Case Report |
Corresponding author: Rada Gancheva ( rada_ga@mail.bg ) © 2024 Rada Gancheva, Joana Pozharashka, Atanas Koundurdjiev, Milena Nikolova-Vlahova, Petya Yankova, Liubomir Marinchev.
This is an open access article distributed under the terms of the Creative Commons Attribution License (CC BY 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Citation:
Gancheva R, Pozharashka J, Koundurdjiev A, Nikolova-Vlahova M, Yankova P, Marinchev L (2024) A clinical case of pityriasis lichenoides chronica presenting with palpable purpura after streptococcal infection. Folia Medica 66(3): 426-430. https://doi.org/10.3897/folmed.66.e111548
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Pityriasis lichenoides is a rare inflammatory skin condition presenting with diffuse red-brown papules with evolution polymorphism and mica-like crust on older skin lesions. We present a 60-year-old female patient with pityriasis lichenoides chronica that manifested ten days after streptococcal pharyngitis. Initially, palpable purpura appeared on the lower extremities and later, erythematous-squamous papules and plaques appeared at the site of the palpable purpura and on the upper limbs and trunk. The patient had no history of hematological malignancy, viral hepatitis, kidney involvement, systemic rheumatic disease, or ANCA-associated vasculitis. After administration of methylprednisolone 20 mg for one month and an antimalarial agent (hydroxychloroquine 200 mg, 1 tablet bid) for three months, the skin lesions subsided without recurrence.
palpable purpura, pityriasis lichenoides chronica, streptococcal pharyngitis
Pityriasis lichenoides (PL) is a group of inflammatory skin disorders manifesting with erythematous macular lesions on the skin of the trunk and extremities and subsequent evolution to papules with hemorrhagic necrosis and the appearance of skin ulcerations[
The etiology of PL is not well understood. It has been associated with many provocative factors and agent, including infections (herpes viruses – Eppstein-Barr and varicella-zoster, herpes simplex 2, HIV, streptococci, toxoplasma, etc.), drugs, vaccines and xenobiotics, malignancies, especially lymphoma.[
In 2023, we observed a patient with PLC developing after streptococcal pharyngitis, manifesting with palpable purpura of the lower extremities. Written informed consent was obtained from the patient prior to any diagnostic or therapeutic procedure.
A 60-year-old female patient was referred to the Clinic of Rheumatology of Sofiamed University Hospital in January 2023 for joint pain and palpable purpura on lower extremities. She reported having streptococcal pharyngitis in November 2022, with high fever, cervical lymphadenopathy, sore throat, and elevated antistreptolysin titer (537.40, normal <200 Todd units). She saw an ENT specialist, who prescribed amoxycillin and clavulonate (875/125 mg bid) for 10 days, but she took the antibacterial treatment for four days and stopped it without further consultations and despite the good tolerability. Ten days after she stopped the antibiotic, she had one episode of diarrhea followed by pain in the knee and ankle joints with palpable purpura in the thighs and lower legs (Fig.
On December 23, 2022, she consulted an allergologist, who diagnosed skin vasculitis and prescribed treatment with methylprednisolone 16 mg/24 h and gradually decreased the dose. By the time of hospitalization, she was taking 4 mg/24 h. For six days, she also received 0.4 ml of fraxiparin subcutaneously. The laboratory tests in outpatient settings (January 4, 2023) revealed increased leukocyte count (12.8 G/l) with high lymphocyte count (5.6 G/l), normal ESR (16 mm/I h) and C-reactive protein (2.5 mg/l), normal urinalysis, and increased rheumatoid factor (21.6 IU/ml, normal <14). The patient had a past history of arterial hypertension and allergy to tetracycline. She reported having taken penicillin antibiotics in the past with good tolerability of the treatment. The patients had been on corticosteroid treatment from December 23, 2023, until the admission to the Rheumatology Clinic at the end of January 2024.
The physical exam revealed multiple rounded erythematous-squamous plaques on the lower extremities and dorsal surface of the palms, single erythematous-squamous papules and plaques on the trunk and upper limbs, yellowish squamous plaques on the neck and the occipital part of the capillitium (Fig.
The clinical-laboratory investigations revealed normal ESR, complete blood count, fibrinogen, and biochemical investigations; positive ASO: 331.8 Todd units (normal <200), and positive rheumatoid factor: 38.4 IU/ml (normal <20); cryoglobulins, ANA, ANCA, anti-MPO, and anti-PR3 were negative; serum immunoglobulins (IgG, IgA and IgM) and C3 and C4 complement fractions were within the normal limits; HIV, VDRL, HBsAg and anti-HCV were negative; normal urinalysis, and negative urine culture. Chest X-ray examination was normal.
On January 30, 2023, while on 4 mg methylprednisolone, the patient underwent punch skin biopsy (3 mm) from erythematous-squamous papule on the left shoulder. The pathohistological investigation revealed irregularly expressed perivascular lymphocytic inflammatory infiltrate, containing here and there single macrophages and segmented nuclear leukocytes in the upper dermis. The infiltrate was more intense in part of the edematous dermal papillae, accompanied by small erythrocyte extravasates, adjacent to basal vacuolar changes, discrete spongiosis of the epidermis with cap-shaped parakeratosis above these changes. The histological findings were compatible with PLC. The patient was started on intravenous methylprednisolone 20 mg a day for 5 days and the skin lesions gradually faded and subsided with disappearance of erythematous-squamous papules and plaques (Fig.
Fading of erythematous-squamous papules and plaques after five days of treatment with 20 mg intravenous methylprednisolone.
After 5 days of intravenous corticosteroid treatment and the described beneficial clinical evolution of the condition, the patient was discharged and was given oral methylprednisolone 8 mg a day (2 tablets) with gradual dose reduction and withdrawal within one month, and the patient was switched to hydroxychloroquine 200 mg bid for three months without recurrence of symptoms.
Pityriasis lichenoides is a rare inflammatory skin condition that can develop after infection, vaccination or other contact with foreign antigens.[
In our patient, the diagnosis PL was based on the typical clinical manifestations preceded by streptococcal infection, the biopsy findings, and the beneficial effect of corticosteroid treatment. Viral hepatitis, HIV and syphilis were ruled out, and there was no data for skin vasculitis or ANCA-associated vasculitis, no sign of hematological malignancy, serum immunoglobulins and complement fractions were normal. The provoking factor in our patient was streptococcal pharyngitis, proven both clinically and serologically. It is well known that streptococci are a frequent provoking factor for the development of PLC, proven in 79% of the cases, mainly using serological markers.[
In conclusion, we present a female patient with PLC, developing after streptococcal infection of the throat and resolving at the background of low/medium dose of corticosteroid treatment followed by antimalarial agent without further relapses.
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All authors have equally contributed to the diagnosis and treatment of the patient. All authors have contributed to the development of the manuscript. All authors have approved the manuscript.