Sec 10 Disorders of Subcutaneous Tissue

Question 1

Occurs in both genders, at any age from childhood to 70 years of age, but is more common in young women in the 2nd-4th decades of life; considered to be a hypersensitivity reaction presents as an acute onset of tender, painful, erythematous, warm nodules and plaques on the anterior and at times the lateral aspect of both lower legs and ankles lasts from 3 to 6 weeks

Answer 1

Erythema nodosum

Question 2

The nodules of EN may persist a few days or weeks, may become confluent, and evolve from an erythematous or purple-like hue to a bruise-like pigmentation called

Answer 2

Erythema contusiforme

Question 3

Histopathology: widened septa with inflammatory infiltrate including multinucleated giant cells; may have Miescher’s granulomas which shows a discrete micronodular aggregate of small histiocytes around a central stellate cleft

Answer 3

Erythema nodosum

Question 4

Treatment: Erythema nodosum

Answer 4

Treatment or removal of the etiologic factor

Question 5

An inflammatory panniculitis, most commonly presenting with ulcerated nodules on the calves, and frequently associated with MTB infection

Answer 5

Erythema induratum

Question 6

A similar disorder to EI, without ulceration appearing in calves and other lower extremity sites was subsequently described without MTB association

Answer 6

Nodular vasculitis

Question 7

Seen most commonly in young to middle-aged women, presenting as recurrent erythematous to violaceous nodules and deep plaques on the lower legs that may be tender; may heal without scarring, but often ulceration leads to scarring; can have a protracted course with recurrent episodes over years and for the most part, patients are in fairly good health

Answer 7

Erythema induratum/Nodular vasculitis

Question 8

Histopathology: shows a mostly lobular panniculitis with extensive adipocyte necrosis and vascular damage-necrotizing vasculitis of small venules in the fat lobule.

Answer 8

Erythema induratum/Nodular vasculitis

Question 9

Treatment: Erythema induratum

Answer 9

Treatment with triple agent antituberculosis therapy

Question 10

Most common form of panniculitis, occurs in association with venous insufficiency, mostly in overweight women

Answer 10

Lipodermatosclerosis

Question 11

Pathogenetic factors in LDS

Answer 11

Elevated hydrostatic pressure-induced increased vascular permeability secondary to down-regulation of tight junctions with extravascular diffusion of fibrin
Microthrombi
Abnormalities in protein S and protein C
Hypoxia
Damage to endothelial cells by inflammatory cells
Upregulation of intercellular adhesion molecule 1 (ICAM-1), vascular cell adhesion molecule 1 (VCAM-1), leukocyte function-associated antigen 1 (LFA-1), platelet- and endothelial-derived factors
Inflammation with wound healing and local collagen stimulation leading to fibrosis and further vascular and lymphatic damage

Question 12

Presents with very painful, poorly demarcated, cellulitis-like erythematous plaques to purple somewhat edematous indurated plaques or nodules on the lower legs, most commonly on the lower anteromedial calf area; scaling may be present; pain can be so intense; may last a few months or even a year

Answer 12

Acute form of LDS

Question 13

Presents with indurated to sclerotic, depressed, hyperpigmented skin on the lower portion of the lower leg, predominantly but not limited to the medial aspect, or in a stocking distribution; described as having an “inverted champagne bottle” or a “bowling pin” appearance

Answer 13

Chronic form of LDS

Question 14

Biopsy for Liposermatosclerosis if warranted

Answer 14

A thin elliptical excision from the margin of an erythematous and indurated area, closed primarily with sutures

Question 15

Histopathology: Dermal stasis changes are present at any stage, early lesions show a sparse infiltrate of lymphocytes in the septa, accompanied by central lobular ischemic fat necrosis recognized by the presence of pale-staining, small anucleate adipocytes. Capillary congestion is also observed within fat lobule.

Answer 15

Lipodermatosclerosis

Question 16

The major universally recommended treatment for LDS

Answer 16

Compression therapy
30–40 mm Hg - more effective
15–20 mm Hg or 20–30 mm Hg - higher rate of compliance

Question 17

Panniculitis directly caused by an infectious agent which can be due to bacteria, mycobacteria, fungi, protozoa, and viruses.

Answer 17

Infection-induced Panniculitis

Question 18

Clinical appearance varies from fluctuant- or abscess-type lesions with purulent discharge and ulcerations to nonspecific erythematous firm nonfluctuant subcutaneous plaques and nodules, purpuric plaques, and EN-type lesions. The most common sites are the legs and feet. Immunosuppression is associated with more widespread abscess-type lesions.

Answer 18

Infection-induced Panniculitis

Question 19

Histopathology: the subcutaneous fat contains a dense infiltrate of neutrophils and some admixed lymphocytes and macrophages, often with extension into the overlying dermis and with abscess formation a common finding. Other features include hemorrhage, vascular proliferation, foci of basophilic necrosis, and sweat gland necrosis.

Answer 19

Infection-induced Panniculitis

Question 20

Most commonly associated with pulmonary and hepatic disease, leading to chronic obstructive pulmonary disease (COPD), hepatic cirrhosis, or hepatocellular carcinoma; the ZZ genotype is at highest risk.

Answer 20

α1 Antitripsin deficiency

Question 21

Patients present with painful erythematous nodules and plaques, but early lesions may have a cellulitic or fluctuant abscess-type appearance. Lesions may ulcerate and discharge an oily material or serosanguineous discharge and resolve with atrophic scars. The lesions appear most commonly on the lower trunk (buttocks) and proximal extremities. Trauma or excessive activity may precede the onset of lesions.

