Lipoatrophic panniculitis of children

Iliana L. Aguirre-Martinez, MD a, Antonio Torrelo, MD b,⁎

aDepartment of Dermatology, National Institute of Pediatrics, Mexico City, México
bDepartment of Dermatology, Hospital del Niño Jesus, Madrid, Spain


Abstract Idiopathic lipoatrophic panniculitis of children is a rare disease of childhood, characterized by repeated attacks of tender subcutaneous nodules followed by the development of permanent lipoatro- phy, often seen on the arms and legs, in association with fever, malaise, and other less common clinical manifestations such as abdominal pain or arthralgia. The pathogenesis is unknown, and autoimmune origins, chromosomal alterations, and other causes have been proposed. The nosology of this condition is confusing in the literature, and the precise diagnosis still relies on a combination of clinicopathologic and laboratory assessments. Methotrexate may be recommended to minimize disease progression and morbidity. © 2020 Elsevier Inc. All rights reserved.
© 2020 Elsevier Inc. All rights reserved.


The term panniculitis refers to a group of inflammatory dis- orders in which the primary site of inflammation is in the subcu- taneous fat.1 Inflammation may involve mainly the adipose septa or the adipose lobules, leading to two main groups, the mostly septal and the mostly lobar panniculitis, respectively2,3 (Table 1).
Historically, the nomenclature of the panniculitides has been inconsistent and a potential source of confusion. For example, stasis panniculitis, lipodermatosclerosis, scleros- ing panniculitis, hypodermitis sclerodermiformis, and chro- nic panniculitis with lipomembranous changes are synonymous terms.1
Another example is Weber-Christian disease, once thought to be a distinct condition, featuring attacks of mostly lobar panniculitis with fever and variable systemic findings.4,5 Later studies and better diagnostic algorithms

* Corresponding author. Tel.: + 34915035900.
E-mail address: [email protected] (A. Torrelo).

led to reclassification of many patients under the diagnosis of Weber-Christian disease into other diseases, such as lu- pus panniculitis, factitial panniculitis, panniculitis associ- ated with pancreatic disease, histiocytic cytophagic panniculitis, and α1-antitrypsin deficiency panniculitis.4 It was then agreed that the Weber-Christian eponym should not be used as a distinct entity and that more specific diagno- ses should be made on clinicopathologic grounds.5
Even after the clinicopathologic reclassification of panni- culitis, especially in children, cases still remained of episodic, mostly lobar panniculitis with fever leading to lipoatrophy that could not be characterized under any recognized panni- culitic condition. The reports of these patients had been pub- lished under the names lipophagic panniculitis, lipoatrophic panniculitis of children, connective tissue panniculitis, Weber-Christian panniculitis, Rothman-Makai panniculitis, and others.6 Some reports were written many years ago, when our knowledge of panniculitis was scarce.7
Most experienced pediatric dermatologists have seen such cases, whose common clinical features include recurrent ep- isodes of inflammatory subcutaneous nodules followed by 0738-081X/© 2020 Elsevier Inc. All rights reserved.

2 I.L. Aguirre-Martinez, A. Torrelo
Lipoatrophic panniculitis of children is characterized by an inflammatory lobar panniculitis followed by the develop- ment of permanent lipoatrophy.13 This condition usually af- fects children, but there are reports about this condition in adults.14 The attacks are often associated with fever and sys- temic involvement from inflamed visceral fat. Lipoatrophy is most likely the end stage of the inflammatory process in the adipose tissue.6
Lipoatrophic panniculitis is rarely reported. Collecting
cases from the literature has been difficult because complete
pathology reports are lacking in many publications. Fewer than 50 cases featuring typical histopathologic features have been reported.13,14 Another source of confusion is the exis- tence of lobar panniculitis in the setting of connective tissue diseases that are sometimes associated with lipoatrophy or “lipodystrophy.” Lipoatrophic panniculitis is often associ- ated with a number of autoimmune phenomena, including the presence of autoantibodies, mostly antinuclear antibodies.6,14

fat atrophy in the affected area. This entity often affects chil- dren and is commonly associated with fever, malaise, ab- dominal pain, hepatosplenomegaly, and some laboratory abnormalities that usually persist beyond the febrile attacks of panniculitis. The term lipoatrophic panniculitis of chil- dren seems appropriate to name this entity.


