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a Both relapses and conversion to systemic disease can occur, so long-term follow-up is needed.

a Both relapses and conversion to systemic disease can occur, so long-term follow-up is needed.

Letterer-Siwe Disease

The disease usually manifests during the first year of life. There is little epidemiologic information (1), but the incidence has been estimated as 0.1-0.5 per 100,000 inhabitants per year. There have been reports on familial cases with autosomal recessive inheritance.

Symptoms include fever, weight loss, rash, lymphadenopathy, hepatospleno-megaly, pancytopenia and purpura. The most common sites of involvement are the scalp and diaper area. Other seborrhoeic sites such as the postauricular area, nasolabial folds, perioral region, and upper trunk are often involved. There are tiny (0.5 mm in diameter) rose-yellow or brownish-red, translucent papules and patches (Fig. 1). In time, the papules become scaly and crusted and may coalesce into plaques. Petechial and purpuric lesions, pustules, and vesicles as well as small erosions can also be seen. Nodules are uncommon, but may be found on the trunk and tend to ulcerate.

Clinical Course and Prognosis

Without treatment, the disease was frequently fatal in the past. Today, the mortality rate is 50-66% assuming major organ dysfunction.

Differential Diagnosis

Seborrhoeic dermatitis, other eczematous lesions and other forms of LCH.

Hand-Schüller-Christian Disease

The typical triad includes osteolytic skull lesions (100%), diabetes insipidus (50%), and exophthalmus (10%). Otitis media, generalized lymphoadenopathy, hepatosple-nomegaly, and pulmonary disease may be additional findings.

Figure 1 Letterer-Siwe disease: Multiple small papules on the scalp in an infant. (Kindly provided by R. Caputo.)

Figure 2 Hand-Schuller-Christian disease: Scaly and crusted papules and plaques in the seborrhoeic areas of the face.

Skin lesions occur in about 30% of cases, usually in the intertriginous areas, most often as papules and nodules which may be ulcerated or superinfected. Typical intertriginous and perinasal lesions as seen in Letterer-Siwe disease may also occur (Fig. 2).

Clinical Course and Prognosis

The disease shows a prolonged course and has a relatively good prognosis with a mortality rate of less than 50%, but detailed data are not available. The biggest problems in survivors are (i) permanent major organ dysfunction due to "internal scarring'' (i.e., persistent liver dysfunction), (ii) endocrine defects, mainly diabetes insipidus, and (iii) bone defects. In patients with cutaneous involvement alone, no matter what age or type of lesions, survival is 100% (age-matched).

Differential Diagnosis

The nodules may be confused with juvenile xanthogranuloma, Spitz's nevus, mastocytoma, or lymphoma. The intertriginous lesions simulate eczema, psoriasis, Hailey-Hailey disease, or tinea corporis.

Eosinophilic Granuloma

Patients have one or a few localized lesions. The most common site of involvement is the bone. A clinically distinct form is the involvement of the maxilla or mandible with loss of teeth (floating teeth). The uncommon cutaneous lesions are deep dermal or subcutaneous nodules which are not clinically distinct (2,3). In rare cases, only the skin is involved.

Clinical Course and Prognosis

Most lesions can be easily treated, but may locally recur. On rare occasions, the disease may spread to multiple organs (4).

Differential Diagnosis

The spectrum of skin lesions with predominant eosinophilic infiltration is wide and includes granuloma faciale, insect bite reaction, and Wells syndrome.

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