Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
Images in Dermatology
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Media and news
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Images
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Snippets
Special Article
Specialty Interface
Studies
Study Letter
Study Letters
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
Therapy Letters
View Point
Viewpoint
What’s new in Dermatology
Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
Images in Dermatology
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Media and news
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Images
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Snippets
Special Article
Specialty Interface
Studies
Study Letter
Study Letters
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
Therapy Letters
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Net Letter
90 (
5
); 703-703
doi:
10.25259/IJDVL_643_2023
pmid:
38594998

Lipodystrophia centrifugalis abdominals infantilis presenting as a giant ulceration and treatment with hydroxychloroquine and baricitinib

Department of Dermatology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Xicheng, Beijing, China.

Corresponding author: Dr. Zigang Xu, Department of Dermatology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Xicheng, Beijing, China. zigangxupek@163.com

Licence
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Zhao X, Miao C, Chen Y, Xiang X, Liu Y, Zhaoyang W, et al. Lipodystrophia centrifugalis abdominals infantilis presenting as a giant ulceration and treatment with hydroxychloroquine and baricitinib. Indian J Dermatol Venereol Leprol. 2024;90:703. doi: 10.25259/IJDVL_643_2023

Dear Editor,

Lipodystrophia centrifugalis abdominalis infantilis (LCAI) is a localised lipodystrophic disease mainly affecting young children. It’s characterised by a depression of the skin resulting from the loss of subcutaneous fat with an erythematous border involving the lower abdomen and the groin region, neck, or axilla.1 Ulceration can occur sometimes, although it is uncommon.2 Here, we describe a case of LCAI with a giant ulceration which was successfully treated with hydroxychloroquine (HCQ) and baricitinib.

A 4-year-old boy presented with recurrent ulceration on his lower abdomen for 1.5 years. Scattered erythema initially appeared on the right lower abdomen when he was 2.5 years old. Then, the lesions gradually extended to the neighbouring region and a giant ulceration developed within it [Figure 1]. The patient had no pain and denied any systemic symptoms, including fever, fatigue, or weight loss. No abnormality was found in the systemic physical examination. Laboratory tests revealed that blood routine, liver function test, and kidney function tests were all within normal reference range. Tests for infective disease were negative, including next-generation sequencing (NGS) of microorganisms in skin lesions. Serology revealed that antinuclear antibody (ANA) and anti-dsDNA antibodies were negative.

Giant ulceration with thick crust on the abdomen and inguinal region surrounded with infiltrated erythema.
Figure 1:
Giant ulceration with thick crust on the abdomen and inguinal region surrounded with infiltrated erythema.

The patient failed to respond to the treatment of antimicrobials and antifungal agents. A biopsy from the erythematous area was performed that showed small amounts of adipocytes with an inflammatory infiltrate of lymphocytes, histiocytes, and a few plasmacytes [Figures 2a and 2b]. LCAI was diagnosed. He was treated with Hydroxychloroquine (HCQ) at a dosage of 5.5 mg/(kg.d). After 4 months, the lesion had a significant improvement with rapid healing of the ulcer and necrotic crusts falling off. However, there was still an erythematous change at the border occasionally [Figure 3a]. Baricitinib was started at a dosage of 1 mg/day after obtaining informed consent from his parents. The erythema disappeared and the ulceration completely healed over 5 months of treatment with baricitinib combined with HCQ with only a residual scar [Figure 3b]. The total course of treatment for hydroxychloroquine was 14 months and for baricitinib, it was 10 months. After discontinuation of the two drugs for 6 months, the lesion didn’t reoccur [Figure 3c]. The patient is still under follow-up.

