Acta Dermato-Venereologica 99-10CompleteContent | Page 24
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SHORT COMMUNICATION
Dowling-Degos Disease and Hidradenitis Suppurativa. Epidemiological and Clinical Study of 15
Patients and Review of the Literature
Eugenia AGUT-BUSQUET 1 , Iris GONZÁLEZ-VILLANUEVA 2 , Jorge ROMANÍ DE GABRIEL 1 , Jose Carlos PASCUAL 2 , Miquel
RIBERA PIBERNAT 1 and Jesús LUELMO 1
Department of Dermatology, University Hospital Parc Tauli of Sabadell, Autonomous University of Barcelona, Barcelona, Spain, and
Department of Dermatology, Alicante University General Hospital, Alicante Institute for Health and Biomedical Research (ISABIAL-FISABIO
Foundation), Alicante, Spain. E-mail: [email protected]
1
2
Accepted May 22, 2019; E-published May 23, 2019
Dowling–Degos disease (DDD), or reticular pigmented
anomaly of the flexural surfaces, is a rare autosomal do-
minant genetic pigmentation disorder (1). DDD occurs
more frequently in women and lesion onset is usually after
puberty. DDD clinically presents as an acquired dot-like
or reticular hyperpigmentation of the skinfolds, depressed
pitted scars in the periorbital region and pinpoint papules
with keratin plugs in the palmar, axillary, cervical, perioral
and gluteal regions, with a characteristic histopatho-
logy (2). The differential diagnosis includes acanthosis
nigricans, Naegeli-Franceschetti-Jadassohn syndrome,
confluent and reticulated papillomatosis, Galli-Galli
disease, Haber syndrome, reticulate acropigmentation of
Kitamura and Dohi, dyschromatosis symmetrica universa-
lis hereditaria and other dyskeratotic syndromes. In 2006
it was established that mutations in KRT5, POFUT1 and
POGLUT1 are implicated in DDD (3, 4).
Hidradenitis suppurativa (HS) is a chronic recurrent
inflammatory disease with a multifactorial aetiopathoge-
nesis. Abnormalities in the innate immune system promote
a maintained high-grade inflammatory state in response
to keratin, corneocytes, bacteria and sebaceous material
that is extruded when a pilosebaceous unit is disrupted (5).
DDD has been associated with conditions such as in-
fundibular cysts, keratoacanthomas (6) and squamous cell
carcinoma (7), while the co-occurrence of DDD with HS
has only been reported in 6 unique clinical cases (2, 6–8).
We report epidemiological and clinical characteristics for
a cohort of 15 patients with DDD, concomitantly affected
by HS, who attended the Dermatology Services of Alicante
Hospital and Sabadell Hospital between 2015 and 2017.
bolic syndrome, hypertension, diabetes mellitus, dyslipidaemia
and pilonidal sinus were reported in a minority of patients (27%,
20%, 20%, 27% and 20%, respectively). Smokers accounted for
80% of the patients and 53% of the patients were overweight, with
a mean BMI of 27.93.
Clinical exploration revealed the patients to have clinical fin-
dings suggestive of DDD (Fig. 1). Hair and nails were normal. To
confirm the diagnosis of DDD, a punch biopsy was performed in
all the patients in either the axilla or the major abdominal flexure
(Fig. S1 1 ). Active acne, nodules, abscesses and sinus tracts were
observed in 46.66%, 100%, 80% and 33.33% of the patients, re-
spectively. The involved body parts were mainly axillae (100%),
groin (93%), nape and back (66.66% each) and gluteal and pubic
areas (53.33% each). Infundibular cysts (mean count 6.67) were
observed in 14 patients. The most common Hurley stage was II
(47%). Mean IHS4 was 9.7.
Regarding the management, during the 2 years of follow-up,
over half the patients (53%) received more than 2 types of treat
ment: 46% received rifampicin 300 mg b.i.d plus clindamycin
300 mg b.i.d for 12 weeks, followed by a maintenance therapy
consisting of topical application of either a 1% clindamycin so-
lution or a 15% resorcinol solution 3 times a week; while 40%
received either sulfone 50 mg b.i.d or acitretin 25 mg q.d; and
13.33% received adalimumab 40 mg/week.
https://www.medicaljournals.se/acta/content/abstract/10.2340/00015555-3225
1
MATERIAL AND METHODS AND RESULTS
Approval for the study was obtained. Data gathered from the
medical records included epidemiological characteristics, clinical
information (including Hurley Stage and International Hidradenitis
Suppurativa Severity Score System (IHS4) (9)) and treatment
information. Results for categorical variables were expressed
as absolute frequencies and percentages. Descriptive statistics
for continuous variables were expressed as means ± standard
deviations (SD). Statistical tests were carried out using SPSS
version 22.0.
The retrospectively reviewed cohort of 15 patients included 8
men (53%) and 7 women (47%). Mean ± SD age was 43.27 ± 12.36
years. All patients reported a family history of HS and 60% re-
ported a family history of DDD. Mean ± SD age of onset for HS
was 15.60 ± 3.69 years. Mean age of evolution was 27.67 ± 14.61
years. A history of acne was reported in 7 patients (47%). Meta-
Fig. 1. Clinical images. a) Patient #13. Symmetrically distributed
hyperpigmented macules with a reticular disposition affecting the perineal
area. Note the 2 nodules and the non-draining fistula. b) Patient #13.
Comedon-like infundibular cysts and follicular plugging. c) Patient #14.
Typical DDD hyperpigmented macules in major skinfolds. Note the inflamed
abscess in the perineal area. d) Patient #14. Note the non-inflammatory
nodules in the right axillae, hyperpigmentation and multiple double-
comedon openings.
This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta
Journal Compilation © 2019 Acta Dermato-Venereologica.
doi: 10.2340/00015555-3225
Acta Derm Venereol 2019; 99: 917–918