Clinical Dermatology Review

ONLINE ONLY ARTICLES - CASE REPORT
Year
: 2022  |  Volume : 6  |  Issue : 2  |  Page : 153-

Reticulate acropigmentation of dohi: Dermatoscopic features in two cases


Jinal Jainendra, Juhi Devyangbhai Shah, Dhruv Ramanbhai Patel, Pragya Ashok Nair 
 Department of Dermatology, Pramukhswami Medical College, Shree Krishna Hospital, Karamsad, Gujarat, India

Correspondence Address:
Pragya Ashok Nair
Department of Dermatology, Pramukhswami Medical College, Shree Krishna Hospital, Karamsad - 388 325, Gujarat
India

Abstract

Reticulate acropigmentation of Dohi is a rare genodermatosis inherited as an autosomal dominant trait. It is a localized form of dyschromatosis universalis hereditarian, characterized by the presence of hyperpigmented and hypopigmented macules with symmetrical, irregular size and shape forming a reticulate pattern over the dorsa of the hands and feet. Onset is normally in the first decade but occasionally be delayed. Biopsy is not diagnostic but helps to rule out its differentials. Dermoscopy is a new investigative tool which gives specific characteristic changes. We report two cases of acropigmentation of Dohi with classical dermatoscopic changes reported previously and some new features. No treatment is effective for this genodermatosis.



How to cite this article:
Jainendra J, Shah JD, Patel DR, Nair PA. Reticulate acropigmentation of dohi: Dermatoscopic features in two cases.Clin Dermatol Rev 2022;6:153-153


How to cite this URL:
Jainendra J, Shah JD, Patel DR, Nair PA. Reticulate acropigmentation of dohi: Dermatoscopic features in two cases. Clin Dermatol Rev [serial online] 2022 [cited 2022 Dec 3 ];6:153-153
Available from: https://www.cdriadvlkn.org/text.asp?2022/6/2/153/354734


Full Text



 Introduction



Dyschromatoses are autosomal dominant pigmentary disorders characterized by the presence of hyper and hypopigmented macules arranged in a reticular pattern. Generalized form is dyschromatosis universalis hereditarian (DUH), and localized form is dyschromatosis symmetrica hereditarian (DSH), also called reticulate acropigmentation of Dohi (RAD). It is a rare genodermatosis, first described in patients from Japan in 1924,[1],[2] a few cases have been reported from India, Europe, and South America.[3] Eight cases belonging to two unrelated Indian families, who had typical features of RAD, are being reported.[2] Biopsy does not have any specific features but will help to rule out other mimics. Dermatoscopy can help in diagnosis which shows characteristic findings.

 Case Reports



Case 1

A 30-year-old male presented to skin outpatient department with a complaint of lesions over the neck since 13 years. The lesions gradually progressed to involve other body sites with itching. No history of fever, contact with any chemical, photosensitivity, photophobia, and any allergy was present. He is a known case of epilepsy since 2007, is on tablet levetiracetam. No co-morbidities or specific family history was present.

Cutaneous examination showed multiple, well-defined, reticular, hyperpigmented, and hypopigmented macules over chest [Figure 1], abdomen, back, [Figure 2]a and [Figure 2]b. Genitals, bilateral upper and lower limbs including palms and soles [Figure 3]a and [Figure 3]b. Scalp and face showed pigmentation with scaling [Figure 1]. Bilateral finger and toenails showed dystrophic changes with longitudinal ridges and blackening. No mucosal lesions, palmar pits, or freckle-like macules were seen over the face.{Figure 1}{Figure 2}{Figure 3}

Dermatoscopy done with handheld LED ILLUCO polarized dermoscope IDS-1100 having ×10 magnification showed reticulate pigment network in honeycomb pattern against pinkish to brownish background with focal hyper and hypopigmentation [Figure 4].{Figure 4}

Biopsy was done keeping differential diagnosis of RAD and DUH. Histopathology showed loss of rete ridges and increased melanin (from hyperpigmented lesions) [Figure 5]a and normal melanin (from hypopigmented lesions) [Figure 5]b in basal and spinous layer with vacuolar degeneration of basal keratinocytes. The underlying upper dermis showed mild perivascular infiltrate and pigmentary incontinence.{Figure 5}

Case 2

A 5-year-old female presented to the skin outpatient department with asymptomatic lesions over whole body since 4 years. No systemic complaints were present. No history of photo or seasonal aggravation, or any allergy present.

