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| CASE REPORT |
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| Year : 2015 | Volume
: 20
| Issue : 1 | Page : 79-81 |
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Pityriasis lichenoides et varioliformis acuta (PLEVA)
Nidhi Yadav1, Sumit Kar1, Nitin Gangane2, Bhushan Madke1
1 Department of Dermatology, Venereology, Leprosy, Mahatma Gandhi Institute of Medical Sciences, Sewagram, Wardha, Maharashtra, India
2 Department of Pathology, Mahatma Gandhi Institute of Medical Sciences, Sewagram, Wardha, Maharashtra, India
| Date of Web Publication | 19-Feb-2015 |
Correspondence Address:
Sumit Kar
Department of Dermatology, Venereology, leprosy, Mahatma Gandhi Institute of Medical Sciences, Sewagram - 442 102, Wardha, Maharashtra
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0971-9903.151751
We report a case of a 3-year-old male child presenting with a polymorphous generalized eruption consisting of crusted maculopapular lesions all over body except for face and mucus membrane. Histopathology showed interface dermatitis along with perivascular infiltrates chiefly comprising of lymphocytes and extravasated red blood cells, indicating the diagnosis of pityriasis lichenoides et varioliformis acuta. He was treated with dapsone and eryhthromycin with success. Keywords: Lymphocytic infiltrate, parapsoriasis, pityriasis lichenoides et varioliformis acuta
How to cite this article:
Yadav N, Kar S, Gangane N, Madke B. Pityriasis lichenoides et varioliformis acuta (PLEVA). J Mahatma Gandhi Inst Med Sci 2015;20:79-81 |
| Introduction | |  |
Pityriasis lichenoides (PC) is a rare papulosquamous disorder of unknown etiology. Pityriasis lichenoides (PL) may appear in two clinical forms: The acute variant, known as pityriasis lichenoides et varioliformis acuta (PLEVA); and the chronic variant or pityriasis lichenoides chronica (PLC). [1] . PLEVA may occur at any age, but most frequently occurs in children and young adults. The etiology of PLEVA is unknown. But it has been postulated to be a response to an inflammatory reaction triggered by infectious agents, an immune complex-mediated hypersensitivity, or an inflammatory response secondary to T-cell dyscrasia. [2] At present, pityriasis lichenoides is considered a lymphocytic vasculitis and it is removed from the parapsoriasis group of disorders. [3],[4]
| Case Report | | |
A 3-year-old male child presented with widespread polymorphous generalized eruption with macules and crusted papules all over the body [Figure 1] sparing the face and mucous membrane. His personal and family history were non-contributory. He was not receiving any medications and there was no apparent episode of any infection before the onset of the eruption. Palms, soles, hair, and nails were unremarkable. Differential diagnosis of PLEVA, transient acantholytic dermatoses, and dermatitis herpetiformis was made. | Figure 1: Multiple violaceous macules and papules (red arrow) present over chest, abdomen, bilateral upper and lower limbs
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Histopathology of skin showed irregular acanthosis, interface dermatitis along with perivascular infiltrates chiefly comprising of lymphocytes. Extravasated red blood cells were also seen [Figure 2]. On clinico-pathological correlation, a diagnosis of pityriasis lichenoides et varioliformis acuta (PLEVA) was made. Immunohistochemistry could not be done.He was treated with oral dapsone 25 mg once at night time and syrup erythromycin (150 mg/5 ml) four times a day and after one month of treatment, there was regression of papular lesions [Figure 3]. | Figure 2: Histopathology of skin showing lymphocytic infiltration (yellow arrow) and extravasated red blood cells (green arrow) in dermis
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 | Figure 3: Post treatment photograph showing regression in papular lesions
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| Discussion | | |
In 1916, Viktor Mucha was the first to publish a case of acute papular-squamous eruption named Parakeratosis Variegata (Unna) or Pityriasis Lichenoides Chronica (Neisser-Juliusberg). [5]
In 1925, R. Habermann suggested a new name for it: Pityriasis Lichenoides et Varioliformis Acuta (PLEVA) [5] also known as Mucha-Habermann disease.
