Dermatology: Practical and Conceptual


Image Letter | Dermatol Pract Concept. 2022;12(4):e2022200 1

Cobblestone-like Skin
Laura Macca1, Luca Di Bartolomeo1, Claudio Guarneri2

1 Department of Clinical and Experimental Medicine, Section of Dermatology, University of Messina, Messina, Italy.

2 Department of Biomedical and Dental Sciences and Morphofunctional Imaging, Section of Dermatology, University of Messina,  

Messina, Italy.

Citation: Macca L, Di Bartolomeo L, Guarneri C. Cobblestone-like skin. Dermatol Pract Concept. 2022;12(4):e2022200.  
DOI: https://doi.org/10.5826/dpc.1204a200

Accepted: March 2, 2022; Published: October 2022

Copyright: ©2022 Macca 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: Claudio Guarneri, MD, Department of Biomedical and Dental Sciences and Morphofunctional Imaging, Section of 
Dermatology, University of Messina, Messina, Italy C/O A.O.U.P. “Gaetano Martino”, via Consolare Valeria, 1 – 98125 Messina (Italy); 
Phone number: +390902212894. Fax number: +390902927691. E-mail: cguarneri@unime.it

Case presentation

A 55-year-old Caucasian man who was positive for obesity, 

hypertension and congestive heart failure presented for a 

sixteen-months history of recurrent lower limbs swelling with 

progressive thickening of the skin. Clinical examination re-

vealed lymphedema with deformed fibrotic enlargement of 

the extremities. Brownish pigmentation, with generalized li-

chenification (Figure 1A) and cobblestone-like papules and 

nodules on the lower two thirds of both legs were also nota-

ble (Figure 1B). Swabs from some ulcerated lesions of the left 

pretibial area were obtained, but cultures did not show any 

pathogens growth. Histological examination of a nodular le-

sion showed hyperkeratosis, parakeratosis, and acanthosis of 

the epidermis, as well as edema and dilated lymphatic spaces 

in the papillary and reticular dermis. No neoplastic changes 

were found. Diagnosis of elephantiasis nostras verrucosa 

(ENV) was made upon clinical and histopathological findings.

Teaching point

ENV is an uncommon dermatological disorder character-

ized by hyperkeratotic, verrucous, and papillomatous pro-

jections [1]. Keong described it as “a non-filariasis chronic 

lymphedema, causing disfigurement of the extremities, and 

it will lead to recurrent infections and disabilities” [2]. The 

diagnosis is based on anamnesis and peculiar skin modifi-

cations  [1]. Differential diagnosis includes venous stasis 

dermatitis, pretibial myxedema and filariasis [1]. Several 

therapeutic options have been described in literature such 

as skin care, weight reduction, compressive medications and 

physiotherapy [2]. Oral retinoids, surgery, and antimicrobi-

als for super infections have also been used [1]. The unique 

target of the therapy is represented by restoring function and 

reducing physical disability. As chronic lymphedema could 

be the antechamber of lymphangiosarcoma, long-term fol-

low-up is highly recommended [2].



2 Image Letter | Dermatol Pract Concept. 2022;12(4):e2022200

References

1. Guarneri C, Vaccaro M. Cobblestone-like skin. CMAJ. 

2008;179(7):673-674. DOI:10.1503/cmaj.080642. PMID: 

18809899. PMCID: PMC2535733.

2. Kar Keong N, Siing Ngi AT, Muniandy P, Voon Fei W. Ele-

phantiasis nostras verrucosa: a rare complication of lower limb 

lymphoedema. BMJ Case Rep. 2017;2017:bcr2017221492. 

DOI:10.1136/bcr-2017-221492. PMID: 28847994. PMCID: 

PMC5624055.

Figure 1. (A) Lymphedema of the lower extremities, with brownish pigmentation and generalized thick-

ening of the pretibial skin. (B) Close view of cobblestone-like papules and nodules on the lower two-thirds 

of left leg.