‣
Signature ✍️
- Nodule: circumscribed skin elevation; Depth up to 2 cm: deeper skin layers affected (dermis → subQ fat)
- Pustule: skin elevation with visible collection of leukocytes (”pus”); Depth less than 1 cm: superficial skin layers affected (epidermis → dermis)
‣
DDx 🏳️🌈
‣
‣
- Sweet Syndrome 2/2 __
- Endogenous trigger
- Malignancy (AML & MDS most common)
- Autoimmune disorders (IBD, Behcet's, SLE, RA, VEXAS, …)
- Exogenous trigger
- Drug-related (ICIs, TKIs, ATRA, G[M]-CSF, TMP-SMX)
- Post-infectious (e.g. pneumococcus)
- Post-vaccination
- Pyoderma Gangrenosum 2/2 __
- IBD (most common), RA
- Paraneoplastic
- Behcet's & others
‣
- Endogenous trigger
- Erythema nodosum (anterior calves 2/2 autoimmunity)
- Lupus erythematosus panniculitis (SLE in majority)
- Secondary panniculitis (polyarteritis nodosa, necrobiosis lipoidica, vasculitis)
- Pancreatic panniculitis (chronic pancreatitis vs. metastatic acinar carcinoma)
- PPP syndrome (pancreatitis, panniculitis, polyarthralgia due to systemic lipase circulation & lipid crystals)
- Gouty panniculitis (MSU crystal deposition)
- Exogenous trigger
- Erythema induratum (posterior calves 2/2 TB)
- Injection & factitial panniculitis
- Syphilitic panniculitis, other post-infectious
‣
- Granulomatous infections
- Mycobacterial (TB, NTM, & Leprosy)
- Endemic fungi & Cryptococcosis
- Others (Brucellosis & Bartonellosis)
- Neoplasms
- Solid
- Cutaneous metastasis
- Liquid
- Leukemias (AML > CML)
- Cutaneous lymphomas
- T-cell lymphomas (e.g. AITL, SPTCL)
- Blastic plasmacytoid dendritic cell neoplasm (BPDCN)
- Lymphomatoid granulomatosis (LYG)
- Anaplastic large cell lymphoma (ALCL)
- Histiocytoses
- Multicentric reticulohistiocytosis (MRH)
- Fibroblastic rheumatism
- NERDS & MONA syndrome
- Malignant histiocytoses
- Autoimmune
- Rheumatoid nodules
- Deposition
- AL amyloidosis
‣
Principles❗️
The Premise 🧠
- If you think of Skin Nodules as representing deeper diseases of the skin affecting the dermis & subQ fat, an anatomical breakdown of the DDx makes sense (see above)!
- Because the dermis is the middle-most skin layer & affected in both Pustular/Nodular disease, the Neutrophilic Dermatosis DDx (i.e. disease of the dermis) is present in both frameworks!
- Infiltrative diseases land in the dermis due to its rich vascular supply, dense connective tissue (allowing for accumulation & collection of infiltrative cells/molecules), & active immune surveillance. (If you think of the dermis as being strategically located between the epidermis & subQ tissue, it makes sense this is a key place for WBCs to accumulate & fight off foreign cells.)
Definitions 📖
- Neutrophilic dermatoses: spectrum of disorders united by dense, sterile neutrophilic infiltrate, as well as pathergy (dermal injury → pustule → ulcer); the infiltrate begins in the dermis (nodule) & can then extend into the epidermis (pustule → ulcer); Sweet syndrome: acute febrile neutrophilic dermatosis (“juicy,” edematous papules & plaques); Pyoderma gangrenosum: rolled borders with violaceous “gun-metal grey” border → net-like cribiform scarring
- Panniculitis: inflammation of the subcutaneous fat
- Leukemia cutis: cutaneous infiltration of cancer cells (Occurs in 10-15% of AML patients, Less common in chronic myeloproliferative neoplasms)
‣