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Paniculite | Hipodermites

Generalidades
  • Tecido adiposo forma 10-20% do peso corporal normal
  • Diferentes tipos de tecido adiposo
    • Castanho - fetal
    • Visceral
    • Hipoderme
  • Heterogenous group of disease affecting the subcutaneous fat (hipoderme)
    • Constituido por
      • Adipócitos - tem vacúolo
      • Tecido conjuntivo
      • Vasos e nervos
    • Organizam-se
      • Lobulos
      • Septos
  • Difficult topic due to:
    • Clinica pouco variada (erythematous subcutaneous nodules on the lower extremities)
    • Biopsia profunda, pernas onde não gostamos de fazer biópsia
    • Achados histopatológicos são difíeis de interpretar
      • Sobreposição morfológica
      • Tempo de evolução da doença
      • Furthermore, many of these disorders are not isolated to the subcutis, and may also affect the dermis and epidermis
  • Processamento histológico diferente
    • Biópsia deve ser elíptica e vai ser cortada longitudinalmente em vez de transversalmente como nos tumores
 
Classification
  • Septal VS lobular
  • Com ou sem vasculite
  • Natureza do infiltrado
  • Presença de depósitos, cristais
  • Necrose dos adipócitos?
    • Lipofágica
      • Macrófagos espumosos
    • Liquefação
      • Celulas fantasma
      • Por digestao enzimatica
    • Hialinização
      • Formação de material de hialina rodeando adipocitos mumificados
    • Membranosa
      • Microquistos
    • Isquémica
      • Palidez dos adipócitos
      • Celulas fantasma pequenas
    • Basofílica
  • Important to realize these are not mutually exclusive… only “mostly” or “predominantly”
  • No algorithm is perfect
Saurat
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notion image
 

Septal

Requena and Sánchez Yus JAAD volume 45 number 2
Requena and Sánchez Yus JAAD volume 45 number 2
  • Cura sem atrofia ou cicatriz (distingue das paniculites lobulares em que geralmente há atrofia ou cicatriz)
  • Tem vasculite?
    • Com vasculite - são vasculites
      • Pequenos vasos - vasculite leucocitoclásica
      • Médios vasos
        • Artéria - poliarterite nodosa
        • Veia - tromboflebite migratória superficial
    • Sem vasculite
      • Linfócitos e plasmócitos predominantes
      • Histiócitos predominantes

Vasculitis

Leucocytoclastic Vasculitis

  • Rarely presents as subcutaneous nodules and only deep
  • Thickened septa with PMN’s, leukocytoclasis of SMALL blood vessels

Superficial Thromboflebitis

Polyarteritis Nodosa

Lymphocytic

Necrobiosis Lipoidica

Morphea/Scleroderma

  • Can be entirely panniculitic!
  • CAN BE EXCLUSIVE TO THE FAT
  • Mainly lymphocytic without granulomas
  • Thickened septa
  • Spaces between collagen disappears
  • DDx: eosinophilic fasciitis

Granulomatous

Subcutaneous Granuloma Annulare

  • Can be entirely panniculitic!
  • CAN BE EXCLUSIVE TO THE FAT
  • Palisading granuloma around necrobiotic collagen and mucin
  • May see nuclear dust, may see EOSINOPHILS

Rheumatoid nodule

  • 20% of RA patients, poorer prognosis
  • May be larger than other entities discussed
  • Palisading granuloma around necrobiosis and FIBRIN

Necrobiotic Xanthogranuloma

  • Yellowish, telangiectatic plaque
  • Predilection for head/neck
  • IgG kappa paraproteinemia
  • Granulomatous, Touton-type giant cells
  • Cholesterol clefts between collagen bundles

Erythema Nodosum

Lobular

Requena and Sánchez Yus JAAD volume 45 number 2
Requena and Sánchez Yus JAAD volume 45 number 2

Lobular: Vasculitis

Leprosy Treatment Reactions

  • Erythema Nodosum Leprosum
    • Th2
    • Type III hypersensitivity
    • Immune-mediated vasculitis of the fat lobules
    • Tratamento
      • TOC: thalidomide
      • Alternatives: MTX or prednisone + clofazimine
  • Lucio’s phenomenon
    • Necrotizing vasculitis
    • Dermal and fat vessels
    • Ulcers
    • Poor prognosis
    • Thalidomide