Lipoatrophic panniculitis of children is a rare entity among the panniculitic syndromes.8 In the last 25 years, the terms lipophagocytic panniculitis and lipoatrophic pannicu- litis have been used in descriptions of case reports.9,10 Under the term lipoatrophic panniculitis, cases have been described involving only the ankles.11,12 Although the clinical and his- topathologic features described may resemble the ones de- scribed in cases with generalized lesions, it remains unclear if lipoatrophic panniculitis of the ankles represents a local- ized variant. Because all these cases were described years ago or their histopathologic and immunohistochemical fea- tures were not performed or it is not possible to review them, there is no certainty that they represent the same disorder we review in this contribution.7


The cause of lipoatrophic panniculitis of children is un- known. Patients do not usually report a trigger of physical trauma (thermal, mechanical, or chemical trauma), but a viral infection is sometimes elicited.7 In some cases, elevated levels of circulating immune complexes have been noted, suggesting an immunologically mediated reaction.15 Similar- ities with α1-antitrypsin deficiency panniculitis suggest that an altered regulation of a normal inflammatory process may be involved.16,17 Responses to cyclosporine support a T- cell mediated inflammatory or autoinflammatory process.18 Infections and postviral infectious responses have been in- vestigated, and no connection has yet been established.19,20 Molecular and gene rearrangement studies have failed to identify clonality. An immune-based cause is likely, with fea- tures of both autoinflammation and autoimmunity. A genetic predisposition to the development of the condition has been proposed.13 One report suggested an association with a kar- yotype abnormality on the long arm of chromosome 10. The authors hypothesized supernumerary alleles for the interleukin-2 receptor gene (mapping to chromosome 10p15 region) may facilitate the development of the condi- tion due to increased sensitivity to circulating interleukin-
2.8 This theory is strengthened by further reports of the dis-
ease responding well to cyclosporine, an interleukin-2 inhib- itor. This karyotype abnormality in one case may be neither at odds nor mutually exclusive with the proposal of an auto- immune cause.13
An immune-based cause is supported by the intimate ap- position of CD83+ histiocytes to adipocytes in the inflamma- tory infiltrate. CD83, a member of the immunoglobulin

superfamily of receptors, is the most terminally differentiated dendritic cell marker involved in antigen presentation.21 Monocytes differentiate into mature dendritic cells under the combined influence of interleukin-4 and granulocyte colony-stimulating factor.22 The dendritic cell is critical for antigen processing, whereby there is almost a paradoxical phenomenon for capturing an antigen at the immature stage and presentation to T cells at the mature phase of its life cy- cle.23 The hallmark of a mature dendritic cell—as opposed to an immature one associated with antigen presentation— is CD83 expression.24 One would have to surmise that the in- timate association of the CD83+ mature dendritic cells to the adipocyte membrane suggests a form of type IV hypersensi- tivity targeting the adipocytes.13
Several features in lipoatrophic panniculitis point to an
autoinflammatory cause, but no mutation in any of the genes related to autoinflammatory diseases has been found. Other types of panniculitis have been reported in the setting of im- munodeficiency and autoinflammation, with reported muta- tions in the LCK, TRNT1, and NFκB2 genes25.

The pathogenesis of lipoatrophic panniculitis of children is unknown and remains speculative. Its association with di- abetes mellitus, Hashimoto thyroiditis, and juvenile rheuma- toid arthritis supports an autoimmune pathogenesis.26 A number of reported cases associate lipoatrophic panniculitis with an autoimmune diathesis, but the overall rarity of lipoa- trophic panniculitis and sparse immunohistochemical, mo- lecular, and cytogenetic analyses make it impossible to have certainty about this association.13 It is possible that the immune abnormalities leading to lipoatrophic panniculi- tis also cause varied immune disturbances in other organs. The histopathologic study of early-phase lesions suggests that the primary process is mediated by neutrophils. Later, phagocytosis of lipids by mononuclear phagocytes leads to resultant clinical lipoatrophy.7,26 The clinical picture, includ- ing early-onset, recurrent attacks with fever, sometimes trig- gered by infections, chronic course, and the absence of important laboratory anomalies besides elevated acute- phase reactants, bears resemblance with autoinflammatory conditions.11
Autoinflammation refers to a group of rare, genetic diseases,
caused by molecular defects that lead to constitutive upregula- tion of the innate immune system. A similar panniculitis with lipoatrophy occurs in CANDLE (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature), an autoinflammatory syndrome caused by mutations genes encod- ing proteasome subunits.27 In CANDLE syndrome, proteasome dysfunction leads to increased type 1 interferon production. Because type 1 interferons link innate and adaptive immunity, patients with interferonopathies often have associated autoanti- bodies in their serum.27 The possibility that lipoatrophic

