Lipomatosis benign symmetric (overview)E88.8

Benign disease characterized by disproportionate, unencapsulated, diffuse subcutaneous fatty tissue hyperplasia (especially in the area of the upper trunk), which leads to a different (e.g. pseudoathletic) habitus depending on its location.

Classification using the distribution pattern (according to Donhauser):

• Type I: Neck and neck type (Madelung fat neck, localized type)
• Type II: shoulder girdle type (pseudoathletic type)
• Type III: Pelvic belt type (gynaecological type)
• Type IV: Abdominal type.

Discussed are latent familial clustering, associations with metabolic disorders(hyperlipoproteinemia type IV, hyperuricemia, diabetes mellitus, hypothyroidism and alcoholism with liver dysfunction) and autonomous growth of fat cells due to resistance to catecholamines.

Occurs mainly in men with alcohol abuse. Men are about 13 times more frequently affected than women. Age: 30-60 years.

Diffuse symmetric, massive, tumor-like, doughy dermal proliferation of fatty tissue. Differentiation of lipomas is difficult because of the lack of encapsulation.

In the pseudoatheltic type, there is diffuse fat proliferation on the upper arms as well as in the shoulder area, resulting in a pseudoathletic habitus due to broad-shoulderedness.

In gynecoid type III, the fat deposits are predominantly found on the abdomen and in the pelvic girdle area.

A rare localized variant manifests exclusively on the soles of the feet. Rapid (within 1-2 years), relapsing development followed by stationary adipose tissue hyperplasia with no spontaneous tendency to regress or degenerate.

In Madelung-Fetthals (type I), mechanical limitation of movement of upper cervical joints, dyspnea due to laryngeal and tracheal compression; planar erythema with telangiectasias of the arms (and legs), livedo reticularis.

Frequent association with internal diseases, esp. polyneuropathy, chronic liver damage, gynecomastia, testicular atrophy, diabetes mellitus, hyperlipidemia, hyperuricemia, lung carcinoma, Kaposi's sarcoma, myalgias, arthralgias, and varicosis.

No causal therapy known. Patients often have frustrated dietary attempts behind them. Due to the autonomy of the fat masses, diet or tumor cachexia shows little effect on the affected areas.

Surgical reduction (especially when compressing important structures) or liposuction are possible in principle, but are followed by a high recurrence rate (informing the patient!). In some patients success with salbutamol (e.g. Salbutamol Atid 8 retard) 16 mg/day is reported.

The treatment of associated diseases, e.g. alcoholism, usually does not change the fatty tissue hyperplasia.

By means of liposuction a lasting reduction of fatty tissue can be achieved. The procedure is complex and requires numerous sessions to achieve an acceptable effect.

Standstill of the disease with absolute abstinence from alcohol is possible. Explosive deterioration after trauma or surgical fat tissue reduction (rhytidectomy, liposuction) has been observed. Adipose tissue hyperplasia cannot be influenced dietetically. Resistance in tumor cachexia.

1. Amon U et al (1995) Abdominal variant of a benign symmetrical lipomatosis Launois-Bensaude. dermatologist 46: 722-725
2. Beikert F et al (2015) Liposuction for benign symmetrical lkpomatosis Lenois-Bensaude type II. Act Dermatol 41: 381
3. Carlin MC et al (1988) Multiple symmetric lipomatosis: treatment with liposuction. Jam Acad Dermatol 18: 359-362
4. Constantinidis J et al (2003) Surgical therapy of Madelung's disease in the head and neck area. HNO 51: 216-220
5. Donhauser G et al (1991) Benign symmetrical lipomatosis Launois-Bensaude type 3 and Bureau-Barrière syndrome. dermatologist 42: 311-314
6. Donhauser et al (1991) Benign symmetrical lipomatosis Launois-Bensaude type III and Bureau-Barrière syndrome. dermatologist 42: 311-314
7. Launois PE, Bensaude R (1898) De l'adénolipomatose symétrique. Bull Soc Méd Hôp Paris, Mémoires 1: 298-318
8. Lee HW et al (2003) Multiple symmetric lipomatosis: Korean experience. Dermatol Surgery 29: 235-240
9. Leung NWY et al (1987) Multiple symmetric lipomatosis (Launois-Bensaude syndrome): effect of oral salbutamol. Clin Endocrinol 7: 601-606
10. Madelung OW (1888) About the fat neck (diffuse lipoma of the neck). Langenbecks Arch Klin Surgeon 37: 106-130
11. Nisoli E et al (2002) Multiple symmetric lipomatosis may be the consequence of defective noradrenergic modulation of proliferation and differentiation of brown fat cells. J Pathol 198: 378-387
12. Novak N et al (2000) Benign symmetrical Launois-Bensaude type II lipomatosis with market systemic involvement and psoriasis. dermatologist 51: 427-430