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CASE REPORT
Year : 2019  |  Volume : 20  |  Issue : 2  |  Page : 172-173

Sporadic Case of dyschromatosis universalis hereditaria showing moderate response to narrow-band ultraviolet-B


1 Department of Dermatology and Venereology, Government Medical College, Kozhikode, Kerala, India
2 Department of Dermatology, District Hospital Mananthavady, Wayanad, Kerala, India
3 Department of Pathology, Government Medical College, Kozhikode, Kerala, India

Date of Web Publication29-Mar-2019

Correspondence Address:
Dr. Sarita Sasidharanpillai
“Rohini”, Girish Nagar, Nallalam PO, Kozhikode - 673 027
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_33_18

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  Abstract 


Dyschromatosis universalis hereditaria (DUH) is a rare genodermatosis where the affected manifests mottled pigmentation with intermingled hyper- and hypo-pigmented macules. On most occasions, the lesions begin on limbs and then extend to trunk. Most of the cases reported in literature are from Japan. It is rarer in Indians. In this report, we describe a 12-year-old male child with DUH, who showed response to narrow-band ultraviolet-B therapy.

Keywords: Child, dyschromatosis universalis hereditaria, narrow-band ultraviolet-B


How to cite this article:
Sasidharanpillai S, Shyam A, Manakkad SP, Abdul Latheef EN, Rahima S, Paul N. Sporadic Case of dyschromatosis universalis hereditaria showing moderate response to narrow-band ultraviolet-B. Indian J Paediatr Dermatol 2019;20:172-3

How to cite this URL:
Sasidharanpillai S, Shyam A, Manakkad SP, Abdul Latheef EN, Rahima S, Paul N. Sporadic Case of dyschromatosis universalis hereditaria showing moderate response to narrow-band ultraviolet-B. Indian J Paediatr Dermatol [serial online] 2019 [cited 2019 Apr 25];20:172-3. Available from: http://www.ijpd.in/text.asp?2019/20/2/172/255200




  Introduction Top


Described by Ichikawa and Higara, dyschromatosis universalis hereditaria (DUH) remains a rare genodermatosis.[1] Most cases reported are from Japan though there are occasional reports from other parts of the world including India. We report a child with the disease who showed moderate response to narrow-band ultraviolet-B (NBUVB).


  Case Report Top


A 12-year-old male child of nonconsanguineous marriage presented with interspersed depigmented and hyperpigmented macules involving the trunk [Figure 1]a, proximal part of upper arms, buttocks, upper thighs, and dorsal aspect of middle phalanges of fingers with scattered lesions on the face. Photosensitivity was absent. The lesions started appearing on the trunk at the age of 1 year and then progressively increased. His growth and development were normal. There was no history of seizures or reduced sweating. His scholastic performance was average. None of his family members were similarly affected. There was no history of exposure to any chemicals.
Figure 1: (a) Depigmented and hyperpigmented macules on the trunk of a patient with dyschromatosis universalis hereditaria; (b) Moderate response to treatment after 3 months of narrow-band ultraviolet-B treatment

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He showed no xerosis, atrophy, or telangiectasia. Palms and soles were spared. Hair, teeth, and nails were normal. Mucosae were uninvolved except for depigmentation noted on both upper and lower lips.

Laboratory parameters including serology for human immunodeficiency virus infection were negative.

Representative lesions from both the depigmented and hyperpigmented macules were biopsied. Reduced and increased basement membrane pigmentations were noted in the depigmented [Figure 2] and hyperpigmented areas [Figure 3], respectively, suggesting a diagnosis of DUH.[2] The absence of photosensitivity, xerosis, atrophy, and telangiectasia observed in our case was not in favour of xeroderma pigmentosum. The child and parents were counseled regarding the benign disease course and the unsatisfactory response to treatment modalities. He was offered NBUVB thrice a week and showed moderate response after 3 months [Figure 1]b.
Figure 2: (a) Skin biopsy specimen from depigmented lesion showing mild hyperkeratosis with decreased pigmentation of basal layer. Dermis showing mild perivascular lymphocytic infiltrate (H and E, ×200); (b) Special stain for melanin in the same specimen showing faint positivity (Mason Fontana, ×200)

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Figure 3: (a) Skin biopsy specimen from hyperpigmented lesion showing mild hyperkeratosis with increased pigmentation of basal layer. Dermis shows melanin incontinence (H and E, ×200); (b) Special stain for melanin in the same specimen shows intense positivity (Mason Fontana, ×200)

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  Discussion Top


DUH is considered as the generalized form of a disease spectrum including dyschromatosis universalis hereditaria, dyschromatosis symmetrica hereditaria, or acropigmentation of Dohi (confined to extremities) and a segmental form called unilateral dermatomal pigmentary dermatosis, where the affected presents with intermingled hypo- and hyper-pigmented macules.[2]

DUH shows autosomal dominant or recessive pattern of inheritance.[2] Sporadic cases like ours have been reported occasionally.[3],[4] Often, it begins in early childhood and mostly involves trunk and extremities. Face is affected in 50% of cases. Involvement of palms, soles, hair, and nails is recorded occasionally.[2]

Most reports document lesions beginning on extremities and extending to trunk, but some patients like ours had lesions manifesting on the trunk and then extending to limbs. Interestingly, in the two other cases, where lesions started on the trunk and extended to involve limbs also, no family history could be elicited as in our patient.[3],[4] A localized form has been described by Dhar and Malakar.[5]

DUH is considered as a disorder of melanosome synthesis rate or melanocyte activity, rather than a disorder of melanocyte numbers.[6]

The moderate response observed following NBUVB by us and by Yadalla et al. points to a role for phototherapy.[2] We report this case to highlight the need to explore the option of NBUVB in similar instances.

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 initials 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.



 
  References Top

1.
Ichikawa T, Higara Y. About a pigmentary anomaly unprecedented. Jpn J Dermatol 1933;34:360-4.  Back to cited text no. 1
    
2.
Yadalla HK, Pinninti S, Babu AR. Dyschromatosis universalis hereditaria: Infrequent genodermatoses in India. Indian J Hum Genet 2013;19:487-90.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Namitha P, Sacchidanand S. Dyschromias: A Series of five interesting cases from India. Indian J Dermatol 2015;60:636.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Manchanda S, Arora R, Lingaraj MM. Sporadic dyschromatosis universalis hereditaria: A rare case report. Indian J Paediatr Dermatol 2017;18:43-5.  Back to cited text no. 4
  [Full text]  
5.
Dhar S, Malakar S. Localized form of dyschromatosis universalis hereditaria in a 14-year-old girl. Pediatr Dermatol 1999;16:336.  Back to cited text no. 5
    
6.
Nuber UA, Tinschert S, Mundlos S, Hauber I. Dyschromatosis universalis hereditaria: Familial case and ultrastructural skin investigation. Am J Med Genet A 2004;125A: 261-6.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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