10.5826/dpc.1303a183
text/xml
2160-9381
2996
en
Roberto Giacomelli
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
Clinicopathological Correlations
Necrobiosis-Lipoidica-Like Skin Lesions in a Patient with Non-Systemic Sarcoidosis
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2023-07-31
2022-11-19
2023-07-31
2023-08-24
e2023183
Dermatol Pract Concept
Roberto Giacomelli
2023/07/31
10.5826/dpc.1303a183
e2023183
Rheumatology, Immunology, and Clinical Medicine Unit, Department of Medicine, Campus Bio-Medico di Roma University; Immunohematology Unit, University Hospital Fondazione Campus Bio-Medico di Roma, Rome, Italy
2160-9381
Clinicopathological Correlations
en
e2023183
Lallas A, Zaballos P, Zalaudek I, et al. Dermoscopic patterns of granuloma annulare and necrobiosis lipoidica. Clin Exp Dermatol. 2013;38(4):425-427. DOI: 10.1111/ced.12126. PMID: 23495727.
Necrobiosis-Lipoidica-Like Skin Lesions in a Patient with Non-Systemic Sarcoidosis
https://dpcj.org/index.php/dpc/article/download/2996/2230
Necrobiosis-Lipoidica-Like Skin Lesions in a Patient
         with Non-Systemic Sarcoidosis
  Pietro Bearzi1, Francesca Trunfio1, Rosa Coppola2, Damiano Currado1, Claudio Conforti3,
        Salvatore Zanframundo2, Lorenzo Nibid4, Michele Donati4, Vincenzo Panasiti2,
                            Luca Navarini1,5, Roberto Giacomelli1,5


1 Rheumatology, Immunology, and Clinical Medicine Unit, Department of Medicine, Campus Bio-Medico di Roma University, Rome, Italy
2 Department of Reconstructive and Aesthetic Plastic Surgery, University Hospital Fondazione Campus Bio-Medico di Roma, Rome, Italy
3 Dermatology Clinic, Maggiore Hospital of Trieste, Trieste, Italy
4 Department of Pathology, University Hospital Fondazione Campus Bio-Medico di Roma, Rome, Italy
5 Immunohematology Unit, University Hospital Fondazione Campus Bio-Medico di Roma, Rome, Italy



Key words: sarcoidosis, necrobiosis lipoidica, granulomatous skin disease, dermoscopy, clinicopathological correlations
Citation: Bearzi P, Trunfio F, Coppola R, et al. Necrobiosis-Lipoidica-Like Skin Lesions in a Patient with Non-Systemic Sarcoidosis.
Dermatol Pract Concept. 2023;13(3):e2023183. DOI: https://doi.org/10.5826/dpc.1303a183
Accepted: February 8, 2023; Published: July 2023
Copyright: ©2023 Bearzi et al. This is an open-access article distributed under the terms of the Creative Commons Attribution-
NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use,
distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding author: Pietro Bearzi, MD, Department of Allergology, Immunology and Rheumatology, University Hospital Fondazione
Campus Bio-Medico di Roma, via Alvaro del Portillo 21, 00128 Rome, Italy. E-mail: pietro.bearzi@unicampus.it




Introduction                                                             elbow joint tenderness. Six months earlier she underwent left
                                                                         elbow MRI and olecranic bone biopsy, revealing a chronic
Ulcerative-atrophic cutaneous sarcoidosis (CuS) represent a              inflammatory process with focal epithelioid non-necrotizing
rare form of sarcoidosis, showing different clinicopatholog-             granulomas and negative cultural test. Furthermore, the pa-
ical variants: morpheaform, necrobiosis lipoidica (NL)-like              tient was taking levothyroxine for Hashimoto thyroiditis.
and lipodermatosclerosis-like [1]. NL-like CuS is character-                  At physical examination the patient presented multi-
ized by round, depressed skin lesions with hypopigmenta-                 ple red-purple atrophic plaques of the left elbow without
tion and telangiectasias, showing histopathological features             joint tenderness (Figure 1A). Upon dermoscopy, the lesions
of both NL and CuS [1]. Only NL has been associated to                   showed a heterogeneous orange-pinkish background with
diabetic microangiopathy [2].                                            white structureless areas and follicular keratotic plugs. The
                                                                         lesions vascular pattern was characterized by linear irregular,

