boards’ fodder Panniculitis Roman Bronfenbrener, MD
Septal Predominant Panniculitis Location Clinical Other findings Pathology Treatment
Erythema Nodosum Usually sym- Acute eruption of EN is a delayed hypersensitivity Predominantly Bedrest (EN) metrically in painful, erythema- reaction pattern to a wide variety septal inflammation NSAIDs pretibial area, tous, subcutaneous of ailments, best remembered Discontinue offending but may be nodules with the BEDREST mnemonic No vasculitis medication more dis- present Bruise-like in Behçet’s Treatment of underlying seminated with Characteristic involvement of final stages of evo- condition results in EN lution Estrogen (Pregnancy/ oral con- Miescher’s microgranu- improvement thighs, fore- traceptive pills) loma : arms, upper Heal without residu- Macrophages aggre- Portends a good prog- arms al scarring Drug (sulfa, β-lactams, iodides gated around empty nosis in sarcoidosis in and bromides) clefts or polymorpho- association with hilar Recent Illness (predominantly nuclear cells lymphadenopathy, streptococcal) uveitis, fever, and arthlargias (Löfgren’s Enteropathic (Inflammatory syndrome) bowel disease) Sarcoidosis Tuberculosis (note: ⅓ of cases with NO disease association)
Morphea associated Trunk and Indurated and May be primary sclerosing Septal panniculitis Recalcitrant to panniculitis extremities depressed plaques, disorder of subcutaneous septa with mucin deposition, traditional morphea some with overly- (morphea profunda) or a subcu- infiltrate of lymphocytes therapies ing sclerodermoid taneous extension of overlying with marked increase changes generalized or linear morphea in plasma cells Intralesional triamcinolone Not typically painful Hydroxychloroquine
α-1 antitrypsin Lower trunk Larger than other Most severe manifestations in Diverse, with both Abstinence from deficiency and proximal panniculitides homozygous (PiZZ) α-1 antitryp- septal and lobular pre- alcohol panniculitis extremities sin deficient patients sentations and at sites of Tender erythema- α-1 antitrypsin trauma tous, violaceous, Other manifestations include Polymorphonuclear supplementation via and purpuric early onset emphysema, hepatic cells splayed between weekly IV infusion. plaques with occa- fibrosis with eventual cirrhosis, collagen bundles early sional ulceration pancreatitis, and angioedema in evolutio After IV infusion, panniculitis resolves Eventual lobular necro- rapidly, but scarring sis with “skip areas” of remains normal fat interspersed amongst necrotizing panniculitis
Lobular Predominant Panniculitis Location Clinical Other findings Pathology Treatment
Erythema Classically Female predomi- TB - associated in some cases Lobular or mixed PPD or Interferon induratum (nodular posterior lower nance (tuberculid) panniculitis Gamma Release Assay vasculitis) legs Tender, erythema- Also nocardia and certain medi- Vasculitis of medium Treatment of underlying Also feet, tous nodules and cations (propylthiouracil) have sized vessels concur- infection if indicated. thighs, but- ulcerative plaques been linked rently with mixed infil- Discontinuation of any tocks and arms trate of lymphocytes, offending medications neutrophils, and granu- lomatous inflammation NSAIDs, steroids, SSKI in lobules. Bedrest, compression stockings, smoking avoidance
Pancreatic Most com- In patients with May develop in up to 2% of Fat necrosis with Treatment of underlying panniculitis monly legs, but acute or chronic patients with pancreatic disease. saponification leading pancreatic pathology may be diffuse. pancreatitis, pan- Can develop before clinical signs to basophilic calcium leads to eventual Face generally creatic carcinoma, of pancreatic disease. deposition resolution of spared or congenital pan- panniculitis creatic duct abnor- Schmid’s triad: Subcutaneous Ghost cells - necrosed malities nodules, arthritis, eosinophilia. lipocytes with Associated with poor prognosis thickened walls Tender, erythema- tous subcutaneous Heal with hyperpigmented and Mixed infiltrate of cells, nodules that may depressed scars occasional multinucle- become conflu- ated giant cells ent and exude oily Resolves with fibrosis liquid and fat atrophy Roman Bronfenbrener, Lupus panniculitis Face, proximal Associated with Associated with chronic cutane- Lobular inflamma- Antimalarial therapy limbs, trunk overlying cutane- ous lupus. Only about 10% have tion with occasional MD, is a PGY-2 at arising in ous lupus lesions diagnostic criteria for systemic granulomas, mucin Pulsing steroids may SUNY Stony Brook in crops. Tends ranging from mild lupus erythematosus deposition, lymphocytic be necessary to spare distal erythema to discoid vasculitis Overlying chronic cuta- New York. extremities lesions Frequently overlying neous lupus may be changes of chronic treated with topical or cutaneous lupus ery- intralesional therapy thematosus irinections DResidency p. 4 • Spring 2014 boards’ fodder Panniculitis Roman Bronfenbrener, MD
