Reticulate pigmented anomaly of flexures
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Synopsis

There is a slight female predominance, and all races are affected equally.
Codes
ICD10CM:L81.8 – Other specified disorders of pigmentation
SNOMEDCT:
239054009 – Reticulate pigmented anomaly of flexures
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Differential Diagnosis & Pitfalls
- Confluent and reticulated papillomatosis (Gougerot-Carteaud syndrome)
- Galli-Galli disease – Considered by some to be on the same disease spectrum as DDD or an acantholytic variant of DDD. It is also autosomal dominant associated with loss-of-function mutation in KRT5. Clinically it appears identical to DDD but can be differentiated by the presence of acantholysis on histology.
- Reticulate acropigmentation of Dohi (dyschromatosis symmetrica hereditaria) – Presents as hyperpigmented and hypopigmented macules scattered along the dorsal surface of the distal extremities. In contrast to DDD, onset of this condition is typically in childhood. Epidermal atrophy and elongation of the rete ridges are not seen in this condition, helping differentiate it from DDD on histology.
- Acropigmentation reticularis of Kitamura – Also autosomal dominant, but there is an earlier age of onset and predilection of lesions on acral sites, which can help differentiate this condition from DDD.
- Granular parakeratosis
- Acanthosis nigricans
- Dermatopathia pigmentosa reticularis
- Haber syndrome – Presents with comedones, pitted scarring, hyperkeratotic follicular papules, hyperpigmented macules, and facial erythema and telangiectasias. It can be differentiated from DDD by the presence of facial erythema resembling that seen in rosacea.
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References
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Last Reviewed:10/06/2021
Last Updated:01/23/2022
Last Updated:01/23/2022