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CONCISE COMMUNICATION
Year : 2021  |  Volume : 12  |  Issue : 3  |  Page : 459-461  

Follicular dowling degos disease with lichen planus in two siblings: A rare association


Department of Dermatology, SCL Hospital, N.H.L. Medical College, Ahmedabad, Gujarat, India

Date of Submission30-Apr-2020
Date of Decision11-Jun-2020
Date of Acceptance22-Aug-2020
Date of Web Publication12-May-2021

Correspondence Address:
Ashish Jagati
OPD Room No. 16, Department of Dermatology, Smt NHL Medical College and SCL General Hospital Saraspur, Ahmedabad - 380 018, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/idoj.IDOJ_309_20

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How to cite this article:
Agarwal P, Jagati A, Neazee S, Patel SV. Follicular dowling degos disease with lichen planus in two siblings: A rare association. Indian Dermatol Online J 2021;12:459-61

How to cite this URL:
Agarwal P, Jagati A, Neazee S, Patel SV. Follicular dowling degos disease with lichen planus in two siblings: A rare association. Indian Dermatol Online J [serial online] 2021 [cited 2021 Jun 22];12:459-61. Available from: https://www.idoj.in/text.asp?2021/12/3/459/315868



Sir,

Dowling-Degos disease (DDD) is a rare autosomal dominant genodermatosis, characterized by multiple, small, asymptomatic, bilaterally symmetrical, progressive, pigmented macules, pitted scar or comedo-like lesions; over the face, neck, arms, trunk, and intertriginous areas. It has also been referred to as “Pigmented reticulate anomaly of flexures”.[1] Its association with perioral pitted scars, epidermoid/trichilemmal cysts, chloracne-like lesions, hidradenitis suppurativa, seborrheic keratoses, palmar pigmentation, and rosacea-like lesions (Haber's syndrome) have been reported in the literature.[2] We hereby present a case of Familial follicular Dowling Degos with lichen planus in two brothers.

A 28-year-old man visited our outpatient clinic with chief complaints of intensely pruritic skin lesions over the lower trunk for 15 days. On cutaneous examination, multiple discrete, polygonal, 1–3 mm diameter papules with violaceous hue over back and lower abdomen were noted. Clinical diagnosis of lichen planus was made [Figure 1]. On further examination multiple asymptomatic, comedo-like lesions and atrophic pitted scars over face, upper chest, and back were noted. History suggested that these asymptomatic lesions were present since childhood. He was accompanied by his 23-year old younger brother who had lesions suggestive of LP over trunk for 10 days and comedo-like and pitted scar like lesions over face, trunk, and flexures since childhood [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d. Dermoscopy of the comedo-like lesion from face showed thick hyperkeratotic, hyperpigmented follicular plug [Figure 3]a and [Figure 3]b. Biopsy from two representing lesions, papular lesion, from trunk and pitted scar-like lesion from face of both brothers. Biopsy from face showed dilated hair follicles with keratinous plug in the infundibulum, elongated rete ridges, focal increase in melanocyte, and horn cysts like changes in the epidermis with normal interfollicular epithelium. Histological changes were suggestive of follicular Dowling degos [Figure 4]a,[Figure 4]b,[Figure 4]c,[Figure 4]d. Lesion from lower back showed irregular epidermal hyperplasia with lymphocytic infiltrate at dermo-epidermal junction suggestive of lichen planus [Figure 5]a,[Figure 5]b,[Figure 5]c. Based on clinical histological and dermatoscopic finding final diagnosis of follicular Dowling Degos associated with lichen planus was made.
Figure 1: Multiple discrete, polygonal, violaceous papules over back

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Figure 2: Multiple asymptomatic, comedo-like lesions and atrophic pitted scars over. (a and b) Face (c) Back (d) Upper chest

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Figure 3: Thick hyperkeratotic, hyperpigmented follicular plug on dermoscopy (a) polarized (b) non-polarized

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Figure 4: Elongated rete ridges, focal increase in melanocyte and horn cysts like changes in the epidermis dilated hair follicles with keratinous plug in the infundibulum on histology (H and E; a 40x, b 100x, c and d 400x)

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Figure 5: Irregular epidermal hyperplasia with lymphocytic infiltrate at dermoepidermal junction (H and E; a 40x, b 100x, c 400x)