panniculitis were related to increased interferon signature is sup- ported by a strong positivity for STAT1 in the lesional skin by immunohistochemistry.7

Ambiguity surrounding lipoatrophic panniculitis, includ- ing older nomenclature such as Weber-Christian disease, as well as other closely related conditions, makes it difficult to determine the frequency of the diagnosis.19 A chart review of children and adolescents in Brazil over a 20-year period (1983-2002) found 35 pediatric and adolescent cases of pan- niculitis, with only 6 patients meeting criteria for what was previously called Weber-Christian disease.28
No racial predilection is reported. Lipoatrophic panniculi- tis is most often identified in children between ages 3 and 13 years7,14 and occurs more commonly in girls (3:1).29 A sum- mary of well-documented pediatric cases of lipoatrophic pan- niculitis is shown in Table 2.

Although lipoatrophic panniculitis of childhood can be a distinct entity, lipoatrophy is a feature in other types of pan- niculitis, and this has created historic confusion, especially with connective tissue panniculitis.8 A classification of pan- niculitides with lipoatrophy is proposed in Table 3.

Clinical features

Patients are usually children with erythematous, inflamed, and tender subcutaneous nodules that are usually symmetric and measure approximately 1 to 2 cm; however, the nodules may be larger (Figure 1). The overlying skin may look nor- mal or show a violaceous discoloration. The new lesions ap- pear in subsequent attacks, whereas the older ones tend to subside, leaving a striking lipoatrophy (Figure 2). Inflamma- tory nodules usually resolve during a period of weeks to months.6,12
The lesions commonly occur on the thighs and legs and may also involve the arms, trunk, and face.6 Though most cases present with scattered nodules, some patients may pres- ent with acute, extensive subcutaneous inflammation, leading to almost complete loss of subcutaneous fat and a lipody- strophic appearance7 (Figure 3).
The attacks are usually associated with systemic clinical manifestations that include fever, malaise, and other less common clinical manifestations such as nausea, vomiting, abdominal pain, weight loss, bone pain, myalgia, and ar- thralgia. Hepatomegaly or splenomegaly may also be present.6,7

Table 2 Summary of reported cases of lipoatrophic panniculitis in children, under several terms, including lipophagic panniculitis, connective tissue panniculitis, annular lipoa- trophic panniculitis of the ankles, Weber-Christian panniculitis, and Rothman-Makai panniculitis. Extracted from refs 7,9,10,13,14,30–33,41.

Source Cases,No./Sex/Age, yrAffected areas Associated findings
Winkelmann et al 17/12 F, 5M/<1-11Legs (n = 14), feet (n = 2), thighs (n = 10), arms (n=9), trunk (n = 5), face (n = 4)

