group of cutaneous lesions exhibiting petechiae, pigmentation, and occasionally telangiectasia in the absence of an associated venous insufficiency or hematological disorder. PPD may resolve spontaneously but tends to persist for months to years. Various treatment modalities such as oral griseofulvin, pentoxifylline, cyclosporine, ascorbic acid, topical corticosteroids, and PUVA therapy have been used with unsatisfactory results. Recently, some studies reported that PPD showed a dramatic response to narrowband ultraviolet B (UVB) phototherapy. In these studies, narrowband UVB phototherapy was an effective treatment method with few side effects. Here, we present the case of a 7-year-old boy with generalized PPD that improved rapidly following narrowband UVB phototherapy.
Subject(s)
Child , Humans , Male , Adrenal Cortex Hormones , Ascorbic Acid , Cyclosporine , Griseofulvin , Pentoxifylline , Phototherapy , Pigmentation , Purpura , PUVA Therapy , Skin Diseases , Telangiectasis , Venous InsufficiencyABSTRACT
BACKGROUND: Pigmented purpuric dermatosis (PPD) comprises a group of dermatoses characterized clinically by pinpoint petechiae and purpura with a brown, red, or yellow patchy pigmented base. Histopathologically, extravasation of erythrocytes with hemosiderin deposition, a perivascular lymphocytic infiltrate on the superficial capillaries, and endothelial cell swelling are observed; however, their etiology remains obscure. There have been few reports regarding the clinical and histopathological characteristics of PPD in Korea. OBJECTIVE: To investigate the epidemiology, etiology, clinical manifestations, and histopathological features of PPD in Korea. METHODS: We retrospectively evaluated the clinical manifestations and histopathological features of 84 patients with PPD diagnosed at our center between January 2003 and December 2013. RESULTS: Of the 84 patients, 44 were male and 40 were female. The mean age of occurrence was 44.6 years and, the most commonly involved sites were the lower extremities (95.2%). Most of the PPD patients were asymptomatic (65.5%), however, others complained of itching (25%), pain (3.6%), heat sensations (3.6%), or tingling sensations (2.3%). The majority (63.1%) had Schamberg's disease, 15.5% had Majocchi's disease, 10.7% had eczematid-like purpura of Doucas and Kapetanakis, 7.1% had Gougerot-Blum syndrome, and 3.6% had lichen aureus. Of the 84 patients, 35.7% of patients had medical problems and 32.1% had etiologic factors including orthostatic hypertension (13.2%), trauma (7.2%), contact dermatitis (4.8%), sex hormones (4.8%), postpartum (1.2%), and diltiazem (1.2%). Histopathologically, extravasation of erythrocytes with or without hemosiderin deposit and a mild-to-dense perivascular or lichenoid lymphocytic infiltrate in the upper dermis were common features. A review of the prognoses of 72 patients revealed that 52.8% were clinically improved and that the most common treatment modality was a topical steroid (79.2%). CONCLUSION: Study results suggested variable clinical manifestations with common histologic features of PPD, and that variable etiologic factors could trigger the occurrence of PPD. We recommend the addition of both trauma and sex hormones as etiologic factors. There was no significant difference between the treatment modalities and the level of clinical improvement.
Subject(s)
Female , Humans , Male , Capillaries , Dermatitis, Contact , Dermis , Diltiazem , Endothelial Cells , Epidemiology , Erythrocytes , Gonadal Steroid Hormones , Hemosiderin , Hot Temperature , Hypertension , Korea , Lichens , Lower Extremity , Pigmentation Disorders , Postpartum Period , Prognosis , Pruritus , Purpura , Retrospective Studies , Sensation , Skin DiseasesABSTRACT
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
Lichen aureus is a rare type of chronic pigmented purpuric dermatosis. The eruptions consist of discrete or confluent golden to brownish lichenoid macules and papules, and are usually asymptomatic. Lichen aureus commonly occurs in young adults, but less frequently in children. We report the first case of multiple lichen aureus occurring in a Korean child.
Subject(s)
Child , Humans , Young Adult , Lichens , 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.