Figure 1.
A 14-year-old previously healthy male on no prior medications presented with a generalized eruption that he had for 2 weeks. He reported recent night sweats and denied objective fevers or weight loss, though he had had a cold 2 months prior to 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 patient’s pain was so significant that he could not even turn his head. He had no improvement in response to systemic and topical steroids 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 YOUR DIAGNOSIS?
Diagnosis: 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 1916 and coined in the literature by Habermann in 1925. Whether PLEVA and pityriasis lichenoides chronica are distinct disorders or are simply on a different spectrum of the same disease process remains controversial.1
Etiology
Although the etiology of PLEVA remains unclear, several triggering factors have been speculated. Current theories suggest that it may be a lymphoproliferative process triggered by infectious agents, 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.2-4
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, most commonly fevers. Other commonly reported associations include arthralgias, myalgias and lymphadenopathy. While generalized arthralgias and myalgias 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.5
However, because the histologic features can be non-specific early in the course of the disease (and can be confused with lupus or erythema multiforme), clinical-pathologic correlation is sometimes necessary to make the proper diagnosis. Hematoxylin and eosin 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 include lymphomatoid papulosis, guttate psoriasis, varicella, Gianotti-Crosti syndrome, arthropod assault and pityriasis rosea.4 Because these entities do not mimic PLEVA histologically, an adequate biopsy sample is usually enough to confirm the suspected diagnosis.
Notably, it is particularly important to differentiate PLEVA from lymphomatoid papulosis considering the increased risk of lymphoma in 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.6
Treatment
Several effective treatment options for PLEVA have been reported in the literature. Oral medications include tetracycline, erythromycin, methotrexate, dapsone, chloroquine and cyclophosphamide.4,7 Oral tetracycline and erythromycin have been utilized most often in case reports. Topical medications including tacrolimus and steroids have been shown to only provide moderate relief of pruritis without improving lesion count or size. Cases with marked system involvement, as in febrile ulcerative Mucha-Habermann disease, require more aggressive treatment. Successful treatment options reported have included high-dose systemic steroids, intravenous immunoglobulins and oral cyclosporine.8,9
Our Patient
Laboratory tests included complete blood count with differential, serum measurements of electrolytes, phosphorus, magnesium, total protein, albumin, alkaline phosphatase, kidney and liver function tests, QuantiFERON-TB Gold In-Tube assay, anti-nuclear, anti-SSA/Ro and anti-SSB/La antibodies. Abnormalities included mild elevations of total bilirubin, alkaline phosphatase and aspartate aminotrannsferase.
Histologic examination demonstrated a lichenoid band of lymphocytic inflammation with interface vacuolar change. Numerous extravasated erythrocytes were present in the superficial dermis and focally in the overlying epidermis. Foci of neutrophils were also present within the epidermis (Figure 2).
Figure 2. Hematoxylin and eosin punch biopsy showing superficial and deep perivascular lymphocytic infiltrate with mild interface change and some extravasated red blood cells.
The patient was started on 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 left-sided neck pain had completely resolved.
Conclusion
PLEVA is a rare inflammatory dermatosis. It is characterized by the sudden eruption of pink papules, crusted papules, papulovesicles and/or necrotic papules. The lesions typically cover the entire body. Associated systemic signs and symptoms include most commonly fevers, as well as arthralgias, myalgias and lymphadenopathy. Treatments options include oral medications (tetracycline, erythromycin, methotrexate, dapsone, chloroquine and cyclophosphamide) and topical medications (tacrolimus and steroids). n
Aslan Pirouz is with the David Geffen School of Medicine at University of California, Los Angeles, division of dermatology.
Dr. Young is with the David Geffen School of Medicine at the University of California, Los Angeles, division of dermatology.
Dr. Binder is with the University of California, Los Angeles Department of Pathology Dermatopathology Division.
Dr. Worswick is with the University of California, Los Angeles Department of Medicine Division of Dermatology.
Disclosure: The authors have no conflicts of interest to report.
References
1. 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.
