Dermatology Online Journal

Granulomatous slack skin
Suzan Kavusi MD, Mohammad Javad Nazemi MD, Maryam Ghiasi MD, Yalda Sedaghat MD, Ali Nasertork MD
Dermatology Online Journal 12 (7): 20

Razi Hospital, Tehran University of Medical Sciences. sukavusi@yahoo.com

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Granulomatous slack skin is a rare type of cutaneous T-cell lymphoma characterized by pendulous plaques and dermal elastolysis. We report a middle-aged woman with a few papules and nodules superimposed on a background of lax skin involving her left axilla. Histological examination revealed changes of granulomatous slack skin.

Clinical synopsis

Figure 1
Erythematous papules on lax skin of left axilla

A 43-year-old woman presented with a few asymptomatic erythematous papules and nodules on a background of pendulous skin of left axilla present for 10 years. Scattered erythematous patches had recently developed on her lower abdomen and posterior thighs. Physical examination revealed a few erythematous papules and nodules superimposed on a circumscribed patch of lax skin involving her left axilla. A few erythematous scaly patches were detected on her left breast, lower abdomen, and posterior thigh. No lymphadenopathy or hepatosplenomegaly was detected on abdominal and axillary CT scan. Peripheral blood smear and complete blood count were unremarkable.

Figure 2	Figure 3
Figure 2. Infiltration of small to medium-sized lymphocytes, admixed with many histiocytes, in dermis with obvious epidermotropism (Hematoxylin and eosin x 400) Figure 3. Noncaseating granulomatous dermal infiltrate with multinucleated giant cells (hematoxylin and eosin x 400)

Multiple biopsies were performed both from the papules and the pendulous skin. The first skin biopsy taken from the papules of left axilla showed mild hyperkeratosis and a prominent infiltrate of small- to medium-sized lymphocytes admixed with many histiocytes, arranged in a band-like pattern in papillary and upper reticular dermis. Obvious epidermotropism of atypical lymphocytes was also detected. A second biopsy, taken from pendulous skin, showed noncaseating granulomatous dermal infiltrate with multinucleated giant cells. In this specimen, full thickness dermal elastolysis and near absence of elastic fibers were detected on Orcein-Giemsa staining. A third biopsy from the patches of abdomen showed diffuse lymphocytic infiltrate within the papillary dermis associated with linear aggregation of lymphocytes along the dermo-epidermal junction.

This case was diagnosed as Granulomatous slack skin associated with classic mycosis fungoides (MF).

She was referred for radiation therapy which was not beneficial. On hematological consultation, they suggested a course of chemotherapy but the patient refused. Her disease has remained stable for 13 months.

Discussion

Granulomatous slack skin (GSS) is an extremely rare subtype of cutaneous T-cell lymphoma, reported in approximately 40 patients, characterized clinically by circumscribed areas of pendulous lax skin with a predilection for the axilla and groins and histologically by a granulomatous infiltrate and dermal elastolysis [1, 2, 3]. Patients may present with a single or a few well-circumscribed poikilodermatous or erythematous patches, plaques, or nodules [1]. Over time lesions may become atrophic hyperpigmented pendulous plaques; they are located most often in the axilla, groin, and abdomen [1]. Three times as many cases have been reported in males than in females [2]. Most patients have an indolent clinical course [3]. Although originally regarded by the EORTC as a provisional entry, the new WHO-EORTC classification formally recognizes it as a variant of MF [3, 4]. Clinical differential diagnoses of GSS include middermal elastolysis and acquired cutis laxa, which can be differentiated from GSS by histological examination [2]. Elastolysis is confined to midreticular dermis in middermal elastolysis and acquired cutis laxa lacks the granulomatous infiltrate seen in GSS [2].

Histologically, GSS is characterized by lymphohistiocytic dermal infiltrate with atypical lymphocytes that may demonstrate epidermotropism [2]. Fully developed lesions show dense granulomatous dermal infiltrate containing atypical T cells with slightly indented to cerebriform nuclei, macrophages, and often many multinucleated giant cells, and destruction of elastic tissue [3]. The main histological differential diagnosis of GSS is granulomatous cutaneous T-cell lymphoma. Granulomatous slack skin may be differentiated from granulomatous cutaneous T-cell lymphoma by the presence of pendulous skin lesions and severe dermal elastolysis [2].

In addition, patients with GSS carry a significant risk for the development of a second malignancy. Granulomatous slack skin may be associated with lymphoproliferative disorders, including Hodgkin and non-Hodgkin lymphoma [2]. Association with classic MF has also been reported [3]. The onset of GSS may follow, be concurrent with, or precede the associated malignancy by many years [2]. In our patient, patches of MF developed several years later.

Extracutaneous involvement is rare with GSS. The most common site is that of granulomatous lymphadenitis, and even this finding is rare [5], as in our case.

There is no reliable treatment for granulomatous slack skin although a variety of agents, including cyclophosphamide, adriamycin, vincristine, chlorambucil, procarbazine, busulfan, nitrogen mustard, corticosteroids, interferon, surgery, PUVA, and radiation therapy have been shown to control the progression of disease and flatten skin lesions with variable success [2].

References

1. Clarijs M, Poot F, Laka A, Pirard C, Bourlond A. Granulomatous slack skin: treatment with extensive surgery and review of the literature. Dermatology 2003; 206(4): 393-7. PubMed

2. Lewis KG, Bercovitch L, Dill SW, Robinson-Bostom L. Acquired disorders of elastic tissue: Part II:decreased elastic tissue. J Am Acad Dermatol 2004Aug ; 51(2):165-85. PubMed

3. Willemze R, Jaffe ES, Burg G, Cerroni L, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005 May 15; 105(10): 3768-85. PubMed

4. Slater DN. The new World Health Organization- European Organization of Research and Treatment of Cancer classification for cutaneous lymphoma: a practical marriage of two giants. Br J Dermatol 2005 Nov; 153(5): 874-80. PubMed

5. Gadzia J, Kestenbaum T. Granulomatous slack skin without evidence of a clonal T-cell proliferation. J Am Acad Dermatol 2004 Feb; 50(2Suppl): S4-8. PubMed

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