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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

• Dyschromatosis universalis hereditaria – This can be differentiated histopathologically as amyloid deposits would not be seen.
• Epidermolysis bullosa simplex with mottled pigmentation – This condition includes blisters, which ACD typically does not. These conditions also differ in their histopathologic appearance.
• Dyskeratosis congenita – This condition also includes nail dystrophy, leukoplakia, and pancytopenia.
• Xeroderma pigmentosa – This condition also features photosensitivity and photophobia.
• Addison disease – This is accompanied by characteristic laboratory findings and differs from ACD histopathologically.
• Poikiloderma-like amyloidosis – In addition to skin findings, this condition generally includes short stature, photosensitivity, blisters, and palmoplantar hyperkeratosis.
• Dowling-Degos disease – Reticular hyperpigmentation in this condition is generally in a flexural distribution.
• Naegeli-Franceschetti-Jadassohn syndrome – Presents with reticular pigmentation, palmoplantar keratoderma, acral bullae in infancy, dental abnormalities, and hypohidrosis.
• Dermatopathia pigmentosa reticularis – This is thought to be allelic to Naegeli-Franceschetti-Jadassohn syndrome, with many similar features, but has alopecia, lack of dental anomalies, and stable reticulate hyperpigmentation.
• Weary-Kindler syndrome – Presents with congenital acral bullae, reticulate hyperpigmentation, development of acral keratoses, photosensitivity, poikiloderma, and severe periodontal disease.

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