Dowling–Degos disease with follicular involvement associated with hidradenitis suppurativa: A manifestation of follicular occlusion phenomenon?
A 30-year-old female patient presented with progressive reticulate pigmentation of the neck, upper chest, back, cubital fossa, and axillae since the age of 15 years. The patient also complained of recurrent multiple tender nodules and plaques associated with sinuses and pus discharge over the axilla...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2020-01-01
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| Series: | Indian Journal of Dermatology |
| Subjects: | |
| Online Access: | http://www.e-ijd.org/article.asp?issn=0019-5154;year=2020;volume=65;issue=4;spage=295;epage=298;aulast=Supekar |
| _version_ | 1828887060522467328 |
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| author | Bhagyashree Babanrao Supekar Kinjal Deepak Rambhia Rajesh Pratap Singh Jayesh Ishwardas Mukhi |
| author_facet | Bhagyashree Babanrao Supekar Kinjal Deepak Rambhia Rajesh Pratap Singh Jayesh Ishwardas Mukhi |
| author_sort | Bhagyashree Babanrao Supekar |
| collection | DOAJ |
| description | A 30-year-old female patient presented with progressive reticulate pigmentation of the neck, upper chest, back, cubital fossa, and axillae since the age of 15 years. The patient also complained of recurrent multiple tender nodules and plaques associated with sinuses and pus discharge over the axillae, groins, and buttocks which healed with scarring for 4 years. There were multiple pitted scars and hyperpigmented macules over the perioral area and cheeks. Scattered comedo-like lesions were present over the upper back, chest, cubital fossae, and buttocks. Histopathology from the pigmented lesion revealed thinning of the suprapapillary epidermis, epidermal hyperplasia with finger-like elongation of the rete ridges, and increased pigmentation of their lower part, suggestive of Dowling–Degos disease (DDD). Biopsy section from comedonal lesion showed dilated infundibulum and antler-like rete ridges suggestive of follicular DDD. We report a case of DDD with follicular involvement and hidradenitis suppurativa (HS) which is a rare association and can be explained on the basis of single underlying defect in follicular epithelial proliferation. DDD-HS has been shown to result from mutations in PSENEN, encoding a critical component of the γ-secretase complex. |
| first_indexed | 2024-12-13T11:57:10Z |
| format | Article |
| id | doaj.art-7669b0ba32444e779563f4b8eb622a9a |
| institution | Directory Open Access Journal |
| issn | 0019-5154 1998-3611 |
| language | English |
| last_indexed | 2024-12-13T11:57:10Z |
| publishDate | 2020-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Dermatology |
| spelling | doaj.art-7669b0ba32444e779563f4b8eb622a9a2022-12-21T23:47:12ZengWolters Kluwer Medknow PublicationsIndian Journal of Dermatology0019-51541998-36112020-01-0165429529810.4103/ijd.IJD_392_18Dowling–Degos disease with follicular involvement associated with hidradenitis suppurativa: A manifestation of follicular occlusion phenomenon?Bhagyashree Babanrao SupekarKinjal Deepak RambhiaRajesh Pratap SinghJayesh Ishwardas MukhiA 30-year-old female patient presented with progressive reticulate pigmentation of the neck, upper chest, back, cubital fossa, and axillae since the age of 15 years. The patient also complained of recurrent multiple tender nodules and plaques associated with sinuses and pus discharge over the axillae, groins, and buttocks which healed with scarring for 4 years. There were multiple pitted scars and hyperpigmented macules over the perioral area and cheeks. Scattered comedo-like lesions were present over the upper back, chest, cubital fossae, and buttocks. Histopathology from the pigmented lesion revealed thinning of the suprapapillary epidermis, epidermal hyperplasia with finger-like elongation of the rete ridges, and increased pigmentation of their lower part, suggestive of Dowling–Degos disease (DDD). Biopsy section from comedonal lesion showed dilated infundibulum and antler-like rete ridges suggestive of follicular DDD. We report a case of DDD with follicular involvement and hidradenitis suppurativa (HS) which is a rare association and can be explained on the basis of single underlying defect in follicular epithelial proliferation. DDD-HS has been shown to result from mutations in PSENEN, encoding a critical component of the γ-secretase complex.http://www.e-ijd.org/article.asp?issn=0019-5154;year=2020;volume=65;issue=4;spage=295;epage=298;aulast=Supekardowling–degos diseasefollicularhidradenitis suppurativa |
| spellingShingle | Bhagyashree Babanrao Supekar Kinjal Deepak Rambhia Rajesh Pratap Singh Jayesh Ishwardas Mukhi Dowling–Degos disease with follicular involvement associated with hidradenitis suppurativa: A manifestation of follicular occlusion phenomenon? Indian Journal of Dermatology dowling–degos disease follicular hidradenitis suppurativa |
| title | Dowling–Degos disease with follicular involvement associated with hidradenitis suppurativa: A manifestation of follicular occlusion phenomenon? |
| title_full | Dowling–Degos disease with follicular involvement associated with hidradenitis suppurativa: A manifestation of follicular occlusion phenomenon? |
| title_fullStr | Dowling–Degos disease with follicular involvement associated with hidradenitis suppurativa: A manifestation of follicular occlusion phenomenon? |
| title_full_unstemmed | Dowling–Degos disease with follicular involvement associated with hidradenitis suppurativa: A manifestation of follicular occlusion phenomenon? |
| title_short | Dowling–Degos disease with follicular involvement associated with hidradenitis suppurativa: A manifestation of follicular occlusion phenomenon? |
| title_sort | dowling degos disease with follicular involvement associated with hidradenitis suppurativa a manifestation of follicular occlusion phenomenon |
| topic | dowling–degos disease follicular hidradenitis suppurativa |
| url | http://www.e-ijd.org/article.asp?issn=0019-5154;year=2020;volume=65;issue=4;spage=295;epage=298;aulast=Supekar |
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