Pigmented Purpuric Dermatoses.

Pigmented purpuric dermatoses (PPD) are a group of histopathologically similar conditions that are primarily differentiated based on morphology. The basic pathological finding is a lymphocytic perivascular infiltrate with hemorrhage limited to the papillary dermis without fibrinoid necrosis of the vessels. The etiology is unknown; they run a chronic course and are fairly resistant to treatment. We present this review for the physicians to kindle interest in this not-so-uncommon entity.

Characteristics and Clinical Manifestations of Pigmented Purpuric Dermatosis

BACKGROUND: Pigmented purpuric dermatoses (PPD) are a spectrum of disorders characterized by a distinct purpuric rash. Although PPD can be easily diagnosed, the disease entity remains an enigma and a therapeutic challenge. OBJECTIVE: The purpose of this study was to investigate the characteristics and clinical manifestations of PPD and to elucidate the relationship between assumed etiologic factors and the clinical manifestations of PPD and treatment responses. METHODS: Retrograde analyses were performed to identify appropriate PPD patients who visited Korea University Medical Center Anam Hospital from 2002 to 2012. RESULTS: Information on 113 patients with PPD was analyzed, and 38 subjects with skin biopsy were included for this study. Schamberg's disease was the most frequent clinical type (60.5%). Concomitant diseases included hypertension (15.8%), diabetes (10.5%), and others. Associated medication histories included statins (13.2%), beta blockers (10.5%), and others. Possibly associated etiologic factors were recent upper respiratory infection (5.3%), high orthostatic pressure due to prolonged standing (2.6%), and strenuous exercise (2.6%). A total of 36 patients (94.7%) were treated with one or more treatment methods, including oral antihistamines, pentoxifylline, topical steroids, and/or phototherapy. There was no significant difference in disease progress according to underlying diseases, medications, or association factors (p>0.05). CONCLUSION: Our overall results were grossly consistent with the existing literature, excluding several findings. Although a possible relationship between PPD and cardiovascular disease or cardiovascular medication was proposed at the beginning of the study, no statistically significant correlations were found according to the specific clinical types and treatment responses (p>0.05).

Eczematid-like Purpura of Doucas and Kapetanakis Showing Clinical Improvement with Narrowband UVB / 대한피부과학회지

Eczematid-like purpura of Doucas and Kapetanakis is a type of pigmented purpuric dermatoses. It is clinically characterized by pruritic seasonal eruptions occurring in the spring and summer, and histopathologically characterized by spongiosis and parakeratosis in the epidermis and by the lymphocyte-mediated leakage of erythrocytes from capillaries in the papillary dermis. We report a case of eczematid-like purpura of Doucas and Kapetanakis that showed clinical improvement with narrowband UVB (NB-UVB). The patient was a 66-year-old man with pruritic, well-demarcated scaly purpuric patches on his feet that appeared 2 months prior. A histopathological study showed mild superficial perivascular lymphocytic infiltration, focal spongiosis with exocytosis, and erythrocyte leakage. We initiated treatment with an oral antihistamine, ascorbic acid, and a topical steroid, but the lesion was aggravated. We therefore treated the patient with NB-UVB for 6 months, and the lesions regressed progressively with residual postinflammatory hyperpigmentation.

Dermatosis purpúrica pigmentarias: a propósito de 5 casos en adolescentes / Pigmented purpuric dermatoses: five cases report in adolescente patients

Presentamos cinco pacientes adolescentes cuyo motivo de consulta fue la presencia de máculas hiperpigmentadas, parduscas, con lesiones puntiformes rojas que no desaparecían a la vitropresión, distribuidas simétricamente en los miembros inferiores. Los exámenes de laboratorio fueron normales, exceptuando el hallazgo de serología positiva para parvovirus B19 en uno de los pacientes, que presentaba además artralgias. El cuadro se observó también en un paciente con hipercalcemia idiopática y en otro con síndrome de McCune-Albright. La histopatología confirmó el diagnóstico de sospecha: púrpura de Schamberg. El tratamiento fue sintomático, con resolución completa de las lesiones en el transcurso de los meses sucesivos en dos de ellos. La púrpura de Schamberg es la dermatosis purpúrica pigmentaria más frecuente en los niños.

