Itchy facial rash spread to back and chest - Clinical Advisor

Itchy facial rash spread to back and chest

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  • CA0311DermClin_Case1

A woman aged 29 years presented with a mildly pruritic facial rash that started suddenly two weeks earlier and spread to her upper chest and upper back over the course of several days. Coincident with the onset of the rash, she experienced malaise, fatigue, and chills that were self-treated with OTC flu medication.

Medical and surgical history was unremarkable, and review of systems was otherwise negative. Physical exam revealed scattered discrete scaling papules, some with central necrosis, on the woman’s forehead, cheeks, and upper chest. A 2-mm punch biopsy of a chest lesion was sent for hematoxylin and eosin stain.
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Microscopic examination revealed parakeratosis and a mid-dermal, superficial perivascular and lichenoid inflammatory cell infiltrate consisting of lymphocytes and histiocytes obscuring the dermoepidermal junction. There was also vacuolar alteration, occasional necrotic keratinocytes, and extravasated erythrocytes at the dermoepidermal junction. These changes...

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Microscopic examination revealed parakeratosis and a mid-dermal, superficial perivascular and lichenoid inflammatory cell infiltrate consisting of lymphocytes and histiocytes obscuring the dermoepidermal junction. There was also vacuolar alteration, occasional necrotic keratinocytes, and extravasated erythrocytes at the dermoepidermal junction. These changes are consistent with a diagnosis of pityriasis lichenoid et varioliformis acuta (PLEVA).

Pityriasis lichenoides, also known as Mucha-Haberman disease, is an uncommon cutaneous disorder best described as a self-limiting lymphoproliferative disorder. It presents acutely as PLEVA and chronically as pityriasis lichenoides chronica (PLC).1 Febrile ulceronecrotic Mucha-Habermann disease is a rare variant characterized by a sudden onset of diffuse large ulceronecrotic lesions accompanied by fever and other systemic signs.

PLEVA occurs most commonly in healthy children and adults younger than age 30 years, with greatest frequency in the preschool and early school-age years. Most cases have been reported in the fall and winter months.2

Lesions of PLEVA begin as erythematous or reddish-brown macules, scaling papules, and papulovesicles. They quickly erupt in crops and progress to papules with central necrosis and hemorrhagic crusting. Physical exam reveals few to many discrete, scattered lesions, mostly in different stages of evolution. The trunk, buttocks, and proximal extremities are the favored sites, although occurrence on the face, palms, soles, and scalp has been documented. Most lesions are asymptomatic, but some patients report mild burning or pruritus. While largely similar to PLEVA lesions, PLC lesions are usually more numerous, demonstrate less necrosis, and often have a fine scale that resembles frosted glass in appearance.

Histologic examination shows necrosis of the keratinocytes, intraepidermal vesiculation, diffuse disappearance of the dermoepidermal junction, dermal edema, confluent para­keratosis, and superficial and deep-dermal lymphohistiocytic infiltrate in a wedge-shaped pattern.3 Often, distinct lymphocytic infiltration around the smaller vessels is noted.

While most cases of PLEVA are idiopathic, several studies have implicated infectious agents and subsequent hypersensitivity reactions. Familial outbreaks, clustering of cases, and comorbid symptoms have been used to support this theory. The most commonly reported associated pathogens are Epstein-Barr virus, Toxoplasma gondii,4 and HIV. In addition, parvovirus,5 adenovirus, herpes simplex, and cytomegalovirus have been associated with PLEVA. One case report describes resolution of PLC in two young patients after tonsillectomy, with throat cultures yielding Staphylococcus aureus and group A beta-hemolytic streptococci.6 In Japan, a 2.5-year-old boy presented with skin lesions consistent with Mucha-Habermann disease that appeared approximately five days after an injection of freeze-dried live attenuated measles vaccine. He responded to both oral and topical corticosteroid therapy.7 More commonly, however, most cases of PLEVA are idiopathic and cannot be attributed to any one cause.