2. Kagaya M, Takahashi H, Akaike J, Gotodo Y, Muraoka S, Wakisaka C. A case of pityriasis lichenoides et varioliformis acuta possibly caused by a primary hepatitis B virus infection. Rinsho Derma (Tokoyo). 2004;46(12):1889-1892.
3. Smith K, Nelson A, Skelton H, Yeager J, Wagner KF. Pityriasis lichenoides et varioliformis acuta in HIV-1+ patients: a marker of early stage disease. The Military Medical Consortium for the Advancement of Retroviral Research (MMCARR). Int J Dermatol. 1997;36(2):104-109.
4. Khachemoune A, Blyumin ML. Pityriasis lichenoides: pathophysiology, classification, and treatment. Am J Clin Dermatol. 2007;8(1):29-36.
5. Hood AF, Mark EJ. Histopathologic diagnosis of pityriasis lichenoides et varioliformis acuta and its clinical correlation. Arch Dermatol. 1982;118(7):478-482.
6. Willemze R, Scheffer E. Clinical and histologic differentiation between lymphomatoid papulosis and pityriasis lichenoides. J Am Acad Dermatol. 1985;13(3):418-428.
7. Skinner RB, Levy AL. Rapid resolution of pityriasis lichenoides et varioliformis acuta with azithromycin. J Am Acad Dermatol. 2008;58(3):524-525.
8. Kim H, 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.
9. 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.
Figure 1.
A 14-year-old previously healthy male on no prior medications presented with a generalized eruption that he had for 2 weeks. He reported recent night sweats and denied objective fevers or weight loss, though he had had a cold 2 months prior to 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 patient’s pain was so significant that he could not even turn his head. He had no improvement in response to systemic and topical steroids 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 YOUR DIAGNOSIS?
Diagnosis: 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 1916 and coined in the literature by Habermann in 1925. Whether PLEVA and pityriasis lichenoides chronica are distinct disorders or are simply on a different spectrum of the same disease process remains controversial.1
Etiology
Although the etiology of PLEVA remains unclear, several triggering factors have been speculated. Current theories suggest that it may be a lymphoproliferative process triggered by infectious agents, 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.2-4
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, most commonly fevers. Other commonly reported associations include arthralgias, myalgias and lymphadenopathy. While generalized arthralgias and myalgias 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.5
However, because the histologic features can be non-specific early in the course of the disease (and can be confused with lupus or erythema multiforme), clinical-pathologic correlation is sometimes necessary to make the proper diagnosis. Hematoxylin and eosin 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 include lymphomatoid papulosis, guttate psoriasis, varicella, Gianotti-Crosti syndrome, arthropod assault and pityriasis rosea.4 Because these entities do not mimic PLEVA histologically, an adequate biopsy sample is usually enough to confirm the suspected diagnosis.
Notably, it is particularly important to differentiate PLEVA from lymphomatoid papulosis considering the increased risk of lymphoma in 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.6
Treatment
Several effective treatment options for PLEVA have been reported in the literature. Oral medications include tetracycline, erythromycin, methotrexate, dapsone, chloroquine and cyclophosphamide.4,7 Oral tetracycline and erythromycin have been utilized most often in case reports. Topical medications including tacrolimus and steroids have been shown to only provide moderate relief of pruritis without improving lesion count or size. Cases with marked system involvement, as in febrile ulcerative Mucha-Habermann disease, require more aggressive treatment. Successful treatment options reported have included high-dose systemic steroids, intravenous immunoglobulins and oral cyclosporine.8,9
Our Patient
Laboratory tests included complete blood count with differential, serum measurements of electrolytes, phosphorus, magnesium, total protein, albumin, alkaline phosphatase, kidney and liver function tests, QuantiFERON-TB Gold In-Tube assay, anti-nuclear, anti-SSA/Ro and anti-SSB/La antibodies. Abnormalities included mild elevations of total bilirubin, alkaline phosphatase and aspartate aminotrannsferase.
Histologic examination demonstrated a lichenoid band of lymphocytic inflammation with interface vacuolar change. Numerous extravasated erythrocytes were present in the superficial dermis and focally in the overlying epidermis. Foci of neutrophils were also present within the epidermis (Figure 2).
