Dowling Degos disease (DDD) and Reticulated Acropigmentation of Kitamura (RAK) are rare genodermatoses inherited as an autosomal dominant trait with variable penetrance. They are considered to be part of a spectrum of reticulated pigmentary dermatoses. A 20 year old women presented with multiple hyperpigmented reticulated macules of size 1-2 mm present over face, neck, maxilla, trunk, genitals and oral mucosa. In addition with reticulated acropigmentation of dorsum of hands and feet, pitting over palmer creases and pits over face with multiple hypopigmented macules over the trunk. Systemic examination and Routine Laboratory investigation were within normal limits. Her family history included similar hyperpigmented lesions in her sister. Skin biopsy was also done. Although rare, but our case connects the overlap between RAK with DDD, the patient showed unique clinical as well as histopathological overlap. When encountering reticulated hyperpigmentation disorders, it is important to recognize the distress they may impart on the patient. Patient was treated with topical tretinoin 0.05% with Azelic acid 20%, but mild improvement was noticed. Unfortunately, these disorders are difficult to manage due to limited therapeutic options.
Published Date: 2020-08-31;