Poststeroid panniculitis – A rare case report

CASE REPORT Year : 2022 | Volume : 23 | Issue : 2 | Page : 165-169

Taru Garg1, Riaz Ahmed1, Apoorva V Bharadwaj1, Shailaja Shukla2
1 Department of Dermatology and STD, Lady Hardinge Medical College and Associated hospitals, New Delhi, India
2 Department of Pathology, Lady Hardinge Medical College and Associated hospitals, New Delhi, India

Date of Submission26-Jul-2021Date of Acceptance27-Jan-2022Date of Web Publication30-Mar-2022

Correspondence Address:
Dr. Apoorva V Bharadwaj
RMO A Hostel, Bangla Sahib Road, Connaught Place, New Delhi - 110 001
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/ijpd.ijpd_116_21

Poststeroid panniculitis (PSP) is a very rare form of lobular panniculitis that occurs due to rapid tapering of long-term corticosteroid therapy. Herein, we present a case of a 2-year-old boy with PSP following discontinuation of oral prednisolone administered for the treatment of West syndrome. Histopathology revealed lobular panniculitis along with the characteristic needle-shaped clefts. Through this case report, we want to highlight the importance of gradual tapering of steroids and discuss important differential diagnoses with such a case scenario.

Keywords: Needle-shaped clefts, poststeroid panniculitis, steroid withdrawal

How to cite this article:
Garg T, Ahmed R, Bharadwaj AV, Shukla S. Poststeroid panniculitis – A rare case report. Indian J Paediatr Dermatol 2022;23:165-9
How to cite this URL:
Garg T, Ahmed R, Bharadwaj AV, Shukla S. Poststeroid panniculitis – A rare case report. Indian J Paediatr Dermatol [serial online] 2022 [cited 2022 Mar 30];23:165-9. Available from: https://www.ijpd.in/text.asp?2022/23/2/165/341455 Introduction

Poststeroid panniculitis (PSP) is a rare complication of long-term corticosteroid therapy, occurring within 1‒40 days of its rapid discontinuation. Around 25 cases have been reported so far, with most of the patients in the pediatric age group.[1],[2],[3] Patients usually present with multiple tender erythematous nodules, most commonly over areas of maximum fat accumulation that occur as a result of steroid therapy. The most common differential diagnoses for this condition include lupus panniculitis, cold panniculitis, and Weber‒Christian disease.[2] Histopathology is characterized by lobular panniculitis and needle-shaped clefts within the histiocytes and adipocytes. Histopathological differential diagnoses include sclerema neonatorum and subcutaneous fat necrosis (SCFN) of newborn.[2] Most of the cases of PSP are self-limited. Persistent cases may require reintroduction of corticosteroids with gradual tapering.[1],[2],[3] Herein, we present a case of a 2-year-old boy with PSP following discontinuation of corticosteroid therapy.

Case Report

A 2-year-old boy presented to us with a history of fever and multiple tender brown-colored nodules over the trunk and extremities for 10 days. The child was a known case of West syndrome and had received oral prednisolone 35 mg/day for 2 months. During this period, parents noticed increased weight gain, fullness of the face, and increased hair growth over the face. As the child did not have any further seizure episodes, prednisolone was tapered to 25 mg for 2 weeks, 15 mg for 2 weeks, and 5 mg for 2 weeks and then stopped. Within 15 days of stopping the drug, the child developed a low-grade fever and multiple painful nodules on the above mentioned sites. There were no other associated or preceding systemic complaints.

On examination, multiple, tender, discrete, well-defined nodules of size varying from half centimeter to one and half centimeter with overlying skin showing hyperpigmentation were present over the anterior trunk, bilateral upper limbs, and lower limbs [Figure 1]a and [Figure 1]b. The child also had cushingoid facies, hypertrichosis over the bilateral preauricular areas and cheeks, and monomorphic acneiform eruptions over the forehead and cheeks [Figure 1]c. The systemic examination was unremarkable.

