subcutaneous panniculitis-like T-cell lymphoma

Definition: Subcutaneous panniculitis-like T cell lymphoma is a rare disease, characterized by a typical clinical presentation mimicking panniculitis, and is often associated with systemic symptoms including hemophagocytic syndrome.

Images

 Subcutaneous Panniculitis-Like T-Cell Lymphoma: CD8(+) lymphs, septal sparring, characteristic rimming of adipocytes

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Subcutaneous panniculitis-like T-cell lymphoma is characterized histologically by subcutaneous infiltration of pleomorphic cytotoxic T cells, mimicking a lobular panniculitis.

Although usually described as having an indolent clinical course, the condition may be complicated by systemic involvement and hemophagocytic syndrome, resulting in a poorer prognosis.

Synopsis

 cellulitis-like skin lesion
 subcutaneous nodules
 macrophage activation syndrome (MAS) (hemophagocytic syndrome - HPS)

• fulminant hemophagocytosis

Types

 CD56- subcutaneous panniculitis-like lymphoma (cytotoxic T cells)
 CD56+ subcutaneous panniculitis-like lymphoma

Associations

 Sjögren syndrome (19922028)
 hemophagocytosis (19744747, 19020949, 18797207, 17014024)

Pediatrics

Subcutaneous panniculitis-like T-cell lymphoma is an uncommon form of cutaneous lymphoma in the pediatric population.

Differential diagnosis

 Cytophagic histiocytic panniculitis with fatal haemophagocytic lymphohistiocytosis in a paediatric patient with perforin gene mutation (17601962)

References

 Subcutaneous panniculitis-like T-cell lymphoma accompanied by Sjögren’s syndrome. Yokota K, Akiyama Y, Adachi D, Shindo Y, Yoshida Y, Miyoshi F, Arai E, Kuramochi A, Tsuchida T, Mimura T. Scand J Rheumatol. 2009 Nov-Dec;38(6):494-5. PMID: 19922028

 Aggressive subcutaneous panniculitis-like T-cell lymphoma with hemophagocytosis in two children (subcutaneous panniculitis-like T-cell lymphoma). Koh MJ, Sadarangani SP, Chan YC, Chan MY, Tan AM, Tan SH, Tay YK, Ng SB. J Am Acad Dermatol. 2009 Nov;61(5):875-81. PMID: 19744747

 Takeshita M, Okamura S, Oshiro Y, Okamoto S, Matsuki Y, Nakashima Y, Okamura T, Shiratsuchi M, Hayashi T, Kikuchi M. Clinicopathologic differences between 22 cases of CD56-negative and CD56-positive subcutaneous panniculitis-like lymphoma in Japan. Hum Pathol. 2004 Feb;35(2):231-9. PMID: 14991542

 Takeshita M, Akamatsu M, Ohshima K, Kimura N, Suzumiya J, Kikuchi M, Okamura T, Nakayama J, Imayama S, Uike N, et al. Angiocentric immunoproliferative lesions of the skin show lobular panniculitis and are mainly disorders of large granular lymphocytes. Hum Pathol. 1995 Dec;26(12):1321-8. PMID: 8522304