Pityriasis lichenoides presents as recurrent crops of spontaneously regressing erythematous to purpuric papules. In PLEVA, individual lesions develop crusts, ulcers, vesicles, or pustules. If dermal damage is extensive, then they may heal with varioliform scars. Lesions are generally asymptomatic. Pityriasis lichenoides is more prevalent in pediatric population but it can affect patients in all age group.
The course of disease is short (11 months) [6] with patients having diffuse distribution of lesions, while those with a peripheral distribution have the longest average clinical course (33 months). [6]
Due to uncertain etiology of PL, clinical management is difficult. Since the condition is self-limiting, evaluation of treatments without adequate controls cannot result in useful recommendations. Case reports suggest the use of multiple oral medications including tetracycline, [7] erythromycin, [8] methotrexate, calciferol, chinoline and acridine derivates, cyclosporine, intravenous gamma globulin, and retinoids. [1] Systemic corticosteroids may have a role in severe cases of PLEVA but should be used with caution especially in pediatric age group. Topical corticosteroids and antihistamines are helpful in symptomatic cases, but they do not alter the course of the disease. Good responses with topical tacrolimus treatment have been reported. [9] There are recent reports of the efficacy of etanercept, oral bromelin, and photodynamic therapy. In our case, we treated the patient with the combination of erythromycin and dapsone. [10],[11] We did keep an eye on hemoglobin level during the course of treatment. The combination of these two safe drugs in a pediatric age group had not been tried till now. We found a dramatic improvement with almost all the lesions disappeared with only residual post inflammatory hyperpigmentation.
In conclusion, we want to emphasize on this rare entity which may present difficulties in diagnosis and therapy. Erythromycin and dapsone was effective in this patient, even though one cannot exclude the possibility of a spontaneous resolution.
| References | | |
| 1. | Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes. J Am Acad Dermatol 2006;55:557-72.  |
| 2. | Fernandes NF, Rozdeba PJ, Schwartz RA, Kihiczak G, Lambert WC. Pityriasis lichenoides et varioliformis acuta: A disease spectrum. Int J Dermatol 2010;49:257-61. |
| 3. | Hood AF, Mark EJ. Histopathological diagnosis of pityriasis lichenoides et varioliformis acuta and its clinical correlation. Arch Dermatol 1982;118:478-82. |
| 4. | Longly J, Demar L, Feinstein RP, Miller RL, Silvers DN. Clinical and histologic features of pityriasis lichenoides et varioliformis acuta in children. Arch Dermatol 1987;123:1335-9. |
| 5. | Nassif PW, Godoy DA, Nakandakari S, Alves CJ, Soares CT. Febrileulceronecrotic Mucha-Habermann disease in adult patient successfully treated with systemic corticosteroid. An Bras Dermatol. 2010;85:891-4. |
| 6. | Wood GS, Reizner G. Other papulosquamous disorders. In: Bolognia JL, Jorizzo JL, Rapini RP, editors. Dermatology. 3 rd ed. US: Elsevier Saunders Publishers; 2012. p. 157-69. |
| 7. | Piamphongsant T. Tetracycline for the treatment of pityriasis lichenoides. Br J Dermatol 1974;91:319-22. |
| 8. | Truhan AP, Herbert AA, Esterly NB. Pityriasis lichenoides in children: Therapeutic response to erythromycin. J Am Acad Dermatol 1986;15:66-70. |
| 9. | Simon D, Boudny C, Nievergelt H, Simon HU, Braathen LR. Successful treatment of pityriasis lichenoides with topical tacrolimus. Br J Dermatol 2004;150:1033-5. |
| 10. | Lazaridou E, Fotiadou C, Tsorova C, Trachana M, Trigoni A, Patsatsi A, et al. Resistant pityriasis lichenoides et varioliformis acuta in a 3-year-old boy: Successful treatment with methotrexate. Int J Dermatol 2010;49:215-7. |
| 11. | Pansé I, Bourrat E, Rybojad M, Morel P. Photochemotherapy for pityriasis lichenoides: 3 cases. Ann Dermatol Venereol 2004;131:201-3. |
[Figure 1], [Figure 2], [Figure 3]
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