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Flexural reticulate hyperpigmentation

MedGen UID:
1054759
•Concept ID:
CN378088
•
Finding
Synonyms: Reticular pigmented anomaly of the flexures; Reticulate hyperpigmentation of the flexures
 
HPO: HP:6000745

Definition

Hyperpigmented macules with a reticulate (net-like) pattern involving the neck, axillae, groins, and other flexures like the inframammary folds in females. Histopathology shows orthokeratosis or hyperkeratosis, thinning of the suprapapillary epithelium, elongation of the rete ridges, hyperpigmentation of the basal layer, and follicular cysts. A perivascular lymphohistiocytic infiltrate can be observed in the papillary dermis. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFlexural reticulate hyperpigmentation

Conditions with this feature

ANE syndrome
MedGen UID:
394313
•Concept ID:
C2677535
•
Disease or Syndrome
Alopecia, neurologic defects, and endocrinopathy syndrome (ANES) is an autosomal recessive disorder characterized by alopecia with skin involvement including multiple facial nevi and flexural hyperpigmentation; moderately to severely impaired intellectual development; progressive motor decline; and endocrine deficiency (summary by Spiegel et al., 2010).
See: Condition Record
ANE syndrome

Recent clinical studies

Etiology

Atypical Disseminated Variant of Galli-Galli Disease: A Review of the Literature.
Yang A, Cheung K, Kossard S, Murrell DF
Am J Dermatopathol 2020 Jul;42(7):484-490. doi: 10.1097/DAD.0000000000001467. PMID: 31449063
Dowling-Degos disease (reticulate pigmented anomaly of the flexures): a clinical and histopathologic study of 6 cases.
Kim YC, Davis MD, Schanbacher CF, Su WP
J Am Acad Dermatol 1999 Mar;40(3):462-7. doi: 10.1016/s0190-9622(99)70498-6. PMID: 10071319

Diagnosis

Atypical Disseminated Variant of Galli-Galli Disease: A Review of the Literature.
Yang A, Cheung K, Kossard S, Murrell DF
Am J Dermatopathol 2020 Jul;42(7):484-490. doi: 10.1097/DAD.0000000000001467. PMID: 31449063
Phenotypic expansion of POFUT1 loss of function mutations in a disorder featuring segmental dyspigmentation with eczematous and folliculo-centric lesions.
Atzmony L, Zaki TD, Antaya RJ, Choate KA
Am J Med Genet A 2019 Dec;179(12):2469-2473. Epub 2019 Sep 30 doi: 10.1002/ajmg.a.61362. PMID: 31566882Free PMC Article
Flexural Hyperpigmentation With Reticulation in an Adolescent Girl.
Bishnoi A, Banerjee N, Parsad D
JAMA Dermatol 2019 Mar 1;155(3):377-378. doi: 10.1001/jamadermatol.2018.5033. PMID: 30649131
Treatment of Dowling-Degos disease with fractional Er:YAG laser.
Yun JH, Kim JH, Choi JS, Roh JY, Lee JR
J Cosmet Laser Ther 2013 Dec;15(6):336-9. Epub 2013 Mar 6 doi: 10.3109/14764172.2013.764437. PMID: 23464495
Dowling-Degos disease.
Georgescu EF, Stănescu L, Popescu CF, Comănescu M, Georgescu I
Rom J Morphol Embryol 2010;51(1):181-5. PMID: 20191141

Therapy

Flexural Hyperpigmentation With Reticulation in an Adolescent Girl.
Bishnoi A, Banerjee N, Parsad D
JAMA Dermatol 2019 Mar 1;155(3):377-378. doi: 10.1001/jamadermatol.2018.5033. PMID: 30649131
Successful treatment of Dowling-Degos disease using intense pulsed light.
Gupta A, Huilgol SC
Australas J Dermatol 2015 Aug;56(3):e63-5. Epub 2014 Dec 13 doi: 10.1111/ajd.12232. PMID: 25495801
Treatment of Dowling-Degos disease with fractional Er:YAG laser.
Yun JH, Kim JH, Choi JS, Roh JY, Lee JR
J Cosmet Laser Ther 2013 Dec;15(6):336-9. Epub 2013 Mar 6 doi: 10.3109/14764172.2013.764437. PMID: 23464495

Prognosis

Flexural Hyperpigmentation With Reticulation in an Adolescent Girl.
Bishnoi A, Banerjee N, Parsad D
JAMA Dermatol 2019 Mar 1;155(3):377-378. doi: 10.1001/jamadermatol.2018.5033. PMID: 30649131

Clinical prediction guides

Galli-Galli Disease Presenting as a Lentigo-like Eruption: A Further Clinical Feature in the Wide Spectrum of Reticulate Pigment Disorders.
Paolino G, Donati M, Didona D, Panetta C, Donati P
Acta Dermatovenerol Croat 2017 Dec;25(4):300-302. PMID: 30064605
Dowling-Degos disease (reticulate pigmented anomaly of the flexures): a clinical and histopathologic study of 6 cases.
Kim YC, Davis MD, Schanbacher CF, Su WP
J Am Acad Dermatol 1999 Mar;40(3):462-7. doi: 10.1016/s0190-9622(99)70498-6. PMID: 10071319
Reticular pigmented genodermatosis with milia--a special form of Naegeli-Franceschetti-Jadassohn syndrome or a new entity?
Tzermias C, Zioga A, Hatzis I
Clin Exp Dermatol 1995 Jul;20(4):331-5. doi: 10.1111/j.1365-2230.1995.tb01336.x. PMID: 8548993
Spectrum of reticulate flexural and acral pigmentary disorders in northern India.
Dhar S, Kanwar AJ, Jebraili R, Dawn G, Das A
J Dermatol 1994 Aug;21(8):598-603. doi: 10.1111/j.1346-8138.1994.tb01801.x. PMID: 7962960

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