ENFERMEDAD DE DOWLING-DEGOS PDF

Dowling-Degos disease is characterized by a lacy or net-like reticulate pattern of abnormally dark skin coloring hyperpigmentation , particularly in the body's folds and creases. These skin changes typically first appear in the armpits and groin area and can later spread to other skin folds such as the crook of the elbow and back of the knee. Less commonly, pigmentation changes can also occur on the wrist, back of the hand, face, scalp, scrotum in males , and vulva in females. Individuals with Dowling-Degos disease may also have dark lesions on the face and back that resemble blackheads, red bumps around the mouth that resemble acne, or depressed or pitted scars on the face similar to acne scars but with no history of acne.

Author:Mazugrel Araktilar
Country:Uganda
Language:English (Spanish)
Genre:Personal Growth
Published (Last):10 August 2004
Pages:86
PDF File Size:18.84 Mb
ePub File Size:18.47 Mb
ISBN:316-3-35713-749-5
Downloads:79932
Price:Free* [*Free Regsitration Required]
Uploader:Sagul



In the year has been indexed in the Medlinedatabase, and has become a vehicle for expressing the most current Spanish medicine and modern. All articles are subjected to a rigorous process of revision in pairs, and careful editing for literary and scientific style. CiteScore measures average citations received per document published. Read more. SRJ is a prestige metric based on the idea that not all citations are the same. SJR uses a similar algorithm as the Google page rank; it provides a quantitative and qualitative measure of the journal's impact.

SNIP measures contextual citation impact by wighting citations based on the total number of citations in a subject field. Dowling-Degos disease DDD or reticular pigmented anomaly of the flexures is a rare autosomal dominant genodermatosis with variable penetrance. Physical examination revealed brownish macules of mm diameter, with an irregular morphology, homogeneous pigmentation, and a reticular pattern, situated predominantly in the posterior cervical region Fig.

In addition, she presented active HS lesions in both axillas Fig. The patient stated that her mother, 2 maternal uncles, and 1 of her sisters had similar lesions. Small brownish macules with a tendency to coalesce in a reticular pattern. A, In the posterior cervical region. B, In the intergluteal fold. Axillary nodules with a seropurulent exudate and scarred appearance, compatible with hidradenitis suppurativa.

The dermatopathology study of a perianal skin lesion revealed basal hyperpigmentation, thin elongated digiform rete ridges, and suprapapillary thinning, as well as dermal melanophages and a mild perivascular lymphohistiocytic infiltrate Fig. We offered topical therapy as the first option, but this was rejected by the patient. The disease has shown no significant change after more than 5 years of follow-up. Since then a number of cases of DDD have been reported, and there have been further revelations about its etiology, pathogenesis, and treatment.

DDD usually develops after puberty and tends to have a progressive course. These macules are situated in the skin flexures submammary, axillas, groin , cervical region, trunk, and anterior surface of the thighs and upper arms. The presence of pinpoint papules with keratin plugs simulating comedones is also common in the palmar, axillary, cervical, perioral, and gluteal regions. Furthermore, depressed perioral scars can develop, 2,3 as was observed in our case.

Dermatologic histopathology is characteristic, showing interconnected hyperpigmented epidermal proliferations projecting in a filiform pattern into the dermis. Basal-layer melanocytes are present in normal numbers.

Epidermal hyperplasia may be observed in the hair follicles, extending into the dermis, with the formation of plugs and follicular dilation. The other histologic feature is a mild perivascular lymphohistiocytic infiltrate. The key feature that differentiates DDD from other disorders of keratinization is the combination of hyperpigmented proliferations derived both from the epidermis and from the follicular wall.

The etiology and pathogenesis of DDD involve mutations that have been shown to produce haploinsufficiency of the keratin-5 gene KRT5 on chromosome 12q.

Keratin-5 dysfunction leads to alterations in organelle transport and in epidermal differentiation. In addition, a study in identified a mutation in chromosome 20 in a family with generalized DDD. DDD has been associated with certain skin diseases, in particular multiple keratoacanthomas, 5 epidermoid cysts, 5 and squamous cell carcinoma. One etiologic and pathogenic factor that has been observed is a defect of epithelial proliferation in the external sheath, leading to follicular occlusion, described as a possible common origin for all these disorders.

