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LYMPHOCYTIC VASCULITIS

Primarily lymphocytic vasculitis is uncommon, although some disorders have a predominantly lymphocytic in?ltrate in later stages (e.g. erythema nodosum, a panniculitis with perivascular inflammation in fat septae, Ch. 22). Pityriasis lichenoides et varioliformis acuta and lymphomatoid papulosis both have features of a lymphocytic vasculitis, and are discussed in Chapter 33. The capillaritis disorders discussed earlier are also lymphocytic, as are chilblains.

Chilblains (perniosis)

Etiology

These lesions are caused by cold, but not at subzero temperatures. They may occur in conjunction with acrocyanosis (Figs 14.11 and 14.12). Lesions may occur on the digits and other bony areas (at any age, Fig.14.13) or over areas of fatty tissue (usually in older children and young adults, mainly women, Fig.22.52).

Figure 14.11 Acrocyanosis demonstrating diffuse dusky purplish color of the skin (left side of picture) compared with a control.

Figure 14.12 Acrocyanosis with chilblains (perniosis) over the proximal interphalangeal joints of the left hand.

Figure 14.13 Chilblains (perniosis). (a) Chilblains on the toes of a young child. These lesions last for several weeks, and may be sore or itchy. (b) Perniosis on the knees of an adult. Symptoms are usually less marked at this site.

Clinical

The lesions occur after cold exposure, but typically cause a hot throbbing quality of pain. They may last several weeks as red tender lumps, and occasionally develop small, necrotic, weeping areas.

Differential diagnosis

The differential diagnosis of chilblains varies according to the sites affected.

    Lesions affecting the digits may be associated with acrocyanosis, and may be confused with Raynaud phenomenon (in which the reperfusion phase may be tender, although the vasospastic whiteness of Raynaud phenomenon is not a feature of chilblains); alternatively, the tenderness and redness may suggest infection or paronychia.

    On areas such as the thigh, overlying fatty tissue, the differential diagnosis includes neutrophilic vascular reactions such as erythema nodosum, nodular vasculitis, and other forms of panniculitis. An important diagnostic feature is that chilblains at such sites are markedly cold to touch.

Treatment

Explanation about warm gloves and clothing to prevent subsequent episodes is important. Non-steroidal antiinflammatory drugs (NSAIDs) may reduce symptoms, and nifedipine can abolish and prevent the digital type but is relatively unhelpful for lesions over fatty areas.

PRACTICE POINTS

White/Cox: Diseases of the Skin, 2ed.(c) 2006, Elsevier Inc. All rights reserved.