Figure 2. Hematoxylin and eosin punch biopsy showing superficial and deep perivascular lymphocytic infiltrate with mild interface change and some extravasated red blood cells.
The patient was started on 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 left-sided neck pain had completely resolved.
Conclusion
PLEVA is a rare inflammatory dermatosis. It is characterized by the sudden eruption of pink papules, crusted papules, papulovesicles and/or necrotic papules. The lesions typically cover the entire body. Associated systemic signs and symptoms include most commonly fevers, as well as arthralgias, myalgias and lymphadenopathy. Treatments options include oral medications (tetracycline, erythromycin, methotrexate, dapsone, chloroquine and cyclophosphamide) and topical medications (tacrolimus and steroids). n
Aslan Pirouz is with the David Geffen School of Medicine at University of California, Los Angeles, division of dermatology.
Dr. Young is with the David Geffen School of Medicine at the University of California, Los Angeles, division of dermatology.
Dr. Binder is with the University of California, Los Angeles Department of Pathology Dermatopathology Division.
Dr. Worswick is with the University of California, Los Angeles Department of Medicine Division of Dermatology.
Disclosure: The authors have no conflicts of interest to report.
References
1. 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.
2. Kagaya M, Takahashi H, Akaike J, Gotodo Y, Muraoka S, Wakisaka C. A case of pityriasis lichenoides et varioliformis acuta possibly caused by a primary hepatitis B virus infection. Rinsho Derma (Tokoyo). 2004;46(12):1889-1892.
3. Smith K, Nelson A, Skelton H, Yeager J, Wagner KF. Pityriasis lichenoides et varioliformis acuta in HIV-1+ patients: a marker of early stage disease. The Military Medical Consortium for the Advancement of Retroviral Research (MMCARR). Int J Dermatol. 1997;36(2):104-109.
4. Khachemoune A, Blyumin ML. Pityriasis lichenoides: pathophysiology, classification, and treatment. Am J Clin Dermatol. 2007;8(1):29-36.
5. Hood AF, Mark EJ. Histopathologic diagnosis of pityriasis lichenoides et varioliformis acuta and its clinical correlation. Arch Dermatol. 1982;118(7):478-482.
6. Willemze R, Scheffer E. Clinical and histologic differentiation between lymphomatoid papulosis and pityriasis lichenoides. J Am Acad Dermatol. 1985;13(3):418-428.
7. Skinner RB, Levy AL. Rapid resolution of pityriasis lichenoides et varioliformis acuta with azithromycin. J Am Acad Dermatol. 2008;58(3):524-525.
8. Kim H, 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.
9. 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.
Figure 1.
A 14-year-old previously healthy male on no prior medications presented with a generalized eruption that he had for 2 weeks. He reported recent night sweats and denied objective fevers or weight loss, though he had had a cold 2 months prior to 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 patient’s pain was so significant that he could not even turn his head. He had no improvement in response to systemic and topical steroids 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 YOUR DIAGNOSIS?
Diagnosis: 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 1916 and coined in the literature by Habermann in 1925. Whether PLEVA and pityriasis lichenoides chronica are distinct disorders or are simply on a different spectrum of the same disease process remains controversial.1
Etiology
Although the etiology of PLEVA remains unclear, several triggering factors have been speculated. Current theories suggest that it may be a lymphoproliferative process triggered by infectious agents, 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.2-4
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, most commonly fevers. Other commonly reported associations include arthralgias, myalgias and lymphadenopathy. While generalized arthralgias and myalgias 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.5
However, because the histologic features can be non-specific early in the course of the disease (and can be confused with lupus or erythema multiforme), clinical-pathologic correlation is sometimes necessary to make the proper diagnosis. Hematoxylin and eosin 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 include lymphomatoid papulosis, guttate psoriasis, varicella, Gianotti-Crosti syndrome, arthropod assault and pityriasis rosea.4 Because these entities do not mimic PLEVA histologically, an adequate biopsy sample is usually enough to confirm the suspected diagnosis.