DDD must be differentiated from other genodermatoses with reticular pigmentation 2,3 : Galli-Galli disease, 10 reticulate acropigmentation of Kitamura, Haber syndrome, and reticulate acropigmentation of Dohi. It has recently been agreed that all these reticular pigmentary diseases are variants of a single entity. There is no completely effective treatment for DDD. Topical hydroquinone, retinoids, and corticosteroids, oral isotretinoin, and some lasers have been used with variable results.

Topical adapalene and tazarotene have been the most successful of the treatments used. This derives from their anti-inflammatory properties as well as their modulating action on gene expression and on keratinocyte differentiation and proliferation. Several types of laser, in particular carbon dioxide and erbium-doped yttrium aluminium garnet Er:YAG lasers, have been shown to be useful in reducing or eliminating the hyperpigmentation. In conclusion, we have presented a case of DDD associated with HS, a previously reported but rare association.

The lesions are progressive and recurrent. Topical and oral retinoids are a safe and accessible treatment option, though not particularly effective. It is important to maintain long-term follow-up in these patients due to the possible association with skin tumors. Actas Dermosifiliogr. ISSN: Open Access Option. Previous article Next article. Issue 4. Pages May Case and Research Letter. Download PDF. Corresponding author. This item has received.

Article information. Show more Show less. Figure 1. Figure 2. Figure 3. Ruiz, M. Actas Dermosifiliogr, 85 , pp. Peteiro, J. Actas Dermosifiliogr, 94 , pp. Betz, L. Planko, S. Eigelshoven, S. Hanneken, S. Pasternack, H. Bussow, et al. Loss-of-function mutations in the keratin 5 gene lead to Dowling-Degos disease.

Am J Hum Genet, 78 , pp. Li, R. Cheng, Z. Am J Hum Genet, 92 , pp. Loo, E. Rytina, P. Hidradenitis suppurativa, Dowling-Degos and multiple epidermal cysts: A new follicular occlusion triad.

Clin Exp Dermatol, 29 , pp. Li, M. Hunt, C. Hidradenitis suppurativa, Dowling Degos disease and perianal squamous cell carcinoma. Australas J Dermatol, 38 , pp. Balus, M. Fazio, A. Amantea, G. Ann Dermatol Venereol, , pp. Bedlow, P. Dowling-Degos disease associated with hidradenitis suppurativa. Clin Exp Dermatol, 21 , pp. Dessinioti, A. Katsambas, C. Hidradenitis suppurativa acne inversa as a systemic disease.

Clin Dermatol, 32 , pp. Actas Dermosifiliogr, , pp. Subscribe to our newsletter. Print Send to a friend Export reference Mendeley Statistics. Recommended articles. Reticulate Hyperpigmentation of the Multimodal Treatment of Calciphylaxis With Sodium

A CENTURY OF STOP-MOTION ANIMATION FROM MELIES TO AARDMAN PDF

Dermatology Online Journal

Her vulvar lesions were treated with Er:YAG laser ablation. However, depending on the definition used, even earlier reports may exist [ 1 ]. These same authors, in , proposed the eponymous name of Dowling-Degos disease for the disorder [ 2 ]. Fewer than 50 cases had been reported by [ 3 ], but since then there have been approximately a dozen additional published reports. A year-old light-skinned woman presented with dark brown vulvar macules that had first been noted at age The lesions were asymptomatic.

AT4008-51E MANUAL PDF

2014, Number 6

In the year has been indexed in the Medlinedatabase, and has become a vehicle for expressing the most current Spanish medicine and modern. All articles are subjected to a rigorous process of revision in pairs, and careful editing for literary and scientific style. CiteScore measures average citations received per document published. Read more. SRJ is a prestige metric based on the idea that not all citations are the same. SJR uses a similar algorithm as the Google page rank; it provides a quantitative and qualitative measure of the journal's impact.

CAKE BLOOD NATION #1 BY DEREKICA SNAKE PDF

Dowling-Degos Disease Associated With Hidradenitis Suppurativa : A Case Report

Skip to search form Skip to main content You are currently offline. Some features of the site may not work correctly. Dowling-Degos disease DDD or reticular pigmented anomaly of the flexures is a rare autosomal dominant genodermatosis with variable penetrance. It mainly affects young women and is characterized by acquired reticular hyperpigmentation of the large skin folds.

27128 EEPROM PDF

Dowling-Degos disease

.

Related Articles