Necrotic Papules in a Teenager With Neck Pain
A 14-year-old previously healthy boy on no prior medications presented with a generalized eruption that had been present for 2 weeks. He reported recent night sweats and denied objective fevers or weight loss, although he had had a cold 2 months before presentation. The patient also complained of severe right-sided neck pain, localized to the sternocleidomastoid muscle, that had begun 2 days before the rash started. The pain was so significant that he could not even turn his head. He had no improvement in response to systemic and topical corticosteroids given to him at another physician’s office.
Physical examination revealed widespread erythematous and necrotic papules on the trunk and extremities, and malar erythema sparing the nasolabial folds (Figure 1). Mucous membranes were spared, and no adenopathy was appreciated.
What is the cause of these erythematous papules?
A. Pityriasis lichenoides et varioliformis acuta
B.Guttate psoriasis
C. Lymphomatoid papulosis
D. Varicella
(Answer and discussion on next page)
Answer: A, pityriasis lichenoides et varioliformis acuta
Pityriasis lichenoides et varioliformis acuta (PLEVA), a rare inflammatory dermatosis, was first recognized as the acute variant of pityriasis lichenoides by Mucha in 19161 and coined in the literature by Habermann in 1925.2 Whether PLEVA and pityriasis lichenoides chronica are distinct disorders or simply are different points on the same disease process spectrum remains controversial.3
Etiology
Although the etiology of PLEVA remains unclear, several possible triggering factors have been considered. Current theories suggest that PLEVA might be a lymphoproliferative process triggered by infectious agents, or an inflammatory response secondary to a T-cell dyscrasia, or an immune complex–mediated hypersensitivity. Implicated infectious agents include adenoviruses, parvovirus B19, Epstein-Barr virus, varicella-zoster virus, HIV, hepatitis B virus, Toxoplasma gondii, Staphylococcus aureus, and Streptococcus pyogenes.4-6
Figure 1. Clinical presentation of the patient showing necrotic pink papules on the left forearm.
Clinical Findings
PLEVA is characterized by the sudden eruption of pink papules, crusted papules, papulovesicles, and/or necrotic papules. As with varicella, lesions typically cover the entire body. In addition, there are associated systemic signs and symptoms, the most common of which is fever. Other commonly reported associations are arthralgia, myalgia, and lymphadenopathy. While generalized arthralgia and myalgia have been reported, to our knowledge this is the first case of isolated muscle group involvement.
Histopathology
The histopathologic findings in PLEVA include intraepidermal lymphocytes and erythrocytes, a superficial and deep dermal perivascular lymphocytic infiltrate, and dermal hemorrhage.7
However, because the histologic features can be nonspecific early in the course of the disease (and can be confused with systemic lupus erythematosus or erythema multiforme), clinical–pathologic correlation sometimes is necessary in order to make the proper diagnosis. Results of hematoxylin and eosin staining of a punch biopsy showed superficial and deep perivascular lymphocytic infiltrate with mild interface change and some extravasated red blood cells.
Differential Diagnosis
The clinical differential diagnosis of PLEVA includes lymphomatoid papulosis, guttate psoriasis, varicella, Gianotti-Crosti syndrome, arthropod assault, and pityriasis rosea.6 Because these conditions do not mimic PLEVA histologically, an adequate biopsy sample usually is enough to confirm the suspected diagnosis.
Notably, it is particularly important to differentiate PLEVA from lymphomatoid papulosis, considering the increased risk of lymphoma associated with the latter. The papules in lymphomatoid papulosis can develop into nodules, tumors, and large plaques; the cutaneous findings also tend to persist significantly longer than they do in PLEVA.8
Treatment
Several effective treatment options for PLEVA have been described in the literature. Oral medications include tetracycline, erythromycin, methotrexate, dapsone, chloroquine, and cyclophosphamide.6 Oral tetracycline and erythromycin have been used most often in published case reports.