Figure 1: (a) skin-colored to erythematous nodules over the trunk (arrow). (b) Nodules over thighs (arrow). (c) Moon-like facies with hypertrichosis and acneiform eruptions

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Hematological investigations revealed normocytic, normochromic blood picture with hemoglobin of 12.8 g/dl, total leucocyte count of 8850 cells/cumm, platelet count of 2.37 lakh, and erythrocyte sedimentation rate of 12 mm/h. Antistreptolysin O titer and C-reactive protein were negative. Serum amylase and lipase were within normal limits. Viral serology for hepatitis B and C viruses was negative. Throat swab for culture and sensitivity was sterile after 48 h of incubation. Mantoux test revealed a normal reading of 2 mm × 2 mm. Urine routine microscopy was normal. Chest radiograph and ultrasound abdomen and pelvis were normal. Antinuclear antibody was negative.

Histopathology revealed lobular panniculitis with mild inflammatory infiltrate consisting of foamy histiocytes and lymphocytes [Figure 2]a. Multiple narrow needle-shaped clefts were present within the histiocytes and necrotic adipocytes [Figure 2]b. Epidermis and dermis were unremarkable.

Figure 2: (a) Low-power magnification (×10) of hematoxylin and eosin stained section showing normal epidermis and dermis. Subcutis showing mild inflammatory infiltrate of histiocytes and lymphocytes. (b) High-power magnification (×40) of hematoxylin and eosin stained section showing needle-shaped clefts within adipocytes (arrow)

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Based on the history, clinical examination, and investigations, a diagnosis of PSP was made. The patient was treated symptomatically for the pain and fever with oral paracetamol 10 mg/kg/dose. Lesions gradually subsided spontaneously over the next 8 weeks.

Discussion

Panniculitis is a broad term referring to inflammation of the subcutaneous fat that occurs as a result of various etiologies such as infections, trauma, drugs, connective tissue disorders, and malignancies. It is challenging at times to differentiate between the various types and identify the etiological factor. Even the histological features may sometimes overlap and cause diagnostic confusion.

PSP is one of the rare forms that occur following long-term administration of high-dose oral or parenteral glucocorticoids followed by their rapid tapering and/or discontinuation.[2] Clinically, it is characterized by tender, at times pruritic, erythematous, or skin-colored nodules over areas where maximum fat accumulation occurs as a result of steroid therapy.[3] Lesions may appear as early as 1 day or as late as 43 days post discontinuation of the drug.[4],[5] The most common sites include cheeks, upper back, neck and sometimes even the jawline, arms, and trunk.[1],[2] Lesions vary in size from half cm to as large as 6 cm.[1] Details of previously reported cases are provided in [Table 1].

Various differential diagnoses based on the clinical presentation include cold panniculitis, lupus panniculitis, and Weber‒Christian disease.[2] In cold panniculitis, lesions occur over areas exposed to cold temperatures within the past 48 h. It is more common in infants and obese females. The lesions resolve with rewarming. In lupus panniculitis, along with positive direct immunofluorescence findings, the overlying skin may show clinical and histological changes of lupus erythematosus and lesions tend to heal with atrophy.[17] Weber‒Christian disease is an idiopathic condition characterized by multiple symmetrical tender nodules along with constitutional symptoms and internal organ involvement. Lesions tend to heal with atrophic scars.[18]

Histopathological features in PSP include lobular panniculitis with lymphohistiocytic inflammatory infiltrate. Foamy histiocytes and multinucleated giant cells maybe present. Needle-shaped clefts are usually present within the histiocytes and necrotic adipocytes.[1],[2],[3] Although characteristic, it is not a specific finding, as needle-shaped clefts are present even in sclerema neonatorum and SCFN. However, the clinical presentation in these conditions is entirely different. Sclerema neonatorum occurs in seriously ill, premature, or low birth weight babies within the 1st week of life and lesions rapidly progress to involve the whole body except for the palms, soles, and genitalia. SCFN occurs in healthy babies in the first few weeks of life and has a good prognosis. Apart from the differences in clinical presentation, on histology, there is the absence of inflammatory infiltrate in sclerema neonatorum and the needle-shaped clefts are arranged radially in SCFN.[17]

In most cases, these nodules resolve spontaneously and gradually with postinflammatory hyperpigmentation over weeks to months. If the lesions are persistent, steroid therapy may be restarted with gradual tapering.[1],[2],[3]

Conclusion

PSP is a rare clinical entity which may mimic conditions which require long-term treatment and cause more morbidity in contrast to good prognosis in these cases. Through our case, we want to highlight the importance of gradual tapering of long-term steroid therapy.

Declaration of consent

The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s) of the patient. In the form the parent(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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