Pigmented Purpuric Dermatosis: Clinicopathologic Characterization in a Pediatric Series

Abstract

Pigmentary purpuras (PPs) are a group of chronic disorders of unknown origin seldom described in children. With this study we sought to better characterize PP eruptions, including clinical evolution and management. A retrospective chart review from 2003 to 2013 querying characteristics of children with a biopsy-proven diagnosis of PP in the Centre Hospitalier Universitaire Ste-Justine dermatology clinic (Montreal, Quebec, Canada) was performed. Follow-up was obtained through telephone interviews. Descriptive statistical analysis was used. Of the 17 subjects, 8 were male and the mean age of onset was 9 years. PP was asymptomatic in 11 patients, pruritic in 3, and of cosmetic concern in 3. Schamberg's disease was the most frequent subtype in 12 cases. Resolution of PP was found in 13 cases with a median duration of less than 1 year (range 6 months-9 years). Five patients experienced spontaneous clearing without treatment, and improvement was observed in 75% of cases treated with topical corticosteroids and 100% with narrowband ultraviolet B (nbUVB). No associated disease, significant drug exposure, or contact allergens were found. Those findings support that PPs in children are idiopathic, chronic eruptions that can benefit from watchful waiting, although topical corticosteroids or nbUVB are may be useful if the patient or family desires faster resolution. This study was limited by its small size, its retrospective nature, and selection and recall bias.

The pathological spectrum and clinical correlation of pigmented purpuric dermatosis—A retrospective review of 107 cases

“…Pigmented purpuric dermatosis (PPD) comprises a group of skin diseases characterized by the presence of petechiae and/or purpura, particularly increased progressive pigmentation on the skin of lower extremities. PPD can be divided into several clinical subtypes according to clinical manifestations: non‐blanchable, red‐brown purpuric macules or pinhead sized puncta in Schamberg disease (progressive pigmentary dermatosis), polygonal to round lichenoid purpuric papules and plaques in pigmented purpuric lichenoid dermatosis of Gougerot and Blum (Gougerot‐Blum purpura), annular purpuric patches with telangiectasia in purpura annularis telangiectodes (Majocchi disease), gold‐brown lichenoid patches or plaques in lichen aureus, and eczematous purpuric macules and patches in eczematoid‐like purpura of Doucas and Kapetanakis (eczematoid‐like purpura) . PPD has also been associated with comorbid conditions .…”

Another, new dermatosis in the spectrum of pigmented purpuric dermatoses or atypical variant of lichen aureus?

“…There are limited data concerning PPD in the pediatric population. It is known that all clinical variants may occur in children, while Schamberg purpura is the most common type [5].…”

“…Regardless of that, current evidence shows that in the case of PPD, especially with atypical clinical presentation, histopathological evaluation should always be performed in order to exclude mycosis fungoides mimicking PPD [4,7,8]. Additionally, cases of PPD that przy czym najczęstszym jest choroba Schamberga [5]. Klasyczny obraz liszaja złocistego (lichen aureus -LA) to pojedyncza, asymptomatyczna, złocistobrązowa plama, często z drobnymi grudkami liszajowatymi na obrzeżu, umiejscowiona najczęściej w obrębie kończyn dolnych.…”

Pigmented purpuric dermatosis: a striking but benign cutaneous entity

“…PPD have a predilection for the lower limbs. They are often chronic and relapsing, with median resolution time of <1 year 1. Histology demonstrates a perivascular infiltration with skin-specific T lymphocytes, erythrocyte extravasation and hemosiderin in macrophages 1.…”

“…They are often chronic and relapsing, with median resolution time of <1 year 1. Histology demonstrates a perivascular infiltration with skin-specific T lymphocytes, erythrocyte extravasation and hemosiderin in macrophages 1. Triggers include infections, medications and gravitational dependence 2.…”