Granulomatous slack skin

Alternate names'

Granulomatous slack skin

Definition

Granulomatous slack skin (GSS) is an extremely rare subtype of T-cell cutaneous lymphoma, characterized by the slow and progressive development of sagging skin of redundant aspect, especially in the flexural areas, such as the axillary and inguinal region. GSS is a very rare cutaneous disorder and less than 50 cases have been reported so far.

Cause

The cause of this condition is unknown but it is often associated with mycosis fungiodes and other lymphoproliferative disorders such as Hodgkin and non-Hodgkin lymphoma.

Signs and symptoms

Signs and symptoms include loose hanging skin and fine wrinkling of the skin or papules. These symptoms are most often located in the axilla, groin and stomach. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

30%-79% of people have these symptoms

• Erythema
• Hodgkin lymphoma
• Redundant skin(Loose redundant skin)
• Stiff skin

5%-29% of people have these symptoms

• Abnormality of the lymph nodes(Abnormal lymph node histology)
• Acute kidney injury
• Hypercalcemia(High blood calcium levels)
• Nephrocalcinosis(Too much calcium deposited in kidneys)

Diagnosis

The initial lymph node biopsy suggested sarcoidosis due to the presence of granulomas. Only the correlation of the clinical and the histological cutaneous findings allowed the redirection of the diagnosis towards T-cell cutaneous lymphoma. Clinical followup for a long period of time was also necessary for the final characterization of GSS and its development into high level T-cell cutaneous lymphoma.

GMF has been described as the differential diagnosis for GSS. A multicentric recent study conducted by WHO/EORTC concluded that striking clinical differences exist between these two entities, but their histological findings overlap. It is therefore not possible to distinguish them only on histopathological basis. The looseness of the skin in flexural areas is found only in GSS. The presence of elastophagocytosis is more characteristic of GSS, however, it can also be observed in GMF.

Treatment

There is no specific treatment regimen for GSS. Treatment options are based on the recommendations for cutaneous T-cell lymphoma and include topical steroids, surgical remvoval of skin folds, radiation therapy and chemotherapy. Multiple therapeutic alternatives for GSS have been described, such as topical and systemic corticosteroids, PUVA, radiotherapy, multidrug therapy, immunosuppressive agents such as azathioprine, immunomodulators such as interferon alpha, as well as combined therapies. None of these therapies, however, present sustained satisfactory results. The surgical removal of redundant skin with aesthetic and functional purposes tends to relapse. The response to isolated PUVA and associated with interferon alpha, chemotherapy with gemcitabine and localized radiotherapy at different times had partial and temporary result.

Prognosis

GSS does not entail life-threatening risks, but its prognosis is defined by the development of lympho-proliferative concomitant diseases, such as Hodgkin disease, non-Hodgkin lymphoma, fungoides mycosis, acute myelogenous leukemia and Langerhans cell histiocytosis, in this order of frequency. This concomitance is very frequent, reaching 50%, according to some authors.

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NIH genetic and rare disease info

• NIH info on Granulomatous slack skin

Granulomatous slack skin is a rare disease.

Granulomatous slack skin Resources
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