Cutaneous examination showed multiple, hyperpigmented and hypopigmented, macules over nape of neck, chest, upper back, buttocks [Figure 6], bilateral upper limbs with palms [Figure 7], and lower limbs including dorsum of foot and soles [Figure 8]a, [Figure 8]b, [Figure 8]c. A few freckles like macules over face and single, hypopigmented patch over labia majora. Oral cavity, nails, and hairs were normal. Dermatoscopy showed reticulate pigment network with focal hypopigmentation and some white dots. Routine investigations in both the patients were in normal range [Figure 9].{Figure 6}{Figure 7}{Figure 8}{Figure 9}

On the basis of clinical, dermatoscopic and histopathological examinations both cases were diagnosed as RAD.

 Discussion



RAD is a rare dyschromatosis characterized by mottled pigmentation developing on acral areas.[1] The exact pathomechanism is not yet understood through a resemblance to reptilian skin, and an evolutionary process mediated by embryonic neural reflexes expressed only in genetically predisposed individuals has been suggested.[2] A genetic mutation has been identified on chromosome IqII-Iq2I as responsible for the production and distribution of melanin. Miyamura et al. were the first to identify the autosomal dominant form of DSH due to mutation in the DSRAD or ADAR1 gene.[4] Adult-onset dyschromatosis often is secondary to chemicals, drugs, physical agents, cutaneous lupus erythematosus, or infection.[5]

The onset is normally reported to occur in the first decade, but occasionally onset at later age may occur as seen in our first case.[2]

RAD is characterized by the presence of hyperpigmented and hypopigmented macules, symmetrical, irregular in size and shape forming a reticulate pattern over the dorsa of the hands and feet and occasionally on the arms and legs.[1] Both of our cases showed the involvement of arms and legs. Skin lesions remain localized on extremities in nearly half of the patients and can also affect the face in the remaining half associated with stabilization in adolescence. Some patients show freckle-like macules on the face as in our second case.[3] There are isolated reports of association with neurofibromatosis type I, thalassemia, polydactyly, and torsion dystonia.[6] The involvement of palms and soles is unusual[2] but it was seen in both our cases without any similar familial complaints.

Dermatoscopy of the pigmented areas shows reticulated hyperpigmented spots, monotonous pigmented spots, reticulated hypopigmented spots, or monotonous hypopigmented spots.[7] Our first case showed reticulate pigment network in honeycomb pattern against pinkish to brownish background with focal hyper and hypopigmentation, while second case showed reticulate pigment with focal hypopigmentation and some white dots.

Biopsy is not diagnostic but shows basal melanosis and hypomelanosis in hyperpigmented and hypopigmented lesions, respectively.[6]

Dermatoscopically, reticulate pigment network in honeycomb pattern against pinkish to brownish background corresponds with perivascular infiltrate and pigmentary incontinence in dermis with the presence of rete ridges. Focal hyperpigmentation and hypopigmentation corresponds to increased and normal melanin in basal layer and were constructed of connected and unconnected pigmented spots respectively[7] while monotonous pigmentation may reflect the hyperpigmentation of basal keratinocytes without the formation of rete ridges.[7]

Other pigmentary disorders need to be differentiated from RAD [Table 1]. No treatment is effective. Fractional CO2 laser and miniature punch grafting combined with a 308 nm excimer laser light have been tried.[12] Split-skin grafting can benefit the patients but is not normally carried out. Camouflage is the most reasonable approach if acceptable.[2]{Table 1}

 Conclusion



Cases are reported due to their rarity and lack of familial involvement. Dermatoscopy showed reticulate pigment network in honeycomb pattern against pinkish to brownish background with focal hyper and hypopigmentation.

 Declaration of patient consent



The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.[13]

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