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Year : 2018  |  Volume : 19  |  Issue : 1  |  Page : 77-79

Dermatopathia pigmentosa reticularis

Department of Skin and VD, Dr. S.N. Medical College, Jodhpur, Rajasthan, India

Date of Web Publication28-Dec-2017

Correspondence Address:
Durgesh Sonare
Room No. 94, PG Hostel, MDM Hospital Campus, Jodhpur, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_79_16

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Dermatopathia pigmentosa reticularis (DPR) is a rare disorder with characteristic triad of generalized reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy. A 12-year-old Indian boy presented with classical features of the triad along with adermatoglyphia, hyperhidrosis, punctate hyperkeratosis of palm, and sole along with keratosis pilaris. Histopathology suggested a diagnosis of DPR with a differential of Naegeli–Franceschetti–Jadassohn syndrome and dyskeratosis congenita.

Keywords: Dermatopathia pigmentosa reticularis, dyskeratosis congenita, histopathology, keratosis pilaris, Naegeli–Franceschetti–Jadassohn syndrome

How to cite this article:
Vats G, Kataria R, Sonare D, Jain V. Dermatopathia pigmentosa reticularis. Indian J Paediatr Dermatol 2018;19:77-9

How to cite this URL:
Vats G, Kataria R, Sonare D, Jain V. Dermatopathia pigmentosa reticularis. Indian J Paediatr Dermatol [serial online] 2018 [cited 2020 Jul 14];19:77-9. Available from: http://www.ijpd.in/text.asp?2018/19/1/77/206085

What was known?

Dermatopathia pigmentosa reticularis is a very rare disorder with a characteristic triad and some less specific skin manifestations.

  Introduction Top

Dermatopathia pigmentosa reticularis (DPR) is a rare autosomal dominant disorder consisting of a characteristic triad.[1] In addition, other variable features include adermatoglyphia, hypohidrosis or hyperhidrosis, and palmoplantar hyperkeratosis.[2] Approximately 13 cases of DPR have been reported worldwide so far. A paucity of reports in literature, unusual association, and its depiction simulating other dermatoses, especially Naegeli–Franceschetti–Jadassohn syndrome (NFJS) and dyskeratosis congenita (DKC) compelled us to report this.

  Case Report Top

A 10-year-old male child presented to us with complaints of diffuse hyperpigmentation all over body which began over the neck at the age of 6 months progressively involving the whole body within a year. The palms and soles were hyperkeratotic and pigmented. Diffuse hyperpigmentation and keratosis pilaris were present over the trunk [Figure 1]. Lingual, buccal, and genital mucosa also showed pigmentation [Figure 2] with normal teeth. Furthermore, the dermatoglyphics of the hands was poorly developed. On the scalp, there was diffuse nonscarring alopecia with dry lustreless, short hair which on dermoscopy did not reveal any abnormality. There was a history of palmoplanter hyperhydrosis along with atrophy of the toe and thumbnail [Figure 3]. The vision and hearing were normal and the child was well built for age. All routine investigations were normal. His kindred was scrutinized and it was unremarkable. Histology revealed normal to mildly acanthotic epidermis with basal layer degeneration, melanophages in dermis, and absence of skin adnexa. Mild perivascular chronic inflammation was present over the dermis [Figure 4]. We arrived at the diagnosis of DPR. Mutational analysis was not done as the patient had monetary constraints.
Figure 1: Diffuse alopecia and truncal keratosis pilaris

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Figure 2: Diffuse pigmentation on glans and oral cavity with normal teeth

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Figure 3: Nail dystrophy and underdeveloped dermatoglyphics

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Figure 4: Acanthotic epidermis with basal layer degeneration, melanophages in dermis, and absence of skin adnexa and mild perivascular chronic dermal inflammation

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  Discussion Top

DPR is a rare pigmentary dermatosis characterized by a triad of widespread reticulate hyperpigmentation, nonscarring alopecia, and nail changes first described by Hauss and Oberste-Lehn in 1958.[1],[3] Pigmentation begins to appear at birth or during infancy and persists throughout life.[4]

Reticulate pigmentation of early onset can be either generalized or localized.[5] Generalized reticulate brown pigmentation with widespread blistering is seen in Epidermolysis bullosa simplex with mottled pigmentation in contrast to the association with involvement of hair, nail, teeth, and eccrine glands is seen in DPR and NFJS.