Notably, it is particularly important to differentiate PLEVA from lymphomatoid papulosis considering the increased risk of lymphoma in 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.6
Treatment
Several effective treatment options for PLEVA have been reported in the literature. Oral medications include tetracycline, erythromycin, methotrexate, dapsone, chloroquine and cyclophosphamide.4,7 Oral tetracycline and erythromycin have been utilized most often in case reports. Topical medications including tacrolimus and steroids have been shown to only provide moderate relief of pruritis without improving lesion count or size. Cases with marked system involvement, as in febrile ulcerative Mucha-Habermann disease, require more aggressive treatment. Successful treatment options reported have included high-dose systemic steroids, intravenous immunoglobulins and oral cyclosporine.8,9
Our Patient
Laboratory tests included complete blood count with differential, serum measurements of electrolytes, phosphorus, magnesium, total protein, albumin, alkaline phosphatase, kidney and liver function tests, QuantiFERON-TB Gold In-Tube assay, anti-nuclear, anti-SSA/Ro and anti-SSB/La antibodies. Abnormalities included mild elevations of total bilirubin, alkaline phosphatase and aspartate aminotrannsferase.
Histologic examination demonstrated a lichenoid band of lymphocytic inflammation with interface vacuolar change. Numerous extravasated erythrocytes were present in the superficial dermis and focally in the overlying epidermis. Foci of neutrophils were also present within the epidermis (Figure 2).
Figure 2. Hematoxylin and eosin punch biopsy showing superficial and deep perivascular lymphocytic infiltrate with mild interface change and some extravasated red blood cells.
The patient was started on 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 left-sided neck pain had completely resolved.
Conclusion
PLEVA is a rare inflammatory dermatosis. It is characterized by the sudden eruption of pink papules, crusted papules, papulovesicles and/or necrotic papules. The lesions typically cover the entire body. Associated systemic signs and symptoms include most commonly fevers, as well as arthralgias, myalgias and lymphadenopathy. Treatments options include oral medications (tetracycline, erythromycin, methotrexate, dapsone, chloroquine and cyclophosphamide) and topical medications (tacrolimus and steroids). n
Aslan Pirouz is with the David Geffen School of Medicine at University of California, Los Angeles, division of dermatology.
Dr. Young is with the David Geffen School of Medicine at the University of California, Los Angeles, division of dermatology.
Dr. Binder is with the University of California, Los Angeles Department of Pathology Dermatopathology Division.
Dr. Worswick is with the University of California, Los Angeles Department of Medicine Division of Dermatology.
Disclosure: The authors have no conflicts of interest to report.
References
1. 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.
2. Kagaya M, Takahashi H, Akaike J, Gotodo Y, Muraoka S, Wakisaka C. A case of pityriasis lichenoides et varioliformis acuta possibly caused by a primary hepatitis B virus infection. Rinsho Derma (Tokoyo). 2004;46(12):1889-1892.
3. Smith K, Nelson A, Skelton H, Yeager J, Wagner KF. Pityriasis lichenoides et varioliformis acuta in HIV-1+ patients: a marker of early stage disease. The Military Medical Consortium for the Advancement of Retroviral Research (MMCARR). Int J Dermatol. 1997;36(2):104-109.
4. Khachemoune A, Blyumin ML. Pityriasis lichenoides: pathophysiology, classification, and treatment. Am J Clin Dermatol. 2007;8(1):29-36.
5. Hood AF, Mark EJ. Histopathologic diagnosis of pityriasis lichenoides et varioliformis acuta and its clinical correlation. Arch Dermatol. 1982;118(7):478-482.
6. Willemze R, Scheffer E. Clinical and histologic differentiation between lymphomatoid papulosis and pityriasis lichenoides. J Am Acad Dermatol. 1985;13(3):418-428.
7. Skinner RB, Levy AL. Rapid resolution of pityriasis lichenoides et varioliformis acuta with azithromycin. J Am Acad Dermatol. 2008;58(3):524-525.
8. Kim H, 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.
9. 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.