We report five adolescent patients whose reason for consulting was the presence of hyperpigmentedbrownish macules with red pointed injuries distributed symmetrically in lower limbs that didn´tdisappear at finger pressing. Laboratory test were normal except for the discovery of positiveimmunoglobulin M for parvovirus B19 in the patient who presented arthralgias. The symptomswere observed in a case with idiopathic hypercalcemia and in another one with Mc Cune Albrightsyndrome.The histopathology confirmed the diagnosis suspected: Schamberg´s disease. The treatmentwas symptomatic with complete resolution of the injuries in two of the cases in the course of thefollowing months. Schamberg’s purpura is the most frequent pigmented purpuric dermatoses in children.

Expression of CD4,CD8 and receptor ? of T cell in pigmented purpuric dermatoses / 重庆医科大学学报

Objective:To analyze the relationship between PPD and MF on the basis of the surface antigen CD4,CD8 and receptor ? of T cell,so as to provide if PPD can represent a cutaneous lymphoid dyscrasia.Methods:The expression of CD4 and CD8 was measured by immunohistochemical technique in specimens obrained from lesional skin of 23 cases of PPD,20 cases of MF and skin of 20 cases of normal persons.The TCR-gamma gene rearrangement was detected with PCR.Results:15 cases of PPD have obvious migrations of lymphocytes to the epidermis.Their 5 cases and 20 cases of MF show T cell epidermotopism;18 cases of PPD,20 cases of MF and 6 cases of normal persons show a dominance of CD4+.In the dermis,the numbers of CD4+ between PPD and MF are 38.2?30.6 and 68.8?47.6(P

The Clinical Manifestations and Etiologic Factors of Patients with Pigmented Purpuric Dermatoses / 대한피부과학회지

BACKGROUND: Pigmented purpuric dermatoses comprise a group of benign dermatoses that are clinically characterized by pinpoint petechiae and purpura on the hyperpigmented base, and histologically by capillaritis without any fibrinoid necrosis of the vessel. The etiology of pigmented purpuric dermatoses is unknown. Identification of triggering factors will contribute to find out the pathogenesis of the pigmented purpuric dermatoses. OBJECTIVES: The aim of this study was to investigate the clinical manifestations of pigmented purpuric dermatoses and to elucidate the relationship between the causative factors and clinical manifestation of pigmented purpuric dermatoses. METHODS: Clinical observation and review of medical records, photographs, and histopathologic specimens were made by analyzing 37 cases of patients with pigmented purpuric dermatoses who visited Our Lady of Mercy Hospital from 1995 to 2003. The present medical status of each patient was surveyed by phone and detailed reviews of the clinical records included attention to clinical manifestations, possible etiologic factors and coexisting diseases of each patient. The statistical methods used was one-way ANOVA test. RESULTS & CONCLUSIONS: The results were as follows; 1. Female to male ratio was 1.47: 1. The mean age at diagnosis was 45.6+/-17.2 years. 2. The mean duration of disease was 10.2+/-9.0 months, and the disease that localized to the lower limbs was in 75.7% of patients. 3. Of the 37 patients, 23 patients were of the Schambergs disease (62.2%), 11 patients were of the Majocchis disease (29.7%), 2 patients were of the Lichen aureus (5.4%), and one patient was of the Eczematid-like purpura of Ducas and Kapetanakis (2.7%). 4. The 37 patients were treated with topical or systemic steroid (73%), pentoxyfylline (10.8%), and there was no difference in the clinical course according to the treatment. Of the 37 patients, 18 patients had improvement or clearing of their lesions. 5. The etiologic factors identified were, orthostatic hypertension (21.6%), exercise (8.1%), metals (5.4%), NSAIDs (2.7%), heat (2.7%), and a wide spectrum of disease was present in patients with pigmented purpuric dermatoses. 6. The 14 of 22 patients without etiologic factors had medical problems, whereas 3 of 15 patients with etiologic factors had medical problems. The 5 of 15 patients with etiologic factors had clearance or improvement of their lesions, but 13 of 22 patients without etiologic factors had clearance or improvement of their lesions.

A Case of Pigmented Purpuric Lichenoid Dermatitis of Gougerot-Blum / 대한피부과학회지

Pigmented purpuric lichenoid dermatitis of Gougerot-Blum is one of the diseases classified under the group name of pigmented purpuric dermatoses that are likely variants of each other resulting from lymphocyte-mediated leakage of erythrocytes. This is characterized by minute, rust-colored, lichenoid papules that tend to fuse into plaques with indistinct borders and develop mainly on the legs, thighs, and lower trunk. We report a case of pigmented purpuric lichenoid dermatitis of Gougerot-Blum on the lower abdomen, buttocks, and lower legs in a 69-year-old male patient.