NFJS and DPR are rare ectodermal dysplasias inherited in an autosomal dominant fashion.[3] Both manifest with poorly developed dermatoglyphics, reticulate hyperpigmentation of the skin, hypohidrosis, and heat intolerance. Palmoplantar keratoderma, nail dystrophy, and enamel defects are common in NFJS, whereas diffuse alopecia is only seen in DPR. Teeth are always severely affected, leading to early total loss in NFJS. In some NFJS pedigrees, the reticulate pigmentation fades after puberty and may disappear completely in old age. In DPR, the hyperpigmentation persists throughout life, showing no tendency of spontaneous fading. The reticulate network of hyperpigmented macules occurs particularly on the trunk, neck, and proximal areas of the limbs.

DKC characterized by reticulate hyperpigmentation, mucosal leukoplakia, bone marrow dysfunction, cytogenetic instability, and a predisposition to malignancy. These patients also have dental findings, adermatoglyphia, palmoplantar hyperkeratosis, and nail anomalies similar to NFJS and DPR patients.[6]

DPR has close histological differential diagnosis with NFJS and DKC. Both these conditions demonstrate hyperpigmentation of basilar keratinocytes with either a normal or slightly increased number of melanocytes. DKC is characterized by occasional atrophy of epidermis, mild interface dermatitis vacuolization with melanophages in the upper dermis, and telangiectasias of superficial vessels, whereas NFJS is identified by epidermal atrophy, vacoular degeneration, hyperpigmentation of basal layer, and pigmentary incontinence. In dermis, there is perivascular chronic inflammation, increased dermal fibrosis, and presence of dermal melanophages. DPR on the other hand is distinguished by clumps of melanin laden melanophages in papillary dermis in a patchy distribution without overlying epidermal hyperpigmentation.

Dereure [7] noted that NFJS and DPR are two allelic ectodermal dysplasias related to mutations of dominant gene coding for keratin 14. Severe keratin 5 and 14 mutations induce downregulation of junction proteins in keratinocytes, which likely underlies all of these diseases. A number of missense mutations in KRT14 causing a DPR/NFJS phenotype have been reported.[8] There is no specific treatment for DPR, except for symptomatic management of the cutaneous distress such as palmoplantar keratoderma, for which topical retinoids and keratolytics may be useful.[9],[10]

  Conclusion Top

When an adult come with generalized reticulate pigmentation, diffuse alopecia, and nail dystrophy, one should think about the differential diagnosis of DPR, NFJS, and DKC; however, these closely mimicking disease can be differentiated on clinical and histological ground.

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Conflicts of interest

There are no conflicts of interest.

What is new?

The exceeding rarity and uncanny similarity to dermatoses such as Naegeli–Franceschetti–Jadassohn syndrome, dyskeratosis congenita urged us to report this rather pristine condition. Furthermore, there was association with reticulate hyperpigmentation on the glans and keratosis pilaris over the trunk, which had not been elicited previously.

  References Top

Hauss H, Oberste-Lehn H. Dermatopathia pigmentosa reticularis. Dermatol Wochenschr 1958;138:1337.  Back to cited text no. 1
Bu TS, Kim YK, Whang KU. A case of dermatopathia pigmentosa reticularis. J Dermatol 1997;24:266-9.  Back to cited text no. 2
Heimer WL 2nd, Brauner G, James WD. Dermatopathia pigmentosa reticularis: A report of a family demonstrating autosomal dominant inheritance. J Am Acad Dermatol 1992;26:298-301.  Back to cited text no. 3
Schnur RE, Heymann WR. Reticulate hyperpigmentation. Semin Cutan Med Surg 1997;16:72-80.  Back to cited text no. 4
Vachiramon V. Approach to reticulate hyperpigmentation. Clin Exp Dermatol 2011;36:459-66.  Back to cited text no. 5
Robles DT. Dyskeratosis Congenita Clinical Presentation. Available from: http://www.medscape.com. [Last cited on 2014 Jun 06].  Back to cited text no. 6
Dereure O. Naegeli-Franceschetti-Jadassohn syndrome and dermatopathia pigmentosa reticularis. Two allelic ectodermal dysplasias related to mutations of dominant gene coding for keratin 14. Ann Dermatol Venereol 2007;134:595.  Back to cited text no. 7
Lugassy J, Itin P, Ishida-Yamamoto A, Holland K, Huson S, Geiger D, et al. Naegeli-Franceschetti-Jadassohn syndrome and dermatopathia pigmentosa reticularis: Two allelic ectodermal dysplasias caused by dominant mutations in KRT14. Am J Hum Genet 2006;79:724-30.  Back to cited text no. 8
Sardana K, Goel K, Chugh S. Reticulate pigmentary disorders. Indian J Dermatol Venereol Leprol 2013;79:17-29.  Back to cited text no. 9
[PUBMED]  [Full text]  
Shanker V, Gupta M. Dermatopathia pigmentosa reticularis: A rare reticulate pigmentary disorder. Indian Dermatol Online J 2013;4:40-2.  Back to cited text no. 10
[PUBMED]  [Full text]  


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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