Segmental pigmented purpura

Lumír Pock1, Stepánka Capková

  • 1Dermatohistopathological Laboratory, 2nd Medical School, Charles University, Prague, Czech Republic.

Pediatric Dermatology
|November 20, 2002
PubMed

Insights

A rare segmental distribution of pigmented purpura (PP) was observed in a pediatric patient. This case highlights the importance of considering vascular and immunologic factors in pediatric skin conditions.

Area of Science:

  • Dermatology
  • Pediatrics
  • Vascular Biology

Background:

  • Pigmented purpura (PP) typically presents with a generalized distribution.
  • Segmental PP, a rare variant, poses diagnostic challenges, particularly in pediatric cases.

Observation:

  • A 5-year-old patient presented with a 7-month history of pruritic, red-brown macules and telangiectases on the chest and right upper extremity.
  • Skin biopsy revealed epidermal hydropic degeneration, vascular dilation, extravasated erythrocytes, and lymphocytes.

Findings:

  • Histopathology confirmed features consistent with pigmented purpura.
  • The patient also exhibited an open foramen ovale, hypogammaglobulinemia (G and A), and increased blood vessel fragility.
  • The skin lesions showed significant improvement within 18 months.

Implications:

  • This case underscores the variability in PP presentation and distribution.
  • Associated systemic findings suggest potential underlying vascular or immunologic dysregulation.
  • Further research into the pathogenesis of segmental PP and its systemic associations is warranted.

Related Concept Videos

Pigmentation01:19

Pigmentation

The color of the skin is influenced by a number of pigments, including melanin, carotene, and hemoglobin. Recall that melanin is produced by cells called melanocytes, which are found scattered throughout the stratum basale of the epidermis. The melanin is transferred to the keratinocytes via melanosomes.
Melanin occurs in two primary forms: eumelanin that provides black and brown pigment and pheomelanin that provides red color. Dark-skinned individuals produce more melanin than those with pale...
Peptic Ulcer01:27

Peptic Ulcer

Peptic ulcers are erosive lesions of the gastric or duodenal lining, most commonly caused by Helicobacter pylori infection. This Gram-negative, helical bacterium has adapted to survive the stomach’s acidic environment by producing urease, which converts urea into ammonia and carbon dioxide. The ammonia neutralizes gastric acid in the bacterium’s immediate environment, allowing colonization of the gastric mucosa. H. pylori attaches to mucus-secreting epithelial cells, penetrates the mucus...
Pleiotropy01:47

Pleiotropy

Pleiotropy is the phenomenon in which a single gene impacts multiple, seemingly unrelated phenotypic traits. For example, defects in the SOX10 gene cause Waardenburg Syndrome Type 4, or WS4, which can cause defects in pigmentation, hearing impairments, and an absence of intestinal contractions necessary for elimination. This diversity of phenotypes results from the expression pattern of SOX10 in early embryonic and fetal development. SOX10 is found in neural crest cells that form melanocytes,...
Peptic Ulcer Disease III: Clinical Manifestations and Complications01:25

Peptic Ulcer Disease III: Clinical Manifestations and Complications

Duodenal UlcersDuodenal ulcers are the most common form of peptic ulcer disease, presenting with chronic, intermittent epigastric pain. Pain typically appears 2–3 hours after meals, especially when the stomach is empty, often waking patients at night. It is characteristically relieved by food or antacids (“pain–food–relief”). Some patients remain asymptomatic until complications like bleeding or perforation emerge, particularly with NSAID or anticoagulant use.Gastric UlcersGastric ulcers share...
Skin Cancer01:30

Skin Cancer

Skin cancer is a type of cancer that occurs when there is an abnormal growth of skin cells, usually triggered by damage to the DNA within the skin cells. It is primarily caused by exposure to ultraviolet (UV) radiation from the sun or artificial sources like tanning beds. Skin cancer is the most common type of cancer worldwide, and its incidence continues to rise.
Basal Cell Carcinoma (BCC): BCC is the most common type of skin cancer, accounting for about 80% of cases. It typically develops in...
Peptic Ulcer Disease I: Introduction01:30

Peptic Ulcer Disease I: Introduction

Peptic Ulcer Disease (PUD) is characterized by mucosal excavation in the esophagus, stomach, pylorus, or duodenum. It can manifest as acute or chronic based on the extent and duration of mucosal involvement.
An acute ulcer, marked by superficial erosion and minimal inflammation, swiftly resolves upon identifying and addressing the underlying cause. In contrast, a chronic ulcer persists, potentially eroding through the muscular wall and forming fibrous tissue.
Peptic ulcers can also be...