Topical medications including tacrolimus and corticosteroids have been shown to provide only moderate relief of pruritus without improving lesion count or size. Cases with marked systemic involvement, such as in febrile ulceronecrotic Mucha-Habermann disease, require more aggressive treatment. Reportedly successful treatment options have included high-dose systemic corticosteroids, intravenous immunoglobulins, and oral cyclosporine.9,10
Our Patient
Abnormal laboratory test results included mild elevations of total bilirubin, alkaline phosphatase, and aspartate aminotransferase. Other laboratory tests included a complete blood count with differential; measurement of serum electrolytes, total protein, and albumin; kidney and liver function tests; interferon-γ assay for detection of Mycobacterium tuberculosis infection; and antinuclear, anti–SS-A/Ro, and anti–SS-B/La antibodies.
Histologic examination demonstrated a lichenoid band of lymphocytic inflammation with vacuolar interface change. Numerous extravasated erythrocytes were present in the superficial dermis and focally in the overlying epidermis. Foci of neutrophils also were present within the epidermis (Figure 2).
Figure 2. Hematoxylin and eosin–stained punch biopsy slide showing superficial and deep perivascular lymphocytic infiltrate with mild interface change and some extravasated red blood cells.
The patient was started on a regimen of erythromycin 500 mg orally twice a day. Total resolution of the rash was noted within 10 days. His response to oral erythromycin was also remarkable in that within 2 days, the right-sided neck pain had completely resolved.
Aslan Pirouz, MD, is a first-year dermatology resident at the David Geffen School of Medicine at the University of California, Los Angeles (UCLA).
Lorraine Young, MD, is a clinical professor of medicine and is co-chief of Dermatology Clinical Services at the David Geffen School of Medicine at UCLA.
Scott Binder, MD, is chief of Dermatopathology, director of Pathology Clinical Services, and a professor and senior vice chair in the Department of Pathology and Laboratory Medicine at UCLA.
Scott Worswick, MD, is a physician in the in the Division of Dermatology in the Department of Medicine at UCLA.
Kirk Barber, MD, FRCPC––Series Editor, is a consultant dermatologist at Alberta Children’s Hospital and clinical associate professor of medicine and community health sciences at the University of Calgary in Alberta.
References
1.Mucha V. Über einen der Parakeratosis variegata (Unna) bzw. Pitryriasis lichenoides chronica (Neisser-Juliusberg) nahestehenden eigentümlichen Fall. Arch Dermatol Syph. 1916;123(4):586-592.
2.Habermann R. VIII. Über die akut verlaufende, nekrotisierende Unterart der Pityriasis lichenoides (Pityriasis lichenoides et varioliformis acuta). Dermatol Z. 1925;45:42-48.
3.Fernandes NF, Rozdeba PJ, Schwartz RA, Kihiczak G, Lambert WC. Pityriasis lichenoides et varioliformis acuta: a disease spectrum. Int J Dermatol. 2010;49(3):257-261.
4.Makiko K, Takahashi H, Atsushi A, Yuko G, Shunji M, Chihiro W. A case of pityriasis lichenoides et varioliformis acuta possibly caused by a primary hepatitis B virus infection [in Japanese]. Hifuka No Rinsho. 2004;46(12):1889-1892.
5.Smith KJ, Nelson A, Skelton H, Yeager J, Wagner KF; Military Medical Consortium for the Advancement of Retroviral Research (MMCARR). Pityriasis lichenoides et varioliformis acuta in HIV-1+ patients: a marker of early stage disease. Int J Dermatol. 1997;36(2):104-109.
6.Khachemoune A, Blyumin ML. Pityriasis lichenoides: pathophysiology, classification, and treatment. Am J Clin Dermatol. 2007;8(1):29-36.
7.Hood AF, Mark EJ. Histopathologic diagnosis of pityriasis lichenoides et varioliformis acuta and its clinical correlation. Arch Dermatol. 1982;118(7):478-482.
8.Willemze R, Scheffer E. Clinical and histologic differentiation between lymphomatoid papulosis and pityriasis lichenoides. J Am Acad Dermatol. 1985;13(3): 418-428.
9.Kim HS, Yu DS, Kim JW. A case of febrile ulceronecrotic Mucha-Habermann’s disease successfully treated with oral cyclosporin. J Eur Acad Dermatol Venereol. 2007;21(2):272-273.
10.Pyrpasopoulou A, Athyros VG, Karagiannis A, Chrysomallis F, Zamboulis C. Intravenous immunoglobulins: a valuable asset in the treatment of a case of septic febrile ulceronecrotic Mucha-Habermann disease. Dermatology. 2007; 215(2):164-165.