Not reported

Martinez et al 1/F/3 Arms, legs, buttocks Chromosome 10q26 abnormalityBillings et al 1/M/3 1/F/61/M/9Arms, legs Arms, legs LegsRheumatoid arthritis Type 1 DM, Hashimoto thyroiditisType 1 DMRoth et al 1/F/4 Ankles, thighs, knees Elevated IgG, IgA, and IgM levelsMadasseri et al 1/F/4 ½ Ankles, calves Weakly positive antinuclear antibodiesDimson and Esterly 1/F/6 Ankles, calves Graves disease, alopeciaareata,positive antinuclear antibodies, elevated IgG and IgM levels
Shelley and Izumi 1/F/6 Ankles Not reportedFalcini et al 1/F/8 Legs Not reported
Melchiorre et al 1/F/12 Calves Not reportedShen et al 1/M/8 Ankles Not reported
Jablonska et al 1/F/15 Ankles, legs Elevated IgG and IgM levelsFernandez-Flores and Barja-Lopez1/F/6 Ankles Low IgA and high IgG
Corredera et al 1/F/13 Ankles Celiac disease, primary hypothyroidism, positive antinuclear antibodiesMasala et al 1/F/18 Right ankle and leg
Bilateral brachydactyly of first and fourth fingers, brachydactyly of fourth right toe
Falcini et al 1/F/18 Ankles, legs Not reported
Mirza et al 1/F/9 Legs Vitiligo, Hashimoto thyroiditis
Kerns et al 1/F/6 Ankles, legs Hashimoto thyroiditis
Santonja et al 1/M/12 Feet, ankles, and legs Rheumatoid factor positive

Shen et al 1/M/8 Ankles, legs, extending to wrists, left buttock, and posterior aspect
of the thighNot reported Ng et al 1/F/12 Arm, trunk, face, neck, and thigh. Not reported
Levy et al 1/M/7 Back, flank, and legs Epstein-Barr virus infection associated with hepatosplenomegaly Marque et al 1/M/4 Legs, thighs, buttocks, scrotum, forearms, hands, and feet Not reported
orrelo et al 5/4 F, 1 M/1½- 8Limbs (n = 4),trunk (n = 2), face (n = 2), buttocks (n = 1), abdomen (n = 1)Enlarged lymph nodes, splenomegaly, osteomyelitis
Weryńska-Kalemba et al 1/M/2 Legs Not reportedKumar et al 1/F/1 yr and 10 mo
DM, diabetes mellitus; Ig, immunoglobulin.
Calves, thighs, palms, fingertips, and toes Hepatosplenomegaly,
high levels of IgG, IgA, C3, and C4
must be wise in the election of the best time and site to obtain a skin sample of enough size and depth to permit an accurate diagnosis.6,34 Laboratory investigations are used to support the diagnosis of lipoatrophic panniculitis and to exclude others.


Our medical literature search found fewer than 50 cases of lipoatrophic panniculitis, and some of them have been asso- ciated with immune disorders, such as insulin-dependent di- abetes mellitus, juvenile rheumatoid arthritis, Graves disease, Hashimoto thyroiditis, alopecia areata, vitiligo, ce- liac disease, Raynaud phenomenon, Crohn disease, and par- tial immunoglobulin A deficiency. Some of these associated conditions develop years after the presentation of lipoa- trophic panniculitis.13,22


In the absence of specific diagnostic algorithms for child- hood panniculitis, clinicians must rest on the diagnostic pro- cedures usually performed in adults. A skin biopsy is a most useful tool for the diagnosis of panniculitis, and clinicians


Histopathologic examination shows a lobar panniculitis without vasculitis. Neutrophils and myeloid cells are more prominent in early lesions, and often surround adipocytes (Figure 4). Later, lymphocytes, plasma cells and eosinophils appear in the infiltrate. In the late stage, macrophages replace granulocytes and complete destruction and phagocytosis of adipocytes (lipophagia) occur731,35 (Figure 5). Even, lipi- dized giant cells have been described.6,12
Immunohistochemistry shows positive stain for myeloperox- idase around individual adipocytes in early stages of the disease (Figure 6). Lymphocytes, both CD4+ and CD8+, are present in the infiltrate in all phases. In a later stage, macrophages stain positive for CD68/PGM1 (Figure 7). Stains with CD56, PD1, FoxP3, CXCL13, CD123, CD30, CD1a, langerin, granzyme and perforin were mostly negative in one study.7

Laboratory studies

Laboratory investigations may identify elevated erythrocyte sedimentation rate and C-reactive protein levels, slight anemia, leukocytosis and thrombocytosis, elevated aspartate amino- transferase or alanine aminotransferase levels, and sometimes positive antinuclear antibody titers or hypocomplementemia.6
Other abnormalities may be due to extensive fatty tissue loss and inability to manage and store lipids, such as hypertri- glyceridemia or hyperglycemia. Increased visceral fat was identified by ultrasound in a patient with almost complete loss of subcutaneous tissue.7 These complications are un- usual in patients with limited areas of lipoatrophy.