The differential diagnoses include Sweet’s syndrome, guttate psoriasis, varicella zoster virus, gianotti crosti syndrome, and lymphatoid papulosis. For the most part, skin biopsy is sufficient to confirm the diagnosis, and any further workup is tailored to the patient’s presentation and clinical symptoms.

Treatment options are largely experimental, as the disease tends to self-resolve, and no randomized controlled trials have been documented. Case reports have shown improvement with oral erythromycin or tetracycline, phototherapy (psoralen UVA and UVB), topical tacrolimus, and in severe cases, retinoids and low-dose methotrexate.8 Wound care with topical mupirocin may be required with large ulceronecrotic lesions.

Retrospective studies have reported the average duration of PLEVA to be anywhere from 1.6 to 18 months; average duration of lesions in PLC is 7.5 to 20 months.2 In some cases—particularly those involving adults—the lesions may persist for several years or may subside only to recur a few months later.

Many recent studies have focused on the molecular components of PLEVA lesions. Because evaluation of the DNA of biopsy specimens often reveals clonal T-cell receptor beta gene rearrangements, many authors suggest that PLEVA is an indolent cutaneous T-cell disorder.9,10 It is theorized that the limited tendency for PLEVA to progress to cutaneous T-cell lymphoma may reflect host immune response and internal countercheck mechanisms of controlling T-cell proliferations. The NIH’s Office of Rare Diseases Research reports a less than 2% incidence of progression from PLEVA to cutaneous lymphoma.

Because of the small risk of progression to a true cutaneous lymphoma in long-standing cases, lesions that persist longer than a year should be closely monitored. A skin biopsy can rule out malignant transformation.

Our patient was started on a 10-day course of oral erythromycin. She was educated on the usual disease progression and tendency to self-resolution. Regular health maintenance with her primary health-care provider was encouraged. The patient was also advised that any lesion that persists after the remaining rash has resolved should be evaluated. Any changes in lesion morphology — including induration, erosion, atrophy, or poikiloderma — should prompt repeat biopsy to rule out malignancy.

Ms. Stern is a family nurse practitioner at Advanced Dermatology & Skin Surgery in Lakewood, N.J. The author has no relationships to disclose relating to the content of this article.


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References

1. Shieh S, Mikkola DL, Wood GS. Differentiation and clonality of lesional lymphocytes in pityriasis lichenoides chronica. Arch Dermatol. 2001;137:305-308.

2. Ersoy-Evans S, Greco MF, Mancini AJ, et al. Pityriasis lichenoides in childhood: a retrospective review of 124 patients. J Am Acad Dermatol. 2007;56:205-210.

3. Longley J, Demar L, Feinstein RP, et al. Clinical and histologic features of pityriasis lichenoides et varioliformis acuta in children. Arch Dermatol. 1987;123:1335-1339.

4. Nassef NE, Hammam MA. The relation between toxoplasmosis and pityriasis lichenoides chronica. J Egypt Soc Parasitol. 1997;27:93-99.

5. Tomasini D, Tomasini CF, Cerri A, et al. Pityriasis lichenoides: a cyto­toxic T-cell-mediated skin disorder. Evidence of human parvovirus B19 DNA in nine cases. J Cutan Pathol. 2004;31:531-538.

6. Masahiro N, Shin’ya A, Hiroshi I. Two girls with pityriasis lichenoides et varioliformis acuta successfully treated by a tonsillectomy. Nishinihon J Dermatol. 1999;61: 430-434.

7. Torinuki W. Mucha-Habermann disease in a child: possible association with measles vaccination. J Dermatol. 1992;19:253-255.

8. Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes. J Am Acad Dermatol. 2006;55:557-572.

9. Magro CM, Crowson AN, Morrison C, Li J. Pityriasis lichenoides chronica: stratification by molecular and phenotypic profile. Hum Pathol. 2007;38:479-490.

10. Magro C, Crowson AN, Kovatich A, Burns F. Pityriasis lichenoides: a clonal T-cell lymphoproliferative disorder. Hum Pathol. 2002;33:788-795.

All electronic documents accessed February 15, 2011.

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