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| ONLINE ONLY ARTICLES - CASE REPORT |
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| Year : 2022 | Volume
: 6
| Issue : 2 | Page : 153 |
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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
| Date of Submission | 12-Feb-2021 |
| Date of Decision | 20-Apr-2021 |
| Date of Acceptance | 12-Jul-2021 |
| Date of Web Publication | 26-Aug-2022 |
Correspondence Address:
Pragya Ashok Nair
Department of Dermatology, Pramukhswami Medical College, Shree Krishna Hospital, Karamsad - 388 325, Gujarat
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/cdr.cdr_11_21
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.
Keywords: Acropigmentation of Dohi, dermatoscopy, dyschromatosis symmetrica hereditarian, reticulate pigmentation
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 |
| 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: Multiple well-defined hyperpigmented to hypopigmented macules in a reticular pattern over chest and neck with scaling over face
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 | Figure 2: Multiple, well-defined, hyperpigmented to hypopigmented macules in a reticular pattern over (a) trunk (b) back
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 | Figure 3: Multiple hyper and hypopigmented macules in a reticular pattern over bilateral (a) palms (b) soles
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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: Dermoscopy showed reticulate pigment network in honeycomb pattern (green arrow) against pinkish to brownish background (red arrow) with focal hyper and hypopigmentation (blue arrow)
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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: (a) Hyperpigmented lesion showed loss of rete ridges and increased melanin content in basal layer and spinous layer with vacuolar degeneration and pigmentary incontinence in upper dermis (H and E stain, ×40). (b) Hypopigmented lesion showed loss of rete ridges with normal melanin content in basal layer and vacuolar degeneration (H and E stain, ×40)
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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: Multiple hyperpigmented and hypopigmented, macules over (a)trunk, (b)back and (c) buttocks and back of upper thighs
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 | Figure 7: Multiple hyperpigmented and hypopigmented, macules over (a) extensors of arms (b) palms
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 | Figure 8: Multiple, hyperpigmented, and hypopigmented macules over (a) back of legs (b) dorsum of foot (c) bilateral soles
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 | Figure 9: Dermoscopy showed reticulate pigment (red arrow) with focal hypopigmentation (blue arrow) and some white dots (green arrow)
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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]
| 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]
| References | | |
| 1. | Barzegari M, Kiavash K, Dyschromatosis symmetrica hereditaria (reticulate acropigmentation of dohi): First report from Iran. Indian J Dermatol 2009:54:S11-3.  |
| 2. | Sharma R, Chandra M. Reticulate acropigmentation of dohi – A report of two unrelated families. Indian J Dermatol Venereol Leprol 2000;66:139-40. [ PUBMED] [Full text] |
| 3. | Hayashi M, Suzuki T. Dyschromatosis symmetrica hereditaria. J Dermatol 2013;40:336-43. |
| 4. | Miyamura Y, Suzuki T, Kono M, Inagaki K, Ito S, Suzuki N, et al. Mutations of the RNA-specific adenosine deaminase gene (DSRAD) are involved in dyschromatosis symmetrica hereditaria. Am J Hum Genet 2003;73:693-9. |
| 5. | Vachiramon V, Thadanipon K, Rattanakaemakorn P. Adult-onset dyschromatoses. Clin Exp Dermatol 2012;37:97-103. |
| 6. | Kantaputra PN, Chinadet W, Ohazama A, Kono M. Dyschromatosis symmetrica hereditaria with long hair on the forearms, hypo/hyperpigmented hair, and dental anomalies: Report of a novel ADAR1 mutation. Am J Med Genet A 2012;158A: 2258-65. |
| 7. | Oiso N, Murata I, Hayashi M, Amatsu A, Yoshida M, Suzuki T. et al. Dermoscopic features in a case of dyschromatosissymmetricahereditaria. J Dermatol 2011;38:91-3. |
| 8. | Manchand S, Arora RR, Lingaraj M. Sporadic dyschromatosis universalis hereditaria: A rare case report. Indian J Paediatr Dermatol 2017;18:43-5. |
| 9. | Koguchi H, Ujiie H, Aoyagi S, Osawa R, Shimizu H. Characteristic findings of handprint and dermoscopy in reticulate acropigmentation of Kitamura. Clin Exp Dermatol 2014;39:85-7. |
| 10. | Geissler S, Dyall-Smith D, Coras B, Guther S, Peters B, Stolz W. Unique brown star shape on dermatoscopy of generalized Dowling-Degos disease. Australas J Dermatol 2011;52:151-3. |
| 11. | Chandrashekhar L. Dermoscopy: A tool to assess stability in vitiligo. In: Khopkar U, editor. Dermoscopy and Trichoscopyin Diseases of the Brown Skin: Atlas and Short Text. New Delhi, India: Jaypee Brothers Medical Publishers; 2012. p. 112-3. |
| 12. | Xu XG, Lv Y, Zhai JL, Li YH, Gao XH, Chen HD. Two novel mutations of the ADAR1 gene in Chinese patients with dyschromatosis symmetrica hereditaria successfully treated with fractional CO2 laser. J Eur Acad Dermatol Venereol 2016;30:1035-8. |
| 13. | Goel R, Bodh SA, Sardana K, Goel A. Dermatopathia pigmentosa reticularis with Salzmann's nodular degeneration of cornea: A rare association. Nepal J Ophthalmol 2015:7;79-81. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]
[Table 1]
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