Acute and chronic inflammatory cells were visible around the ulceration (Haematoxylin & Eosin, 50x).
Figure 2a:
Acute and chronic inflammatory cells were visible around the ulceration (Haematoxylin & Eosin, 50x).
Subcutaneous fat necrosis with an infiltrate of lymphocytes, histiocytes, and a few plasmacytes (Haematoxylin & Eosin, 200x). Lipodystrophia centrifugalis abdominals infantilis presenting as a giant ulceration and treatment with hydroxychloroquine and baricitinib
Figure 2b:
Subcutaneous fat necrosis with an infiltrate of lymphocytes, histiocytes, and a few plasmacytes (Haematoxylin & Eosin, 200x). Lipodystrophia centrifugalis abdominals infantilis presenting as a giant ulceration and treatment with hydroxychloroquine and baricitinib
Ulcer rapidly healing and necrotic crusts falling off after the treatment of HCQ for 4 months. But there still had an erythematous change at the border occasionally.
Figure 3a:
Ulcer rapidly healing and necrotic crusts falling off after the treatment of HCQ for 4 months. But there still had an erythematous change at the border occasionally.
After the treatment of baricitinib combined with HCQ for 5 months.
Figure 3b:
After the treatment of baricitinib combined with HCQ for 5 months.
After discontinuation of the two drugs for 6 months.
Figure 3c:
After discontinuation of the two drugs for 6 months.

Ulceration is a rare skin manifestation of LCAI and only four patients have been reported to present with it,3 but such a large one has never been reported. We had considered the other diseases that can present such an ulcer [Table 1], but none of them were consistent with the condition of this patient. The etiopathogenesis of LCAI remains unknown and several probable causes have been proposed, including trauma, infection, metabolic or genetic abnormalities, apoptosis, and immunological dysfunctions.1 The principal mechanism underlying ulcer formation may be the vasculitis of the lobular vessels resulting from subcutaneous inflammation. We decided to treat with HCQ because the pathological feature of the disease was predominantly lobular panniculitis which resembled the pattern in lupus erythematosus panniculitis. The result showed it can also be a good choice for the inflammation of LCAI. Besides, baricitinib also showed an inspiring efficacy. The Janus kinase (JAK) inhibitors target and block cytokine signalling mediated by the JAK-signal transducer and activator of transcription (JAK-STAT) pathway, thereby regulating immune response and cell growth and are effective in various skin disorders such as psoriasis, atopic dermatitis, alopecia areata, and lupus erythematosus.4 The JAK1 and JAK2 inhibitor baricitinib was reported to be effective in patients with DM-associated panniculitis.5 We tried to use baricitinib on our patient and it turned out to be effective. To conclude, severe ulceration is rare in LCAI and our case indicated that HCQ combined with baricitinib might be a good approach in such giant ulcerations of LCAI.

Table 1: Differential diagnosis of the case
Disease Differentiating features
infectious granuloma Pathogens can be detected by culture or PCR; histopathology shows granulomatous inflammation
vasculitis Histopathology shows vessel wall and peri-vascular infiltrated by inflammatory cells and vessel wall necrosis
lymphoma Histopathology shows a predominantly lobular infiltrate predominantly composed of lymphocytes with atypia

PCR: Polymerase chain reaction

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

This study was supported by the Beijing Natural Science Foundation (7212037).

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

References

  1. . Lipodystrophia centrifugalis abdominalis infantilis: Statistical analysis of 168 cases. Pediatr Dermatol. 2012;29:437-41.
    [CrossRef] [PubMed] [Google Scholar]
  2. , . Lipodystrophia centrifugalis abdominalis infantilis with ulceration. Dermatol. 2000;200:280-1.
    [Google Scholar]
  3. , , . Lipodystrophia centrifugalis abdominalis infantilis manifesting as recurrent skin ulcerations. Int J Dermatol. 2009;48:1113-5.
    [CrossRef] [PubMed] [Google Scholar]
  4. , , . Emerging topical and systemic JAK inhibitors in dermatology. Front Immunol. 2019;10:2847.
    [CrossRef] [PubMed] [PubMed Central] [Google Scholar]
  5. , , , , , , et al. Improvement of cutaneous inflammation and panniculitis in patients with dermatomyositis by the Janus kinase inhibitor baricitinib. Br J Dermatol. 2022;187:432-5.
    [Google Scholar]

Fulltext Views
2,442

PDF downloads
2,812
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections