How are the sclerotic lesions of Buschke-Ollendorff syndrome (also known as scleroderma adultorum) distributed?

Distribution of Sclerotic Lesions in Buschke Scleroedema Adultorum

The sclerotic lesions in Buschke scleroedema adultorum characteristically begin at the neck and shoulders, then extend to the rest of the trunk and proximal limbs, while distinctively sparing the hands, feet, and distal extremities. 1

Typical Distribution Pattern

The disease follows a predictable anatomical progression:

• Initial involvement: The neck and shoulders are the primary sites where scleroedema first manifests 1
• Secondary extension: The thickening and tightening of skin subsequently spreads to involve the rest of the trunk 1, 2
• Proximal limb involvement: The disease can extend to the proximal portions of the upper and lower limbs 1
• Characteristic sparing: The distal extremities (hands and feet) are characteristically not affected, which is a key diagnostic feature distinguishing this condition from systemic sclerosis 1

Clinical Presentation

The distribution creates a distinctive clinical picture:

• Oedematous induration: Patients develop progressive, oedematous thickening of the affected areas, particularly the trunk 1
• Functional impairment: The neck and trunk involvement can lead to impaired mobility, dysphagia, and in severe cases, restrictive respiratory syndrome due to chest wall rigidity 1
• Head involvement: The head may be affected along with the neck in the initial distribution 3
• Upper trunk predominance: The upper trunk shows more prominent involvement than lower areas 3

Clinical Subtypes and Distribution

The distribution pattern remains consistent across the three recognized subtypes of scleroedema adultorum:

• Type 1 (post-infectious): Acute onset following respiratory infections, with the same neck-to-trunk distribution 2, 4
• Type 2 (paraproteinemic): Insidious evolution associated with monoclonal gammopathy, maintaining the characteristic distribution 1
• Type 3 (diabetic): Associated with type 2 diabetes mellitus, showing identical anatomical involvement patterns 1, 4

Distinguishing Features

The distribution pattern helps differentiate scleroedema from other sclerosing conditions:

• Unlike systemic sclerosis, which typically involves the hands and face with distal-to-proximal progression, scleroedema shows proximal-to-distal spread with distal sparing 1
• The symmetrical involvement of neck, shoulders, and trunk without hand involvement is pathognomonic 1, 3