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DDD is a rare genodermatosis transmitted by autosomal dominant pattern but can also occur sporadically. It was first described initially by Dowling and Freudenthal in 1938 and Degos and Ossipowski in 1954.[3] It is due to dysfunctional mutations in the keratin 5 gene (KRT5) located on chromosome 12q leading to haploinsufficiency in the keratin gene resulting in defective melanosome uptake and defect in hair follicle structure and sebaceous glands.[4]

The affected patients are usually females in their thirties or forties having acquired progressive hyperpigmentation predominantly in flexures (axillae, groins, intergluteal and inframammary folds, anti-cubital fossa, neck), whereas trunk, arms, face, scalp, and genitalia are also involved in some cases. Isolated involvement of the genitalia has also been reported.[3] Other notable features include pitted follicular, perioral, and facial scars in patients with no previous history of acne and hyperkeratotic comedo-like follicular papules in the neck and/or back (dark dot follicles). It is usually asymptomatic but pruritus of the affected areas can be present. Histopathology of the lesion demonstrates dusky, dappled disfigurements, and dark dot depressions and disclosing digitate down growth delving dermally giving an antler like appearance.[5]

Variants of DDD are generalized as galli-galli disease-(acantholytic cell Variant), follicular DDD, haber's syndrome (pigmented keratotic papules on axilla, neck, and torso with pitted scars on face and persistent facial erythema), pigmentation reticularis Faciei et -Colli.[2] There are several reports of an increased prevalence of epidermal cysts, hidradenitis suppurativa, keratoacanthomas, and perianal squamous cell carcinoma among those diagnosed with DDD.[6] DDD have been reported in several rare association in past like Hidradenitis suppuritiva, Dariers disease, Reticulate acropigmentation of kitamura, Malignant melanoma, and Psoriasis.[7],[8],[9],[10],[11] To best of our knowledge simultaneous overlap of follicular Dowling Degos and lichen planus has not been reported.

To conclude, an uncommon entity like Dowling Degos, though limited to skin and relatively harmless, should be diagnosed promptly as it has multiple variants and have increased risk of other concerning cutaneous diseases.

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.
Kumar AS, Pandhi RK, Jacob M, Singh MK. Dowling-Degos disease. Indian J Dermatol Venereol Leprol 1986;52:48-51.  Back to cited text no. 1
    
2.
Mahajan SH, Mahajan SA, Khopkar US, Kharkar VD. Follicular dowling–Degos disease: A rare pigmentary dermatosis. Indian Dermatol Online J 2017;8:487-9.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Jones EW, Grice K. Reticulate pigmented anomaly of the flexures: Dowling Degos disease, a new genodermatosis. Arch Dermatol 1978;114:1150-7.  Back to cited text no. 3
    
4.
Tambe S, Patil P, Saple D. Successful management of dowling-degos disease with combination of Q-switched Nd:YAG and fractional carbon dioxide laser. J Cutan Aesthet Surg 2017;10:60-2.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Rathoriya SG, Soni SS, Asati D. Dowling–Degos disease with reticulate acropigmentation of Kitamura: Extended spectrum of a single entity. Indian Dermatol Online J 2016;7:32-5.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Singh S, Khandpur S, Verma P, Singh M. Follicular Dowling Degos disease: A rare variant of an evolving dermatosis. Indian J Dermatol Venereol Leprol 2013;79:802-4.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
McGrath JA. Concurrent hidradenitis suppurativa and Dowling–Degos disease taken down a 'Notch'. Br J Dermatol 2018;178:328.  Back to cited text no. 7
    
8.
Strausburg M, Linos K, Staser K, Mousdicas N. Dowling–Degos disease co-presenting with Darier disease. Clin Exp Dermatol 2016;41:410-2.  Back to cited text no. 8
    
9.
Shen Z, Chen L, Ye Q, Hao F, dos Santos VM. Coexistent Dowling-Degos disease and reticulate acropigmentation of kitamura with progressive seborrheic keratosis. Cutis 2011;87:73-5.  Back to cited text no. 9
    
10.
Gupta V, Sahni K, Khute P, Sharma VK, Ali MF. Dowling-Degos disease and malignant melanoma: Association or mere coincidence? Indian J Dermatol Venereol Leprol 2015;81:627-8.  Back to cited text no. 10
[PUBMED]  [Full text]  
11.
Topaloglu FD, Yontem O, Turkoglu Z, Agirgol S, Kaya H, Ozkok TA. A case of co-occurence of Dowling-Degos disease and psoriasis: Association or a rarely-seen coincidence? G Ital Dermatol Venereol 2018;153:884-5.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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