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Prurigo Pigmentosa: A Clinicopathological Report of Three Middle Eastern Patients
N Almaani1,2, A H Al-Tarawneh2,3, H Msallam2
1Department of Dermatology, Faculty of Medicine, The University of Jordan, Amman, Jordan.
Abstract:
Prurigo pigmentosa is a unique cutaneous inflammatory disorder characterized by a sudden onset of pruritic and erythematous macules, urticarial papules, and plaques that may coalesce to form a reticulated pattern. Lesions typically heal within weeks leaving a reticulated and mottled postinflammatory hyperpigmentation. The majority of reported cases originate from Japan with much fewer cases described worldwide without predominant ethnicity. The histopathological features of prurigo pigmentosa can be nonspecific; however, distinct features exist for each stage of the disease. The aetiology of prurigo pigmentosa is not fully understood. However, ketoacidosis has been implicated in the pathogenesis and indeed prurigo pigmentosa has been associated with ketoacidotic states such as diabetes mellitus, fasting, dieting, and anorexia nervosa. In this report, we present 3 Jordanian patients with prurigo pigmentosa and describe their clinicopathological features. One patient developed prurigo pigmentosa while fasting during the month of Ramadan and another was undertaking a strict diet. No associations were identified in the third patient. In view of the largely nonspecific clinical and histological features, a high index of suspicion is required as many cases of prurigo pigmentosa are probably undiagnosed.
Insights
Prurigo pigmentosa is a rare skin condition causing itchy, red lesions that heal with mottled hyperpigmentation. This report details three Jordanian cases, highlighting its association with ketoacidosis, fasting, and dieting.
Area of Science:
- Dermatology
- Cutaneous inflammatory disorders
- Pathophysiology of skin conditions
Background:
- Prurigo pigmentosa is a rare inflammatory skin disorder.
- It presents with pruritic, erythematous macules, urticarial papules, and plaques, often forming a reticulated pattern.
- Lesions resolve leaving reticulated postinflammatory hyperpigmentation.
Observation:
- Most reported cases originate from Japan, with fewer documented globally.
- Histopathological features can be nonspecific, varying by disease stage.
- Ketoacidosis, associated with diabetes mellitus, fasting, dieting, and anorexia nervosa, is implicated in its pathogenesis.
Findings:
- This report describes three Jordanian patients with prurigo pigmentosa.
- Two patients developed the condition during fasting (Ramadan) or strict dieting.
- Clinicopathological features of these cases are presented.
Implications:
- The study expands the geographical and ethnic reporting of prurigo pigmentosa.
- It reinforces the association between prurigo pigmentosa and ketoacidotic states.
- A high index of suspicion is crucial for diagnosing prurigo pigmentosa due to nonspecific clinical and histological findings.
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