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Unusual hyperpigmentation developing in congenital reticular ichthyosiform erythroderma (ichthyosis variegata)
A Brusasco1, S Cambiaghi, G Tadini
1Centre for Inherited Cutaneous Diseases, Institute for Dermatological Sciences, IRCCS, Ospedale Maggiore, University of Milan, Via Pace 9, 20122 Milan, Italy.
The British Journal of Dermatology
|January 20, 1999
Summary
A new clinical feature, dark hyperpigmented macules, emerged in a patient with congenital reticular ichthyosiform erythroderma. This finding, linked to melanosome accumulation, is unique among ichthyotic disorders.
Area of Science:
- Dermatology
- Genetics
- Pathology
Background:
- Congenital reticular ichthyosiform erythroderma (CRIE) is a rare ichthyotic disorder.
- It is characterized by erythematous skin and a distinct ultrastructural pattern in keratinocytes.
Observation:
- A 23-year-old woman with CRIE developed unusual, nearly black hyperpigmented macules on her limbs at age 18.
- These lesions were not previously documented in CRIE or other ichthyotic conditions.
Findings:
- Histological analysis confirmed the lesions' association with CRIE.
- Melanosome accumulation in activated dendritic melanocytes caused the dark pigmentation.
- A pigment transfer defect in pathological keratinocytes was hypothesized for postinflammatory hyperpigmentation.
Implications:
- This case highlights a novel clinical manifestation in CRIE.
- Understanding this feature may offer insights into pigmentary abnormalities in ichthyotic disorders.
- Epidermal hyperplasia might play a role in the absence of similar findings in other erythrodermic ichthyoses.