Dermatology: Practical and Conceptual


Letter  |  Dermatol Pract Concept 2020;10(2):e2020044 1

Dermatology Practical & Conceptual

Introduction

Granulomatous slack skin (GSS) is a very rare subtype of 

mycosis fungoides (MF), with fewer than 50 cases already 

reported. It is 1 of the 3 variants of MF, along with folliculo-

tropic MF and pagetoid reticulosis (World Health Organiza-

tion-European Organization for Research and Treatment of 

Cancer, 2018). There is a male predominance and an onset 

usually during adulthood.

Case Presentation

A 74-year-old man presented with a 4-year history of a 

lesion on his right axilla. During these years the patient had 

received various diagnoses, including intertrigo, dermatitis, 

and inverse psoriasis. Based on his experience, minimal remis-

sion was observed only with the use of a medium-potency 

topical steroid, but the lesion relapsed after treatment discon-

tinuation. Clinical examination revealed a well-circumscribed 

erythematous plaque, consisting of pendulous, wrinkled, 

lax skin, unilaterally affecting the axillary fold (Figure 1). 

Dermoscopy showed pale orange areas on an erythematous 

background and fine linear vessels (Figure 2A), which were 

suggestive of a granulomatous dermatosis. GSS was our pre-

ferred diagnosis, owing to the striking clinical picture and the 

dermoscopic findings. Histological examination following a 

biopsy revealed a dense granulomatous infiltrate in the der-

mis, composed of atypical lymphocytes, macrophages, and 

giant cells (Figure 2B), along with a loss of elastic tissue. The 

epidermis was infiltrated by small, atypical lymphocytes with 

cerebriform nuclei. The atypical T cells had a CD3+, CD4+, 

CD7−, CD8− phenotype. The latter findings, as well as the 

monoclonal rearrangement of the T cell receptor genes, led to 

the diagnosis of GSS. Atypical lymphocytes were not present 

in the peripheral blood smear. Full-body CT scan and the rest 

of the laboratory investigation results were unremarkable. 

The patient partially responded to re-PUVA treatment and 

then he was referred for radiotherapy.

Conclusions

GSS is clinically characterized by slow, progressive develop-

ment of large areas of asymptomatic, pendulous, lax skin 

in the major skin folds, with a predilection for axillae and 

groins. Clinically, differential diagnosis includes acquired 

cutis laxa, anetoderma, as well as other dermatoses involving 

Granulomatous Slack Skin: A Case Report
George Balais,1 Aimilios Lallas,1 Elizabeth Lazaridou,2 Linda Kanatli,3 Zoe Apalla4

1 First Dermatology Department, Aristotle University of Thessaloniki, Greece

2 Second Dermatology Department, Aristotle University of Thessaloniki, Greece

3 Dermatology Department, Bozova National Hospital, Bozova Sanliurfa, Turkey

4 State Dermatology Department, Hippokratio General Hospital, Thessaloniki, Greece

Key words: granulomatous slack skin, mycosis fungoides, T cell, lymphoma, lymphocytes 

Citation: Balais G, Lallas A, Lazaridou E, Kanatli L, Apalla Z. Granulomatous slack skin: a case report. Dermatol Pract Concept. 
2020;10(2):e2020044. DOI: https://doi.org/10.5826/dpc.1002a44

Accepted: January 30, 2020; Published: April 3, 2020

Copyright: ©2020 Balais et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Funding: None.

Competing interests: The authors have no conflicts of interest to disclose.

Authorship: All authors have contributed significantly to this publication.

Corresponding author: George Balais, MD, 83, Kykladon str., 13231, Petroupolis, Athens, Greece. E-mail: yorgosdmp95@gmail.com

https://doi.org/10.5826/dpc.1002a44
mailto:yorgosdmp95@gmail.com


2 Letter  |  Dermatol Pract Concept 2020;10(2):e2020044

the skin folds [1]. To our knowledge, dermoscopic features of 

GSS have never been described in the literature. As expected 

for a granulomatous process, the dermoscopic clues included 

orange areas and fine linear vessels. The latter pattern is not 

diagnostic, but it is suggestive of histological presence of 

granulomas in the dermis.

The histological analysis reveals a dense, diffuse dermal 

infiltrate of atypical, irregular, convoluted lymphocytes (with 

a CD3+, CD4+, and CD8− phenotype) that may extend to 

the subcutaneous tissue, together with diffuse, multinucleated 

giant cells and histiocytes that display prominent elasto-

phagocytosis and lymphophagocytosis. The granulomatous 

infiltrate is correlated with simultaneous loss of elastic fibers. 

Molecular analysis reveals a monoclonal rearrangement of 

the T cell receptor genes. The diagnosis of GSS, as in our case, 

is based on the typical clinical and histological features [2].

The disease follows an indolent course, with an excellent 

prognosis. The reported 5-year survival rate is almost 100%. 

However, since there is an association with other lymphoid 

neoplasias (mainly with Hodgkin lymphoma), lifelong mon-

itoring is recommended [2].

There is no standard of care for GSS. Common thera-

peutic strategies are PUVA, local or total skin electron beam 

radiotherapy, nitrogen mustard, interferons, corticosteroids, 

retinoids, azathioprine, methotrexate, or chemoimmunother-

apy [2]. Surgical excision can be considered, but it is only 

palliative, and recurrences usually occur [1].

References

1. Cerroni L. Granulomatous slack skin. June 2009. Available at: 

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en& 

Expert=33111. Accessed January 10, 2020.

2.  Shah A, Safaya A. Granulomatous slack skin disease: a review, in 

comparison with mycosis fungoides. J Eur Acad Dermatol Vene-

reol. 2012:26(12):1472-1478.

Figure 1. A well-circumscribed erythematous plaque, consisting 

of pendulous, wrinkled, lax skin, occupying the axilla is typical of 

granulomatous slack skin.

Figure 2. (A) The dermoscopic pattern consisting of pale orange ar-

eas on an erythematous background together with fine linear vessels 

is indicative of a granulomatous skin disease. (B) Dermal infiltrate 

consisting of atypical, irregular, convoluted lymphocytes, together 

with multinucleated giant cells (H&E, ×40).

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=33111
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=33111