Fig. 1 A, B, Subcutaneous nodules with overlying erythema in an infant with early-phase lipoatrophic panniculitis.

Fig. 2 A, B, Areas of lipoatrophy in two girls with lipoatrophic panniculitis. These areas had transient subcutaneous nodules weeks before.

Differential diagnosis Idiopathic lipoatrophic panniculitis of children should be differentiated from other forms of panniculitis,14 including vemurafenib-induced panniculitis in children and adoles- cents,36 granuloma annulare, deep morphea, erythema nodo- sum, foreign body granulomatous reaction, deep erythema annulare centrifugum, lupus erythematosus panniculitis, ery- thema migrans, cellulitis, and urticarial vasculitis. The char- acteristic histologic and immunohistochemistry features of lipoatrophic panniculitis and absence of vasculitis are helpful for the diagnosis.9

In children with unexplained panniculitis, particularly with onset before the age of 2 years, inherited immunodeficiency

Fig. 3 A, B, Extensive form of lipoatrophic panniculitis, with generalized lipoatrophy preceded by generalized inflammation and fever.

Fig. 4 Early phase of lipoatrophic panniculitis: lobar panniculitis with a mixed infiltrate containing myeloid cells and polymorphonu- clears. These surround individual adipocytes (hematoxylin-eosin stain, original magnification ×20).should be ruled out with appropriate immunologic stud- ies.21,37,38 Familial Mediterranean fever has also been re- ported as a cause of idiopathic lobar panniculitis in children39 Genetic syndromes of lipodystrophy may also be consid- ered in the differential, but lipoatrophy in these cases is not preceded by inflammatory nodules in the subcutaneous



No uniformly effective therapy for lipoatrophic panniculi- tis is known. Clinical experience, especially in children and adolescents, has pointed to the value of corticosteroids and immunosuppressive agents.10,14,33 Therapeutic responses have been reported mainly with the use of methotrexate,

Fig. 5 Late phase of lipoatrophic panniculitis: lobar panniculitis with some lymphocytes and many macrophages. Phagocytosis of adipocytes is a typical feature (hematoxylin-eosin stain, original magnification

Fig. 6 Immunohistochemistry in the early phase of lipoatrophic panniculitis: The infiltrate in lobar panniculitis is rich in myeloper- oxidase (MPO)–positive cells (A), and MPO-positive cells surround adipocytes individually (B).

antimalarial agents (eg, hydroxychloroquine, chloroquine), cyclosporine, dapsone, azathioprine, and saturated solution of potassium iodide, with varying degrees of efficacy.6,7,9,14 6,7,9,14 Systemic steroids may be effective in suppressing acute exacerbations. Long-term maintenance with oral meth- otrexate is often efficacious to prevent recurrent attacks. Non- steroidal anti-inflammatory agents may reduce fever, arthralgias, and other signs of malaise.7,14 In refractory cases, anti–tumor necrosis factor-α therapy and baricitinib could be considered.
Lipoatrophic panniculitis often represents a disfiguring condition that may require reconstructive surgery.40 Meta- bolic complications require appropriate management by an experienced endocrinologist Prognosis and outcome

The course of lipoatrophic panniculitis in children is vari- able. Some patients have a very mild course, with primarily

Fig. 7 Immunohistochemistry in the late phase of lipoatrophic panniculitis: The infiltrate in lobar panniculitis is mostly composed of CD68+ macrophages (A), and such cells also surround adipo- cytes individually (B).cutaneous manifestations, whereas others present with a se- vere febrile disease with continuous activity, leading to pros- tration and malaise. Death may occur in patients with inflammation involving critical visceral organs (lungs, heart, intestines, spleen, kidney, and adrenal gland).6,33

Exacerbations of the cutaneous nodules may occur for sev- eral years,6,10,31 but in many cases the disease enters into long-term remission. The long-term outcome and prognosis are unknown, as are possible complications or associations with other conditions.
Declaration of competing interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared CL-14377 to influence the work reported in this paper.


Dr. Luis Requena provided the histologic pictures


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