Case Presentation                                                        arborizing vessels (Figure 1 B and C) and peripheral hairpin
                                                                         vessels (Figure 1C).
A non-diabetic-63-year-old Caucasian female came to our at-                   Skin punch-biopsy revealed numerous non-necrotizing
tention for a one-year history of painful violaceous erythem-            epithelioid granulomas and Langhans giant cells, located
atous plaques on the left forearm and elbow with ipsilateral             in the superficial and deep dermis. Granulomas were



Research Letter | Dermatol Pract Concept. 2023;13(3):e2023183                                                                          1
    Figure 1. (A) multiple red-purple ulcerated plaques of the left elbow, associated with joint pain. (B,C) Dermoscopy showed a hetero-
    geneous orange, pinkish, reddish background with white structureless areas and aspecific vascular pattern: linear irregular, arborizing
    vessels plus peripheral haipin vessels. (D) Tissue slide showing non-necrotizing epithelioid granulomas (arrow) with Langhans giant
    cells (arrowhead) surrounded by lymphocytes. Note the areas of necrobiosis (asterisk) (H&E stain, 5x, scale bar: 500 µm).




surrounded by intense lymphocytic infiltrate with numerous               middle-aged females, without history of diabetes/glucose in-
plasma cells. Moreover, palisading histocytes around areas               tolerance [2,3]. In our patient, the lesions were located at the
of necrobiosis were also prominent (Figure 1D). Other                    upper limbs, while NL and NL-like CuS affect mostly the
causative agents for granuloma formation (ie foreign body,               lower limbs [3]. The coexistence of CuS with NL in the same
microorganisms) were clinically excluded and supported by                lesion underlie the possibility that these represent different
negative Periodic Acid-Schiff (PAS) and Ziehl-Neelsen spe-               stages of the same granulomatous skin disease [3].3
cial stains. In conclusion the clinical, dermoscopic and histo-              Dermoscopy is useful for differential diagnosis of
pathological findings plus the negative systemic assessment              granulomatous skin diseases [4]. NL presents heteroge-
(ie thoracic-CT, pulmonary function test, electrocardiogram,             neous orange-pinkish background and comma/serpentine/
serum ACE, CRP, VES), supported the diagnosis of localized               arborizing vessels [5]. On the other hand, CuS presents a
NL-like CuS with previous ipsilateral bone sarcoidosis. Topi-            pink homogeneous background, white structureless areas
cal betamethasone cream applied twice daily was prescribed.              and fine white scales with disease stage-dependent vascular
At four-months follow up the lesions appeared smaller, less              patterns [4,5]. Our NL-like CuS case showed mixed der-
infiltrated with reduction of referred pain and negative                 moscopic findings: background color compatible with NL,
blood tests.                                                             white structureless areas typical of CuS, and arborizing plus
                                                                         peripheral hairpin vessels compatible with both CuS and

Conclusions                                                              NL [4,5].
                                                                             To the best of our knowledge, this is the first case of upper
Ulcerative/atrophic CuS and NL are granulomatous-skin                    limb NL-like CuS associated to localized sarcoidosis. This
disorders of unknown etiology, sharing similar clinicopath-              CuS variant is associated to systemic sarcoidosis; thus, com-
ological features [2,3]. As for our case, NL-like CuS affects            plete staging and strict follow up is crucial for treatment.2



2                                                                             Research Letter | Dermatol Pract Concept. 2023;13(3):e2023183
First line treatment for localized NL-like CuS is based on              3. Chandan N, Jerdan K, Tsoukas M, Braniecki M. Sarcoidosis
topical/intralesional corticosteroids, while oral prednisone               With Cutaneous Manifestations of Necrobiosis Lipoidica.
                                                                           Am J Dermatopathol. 2020;42(4):308-311. DOI: 10.1097
should be considered for recalcitrant/systemic disease [2,4].
                                                                           /DAD.0000000000001488. PMID: 31313692.
                                                                        4. Ramadan S, Hossam D, Saleh MA. Dermoscopy could be use-
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Research Letter | Dermatol Pract Concept. 2023;13(3):e2023183                                                                            3