Lobular Predominant (cont.) Panniculitis Location Clinical Other findings Pathology Treatment Another new Boards’ Fodder, Dermatomyositis Trunk and Sometimes seen Some studies have associated Lymphocytic and Treat underlying panniculitis extremities with established dis- DM panniculitis with favorable plasmacytic lobular dermatomyositis with featuring Heritable ease, but rarely the prognosis or mixed lobuloseptal immunosuppressive initial manifestation inflammation therapy Disorders of Connective Tissue May be seen in Occasional response histologic sections to plaquenil by Margaret without any overly- ing clinical lesions Mioduszewski, MD, PGY-4, is now Traumatic Cold Cold panniculi- Patient’s history is key to diag- Cold panniculitis - mixed Removal of inciting panniculitis Panniculitis - tis - children with nosis; however, many patients infiltrate with septal and event available online small children history of popsicle with psychiatric illness will deny lobular inflammation, in pdf format. on cheeks and ingestion injection of substance granulomas, mucin, and Intralesional steroids chin adipocyte necrosis Download it today Sclerosing lipogran- Surgical excision if Sclerosing uloma - injection of Sclerosing lipogranu- localized process with at www.aad.org/ lipogranuloma mineral oil, paraffin, loma - “Swiss cheese” foreign body present DIR. - male genitalia or other substances appearance of vacuoles Traumatic - into male genitalia (injected foreign sub- sites of blunt stance) within subcuta- trauma or Traumatic - reports neous fat injected due to injection material of Vitamin K, filler Injection panniculitis - materials, as well as Localized inflammation factitious substanc- with polarizable foreign es (milk, feces) body, granulomatous reaction, surrounded by mixed infiltrate
Lipodermatosclerosis Medial lower Acute phase - tender, Chronic venous stasis changes Early - lobular necrosis Leg elevation extremities erythematous nod- with increased capillary perme- and lymphocytic septal ules and plaques, ability and resultant anoxia leads infiltrate with congested Compression support Usually back- “pseudo cellulitis” to sclerosing panniculitis capillaries and hemo- stockings ground of sta- appearance siderin deposits sis dermatitis Clinically most significant in Intralesional kenalog Chronic phase - dependent areas Chronic - “lipomem- Oral androgen therapy indurated red-brown branous changes” with and pentoxyfilline are plaque. “Inverted Early lesions may mimic other thickening of adipocyte panniculitides or even infectious other therapeutic wine bottle” appear- membranes alternatives ance of leg processes
Infection-induced pan- May be at sites Subcutaneous fat Causative agents include May mimic other pan- Appropriate niculitis of direct involvement by bacterial, mycobacterial, fungal, niculitides, although antimicrobial therapy innoculation, infectious process and parasitic organisms commonly a mixed or as dissemi- neutrophil predominant Surgery for more nated lesions in Frequently immuno- Pathogen directly present within infiltrate with vascular vegetative processes septic patients. suppressed patients. subcutis proliferation and (i.e Botryomycosis) Traumatic panniculitis may be hemorrhage. associated with concurrent Microbial stains (i.e. infectious panniculitis Gram stain, acid fast stain, PAS, etc.) are essential for diagnosis
Lobular with Crystal Formation Panniculitis Location Clinical Other findings Pathology Treatment
Sclerema Widespread Diffusely hardened Death frequently from sepsis, Increased saturated Treatment of underly- neonatorum with volar and skin in ill premature heart failure, or respiratory failure fat content in lipocytes ing prematurity-related genital sparing infants occuring predisposes to crystal- medical conditions within first week lization after birth Even with treatment, Needle-shaped clefts 75% of infants suc- in adipocytes with cumb to death minimal surrounding inflammation Sclerema neonatorum is a sign of underlying illness, not the cause of demise
Subcutaneous fat Cheeks, trunk, Localized and Hypercalcemia, Needle-shaped clefts Self-limiting process - necrosis of the thighs circumscribed sub- thrombocytopenia, in adipocytes with neu- no treatment necessary newborn cutaneous nodules hypertriglyceridemia trophils, lymphocytes, with induration, and macrophages with Follow calcium for but generally freely occasional giant cells associated hypercal- mobile cemia (increased 1α hydroxylase by macro- Full term infant in phages in subcutane- first month of life ous fat)
Post-Steroid Cheeks, upper Toddlers to ado- History of rapid steroid withdrawl Lobular involvement Self-limited - no Panniculitis extremities, lescents previously in children with high cumulative with needle-shaped treatment necessary trunk treated with system- doses clefts, multinucleated ic glucocorticoids giant cells, lympho- Restarting steroids and (1-2 weeks prior to cytes, and macro- initiating slow taper presentation) phages may help
References 1. Bolognia JL, Jorizzo JL, Schaffer JV, editors. Bolognia Textbook of Dermatology. 3rd ed. Spain: Mosby Elsevier publishing; 2012: chapter 100. 2. James WD, Berger TG, Elston DM, eds. Andrews’ Diseases of the Skin: Clinical Dermatology. 11th ed. Philadelphia, Pa: Saunders Elsevier; 2011: chap 23. 3. Lebwohl M. The Skin and Systemic Disease: A Color Atlas and Text. 2nd ed. Churchill Livingstone; 2003. irinections www.aad.org/DIR Spring 2014 • p. 5 DResidency