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Granulomatous slack skin

ORPHA33111
Synonym(s) -
Prevalence <1 / 1 000 000
Inheritance Not applicable
Age of onset Adult
ICD-10
  • C84.0
OMIM -
UMLS
  • C0376407
  • C0457002
MeSH -
MedDRA -

Summary

Disease definition

Granulomatous slack skin (GSS) is a variant of mycosis fungoides (MF; see this term), a form of cutaneous T-cell lymphoma, and is characterized by the presence of circumscribed areas of pendulous lax skin.

Epidemiology

Less than 50 cases have been reported in the literature so far. GSS predominantly affects males (sex ratio: 2.9:1).

Clinical description

Onset usually occurs in adulthood. The disease is characterized by the slow development of large areas of pendulous, lax skin in the major skin folds (axillae and groins). Although GSS is considered as a variant of MF, an association with Hodgkin lymphoma was observed in approximately one-third of the reported patients.

Etiology

The etiology is unknown. A chromosomal translocation, t(3;9)(q12;p24), was found in one case.

Diagnostic methods

The diagnosis is based on typical clinical findings and on histologic features revealing a dermal infiltrate of T-cells (with a CD3+, CD4+ and CD8- phenotype) with granulomatous features and the presence of multinucleated giant cells. Molecular analyses reveal a monoclonal rearrangement of the T-cell receptor genes.

Differential diagnosis

Acquired cutis laxa (see this term) can be differentiated from granulomatous slack skin by histological examination.

Management and treatment

No reliable treatment exists. Surgical excision of the pendulous skin folds is usually performed, but is only palliative and recurrence is the rule. Radiotherapy has been used in some patients. A variety of other therapies (mostly treatments for MF) have been tested in anecdotal cases with variable success.

Prognosis

Most patients have an indolent clinical course.

Expert reviewer(s)

  • Pr Lorenzo CERRONI

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