Erythema Induratum

Metastatic Crohn’s Disease

  • Difficult diagnosis
  • Location is sometimes helpful (anogenital)
  • Rare manifestation to have vasculitis in Crohn’s (Chron’s is usually granulomatous not vasculitic, but extraintestinal Crohn’s is the exception, may be vasculitic)
  • On lower extremities, mimicks PAN and EN
    • Both which can occur in conjuntion with Crohn’s
  • Can be septal or lobular
    • NON-caseating (distinguishes from EI)

Lobular: Paucicellular (non-vasculitic)

Sclerosing Panniculitis

  • Lipodermatosclerosis
  • Wood-like indurated plaques with telangiectasias, hyperpigmentation and edema in an inverted bottle shape of ankles
  • Underlying chronic venous insufficiency
    • Not evident on vascular studies
  • Histologic
    • Paucicellular, dermal fibrosis, and “lipomembranous” or “membranocystic” changes
    • Cystic spaces with an eosinophilic corrugated membrane
    • Necrose membrano quística

Calciphylaxis

Lobular: Lymphocytes

Cold Panniculitis

Lupus Panniculitis

  • A type of CCLE (Chronic Cutaneous Lupus Erythematosus), rarely seen in SLE (Acute Lupus Erythematosus)
  • Red, subcutaneous nodules which heal with atrophy
    • If overlying & overt DLE, called “lupus profundus”
    • Favorecem localizações atípicas para paniculite (membros superiores, face)
  • Histology
    • May see overlying changes of DLE
    • Lobular, lymphocytic panniculitis with plasma cells
    • “Lymphoid follicles” with plasma cells in periphery
    • May see lymphocytic dust
    • Densidade do infiltrado é tal que pode fazer DDx com linfoma
    • Imunohistoquímica - identificação de agregados de celulas dendriticas plasmocitoides CD 123+

Lobular: Neutrophils

Pancreatic Panniculitis

  • Associated with:
    • Acute or chronic pancreatitis
    • Also seen in acinar-cell type of pancreatic cancer
  • Subcutaneous nodules ulcerate, extruding brownish, oily fluid (fat in the cell being degraded)
  • Follows the course of pancreatitis (but not cancer)
  • Arthritis → because of necrosis of periarticular fat tissue
  • High lipase in most cases pathogenic
    • However multifactorial because only 2-3% of pancreatitis patients have panniculitis, and panniculitis has been seen with normal lipase levels
  • Histology
    • Ghost adipocytes: coagulative necrosis of fat
    • Saponification: proteolytic digestion of fat leads to dystrophic calcification
    • Neutrophils in periphery

Alpha1 Antitrypsin Deficiency

  • Only seen in the homozygous type (PiZZ)
  • Emphysema, hepatitis, cirrhosis, vasculitis, angioedema and panniculitis
  • Do not debride (precipitant)
  • Histology: interstitial neutrophils in the lobules

Infective Panniculitis

Factitial Panniculitis

  • Foreign body reaction
  • Begins neutrophilic → granulomatous
  • Iatrogenic
    • Vitamin K, pentazocine, povidone
  • Cosmetic
    • Silicone or paraffin (mineral oil) → “swiss cheese”
  • Psychiatric
    • Urine, feces, milk, mustard, etc.

Lobular: Histiocytes

Subcutaneous Sarcoidosis

  • AKA Darier-Roussy subtype
  • DDx is EN (but EN with more overlying erythema) - eritema nodoso é uma paniculite reativa no contexto de um doença subjacente (sarcoidose)
  • Lobular not septal
  • Histology
    • Non-caseating granulomas in the fat lobules
    • Pequenos granulomas característicos

Traumatic Panniculitis

  • From blunt trauma
    • Legs and breasts most commonly
    • May be seen as a nodule post-surgically and mistaken for recurrence
  • Histology
    • Cystic spaces in the lobules of fat necrosis
    • Anuclear adipocytes
    • May see foamy histiocytes

Subcutaneous Fat Necrosis of Newborn

Sclerema Neonatorum

Subcutaneous Panniculitis-like T-cell Lymphoma

  • More generalized, F>M
  • Histology
    • Atypical lymphocytes may form ring around adipocyte
    • Cytophagosis by histiocytes (bean-bag cells)
      • May be seen in bone marrow
      • Up to 20% may develop hemophagocytic syndrome
    • CD3+, CD8+, perforin, and TIA-1