Answer 21

α1 Antitripsin Deficiency Panniculitis

Question 22

Histopathology: Early lesions may reveal edema and degeneration of adipocytes, with ruptured and collapsed cell membranes and a perivascular mononuclear infiltrate; splaying of neutrophils between collagen bundles throughout the overlying reticular dermis, considered an early and distinctive diagnostic clue. More advanced lesions have masses of neutrophils and histiocytes associated with necrosis and replacement of fat lobules. A focal pattern of involvement is another distinguishing feature with large areas of normal fat in immediate proximity to necrotic septa and fat lobules.

Answer 22

α1 Antitripsin Deficiency Panniculitis

Question 23

Due to release of pancreatic enzymes such as lipase, amylase, and trypsin into the circulation, promoting vascular permeability, leading to release of fatty acids from adipocytes and subsequent fat necrosis.

Answer 23

Pancreatic panniculitis

Question 24

Presents with lesions appearing most frequently on the lower legs, especially the periarticular areas. Lesions often appear in crops, ill-defined erythematous to red–brown edematous tender nodules, which in mild cases may involute and resolve with atrophic hyperpigmented scars, may have central “softer” areas or may become fluctuant, abscess-like, and drain oily material.

Answer 24

Pancreatic panniculitis

Question 25

Histopathology: demonstrate lobular fat necrosis with distinctive qualities. Adipocytes lose their nuclei but maintain peripheral outlines, forming characteristic “ghost cells”. With saponification, calcification occurs, producing fine granular basophilic deposits within and around individual necrotic adipocytes.

Answer 25

Pancreatic panniculitis

Question 26

Rare variant of lupus erythematosus (LE), which primarily affects the subcutaneous AT presents with tender and painful and usually appear on the upper arms (lateral aspect), shoulders, face, scalp, hips, buttocks, breasts, and rarely on the lower extremities. The lesions are deep subcutaneous nodules with or without any surface changes.

Answer 26

Lupus Erythematosus Panniculitis

Question 27

Histopathology: vacuolar alteration of the basal cell layer, thickened basement membrane, mucin deposition between dermal collagen bundles, and a superficial and deep perivascular inflammatory infiltrate of lymphocytes. The AT shows a mostly lobular or mixed lobular and septal panniculitis, with lymphocytes, often with formation of lymphoid follicles.

Answer 27

Lupus Erythematosus Panniculitis

Question 28

First line treatment for Lupus Erythematosus Panniculitis

Answer 28

Antimalarials (Hydroxychloroquine + Quinacrine)

Question 29

Presents as painful indurated erythematous nodules and plaques on the arms, buttocks, thighs, and abdomen; these may ulcerate and result in lipoatrophy. The nodules and plaques usually present in association with the characteristic cutaneous manifestations.

Answer 29

Panniculitis in association with Dermatomyositis

Question 30

Histopathology: mostly lobular panniculitis, or mixed lobular and septal panniculitis, with lymphocytic and plasma cell infiltrates, hyaline sclerosis and fibrosis of septal collagen bundles, progressive replacement of the fat by fibrosis, and calcification (25%).

Answer 30

Panniculitis in association with Dermatomyositis

Question 31

Rare panniculitis that occurs in the first few weeks of life. Lesions are sharply demarcated, erythematous to violaceous, firm, indurated nodules, or plaques located on the back, shoulders, arms, buttocks, thighs, or face, but usually not on the anterior trunk.

Answer 31

Subcutaneous Fat Necrosis of Newborn

Question 32

Histopathology: mostly lobular panniculitis with focal necrosis of the fat lobule and a dense inflammatory infiltrate of lymphocytes, histiocytes, and foreign body giant cells; a few eosinophils may insinuate between the fat cells. Many adipocytes retain their cellular outlines, but contain fine eosinophilic strands and granules as well as needle-shaped clefts in radial array.

Answer 32

Subcutaneous Fat Necrosis of Newborn

Question 33

Presents with circumscribed, red to violaceous, subcutaneous nodules or plaques on the face and thighs, and rarely of the scrotal fat in prepubertal boys. Follows exposure to cold weather, popsicles, ice packs, and swimming in cold ocean water.

Answer 33

Cold Panniculitis

Question 34

Histopathology: lobular panniculitis (lymphohistiocytic or mixed infiltrate) without vasculitis. Perivascular lymphohistiocytic infiltrate involving blood vessels at dermosubcutaneous junction and within overlying dermis.

Answer 34

Cold Panniculitis

Question 35

Reaction in AT induced by external factors, usually injection of foreign materials or any means of trauma. lesions present a spectrum of clinical findings, from papules and nodules to erosions or ulcerations with perfect round or angulated appearance.

Answer 35

Factitial Panniculitis

Question 36

Heterogeneous group of disorders characterized by selective loss of adipose tissue.

Answer 36

Lipodystrophies

Question 37

The extent of fat loss only in small areas

Answer 37

Localized lipodystrophy

Question 38

The extent of fat loss is more extensive fat loss, involving the extremities

Answer 38

Partial lipodystrophy

Question 39

The extent of fat loss involved entire body

Answer 39

Generalized lipodystrophy

Question 40

Most common lipodystrophy, AD

Answer 40

Familial partial lipodystrophy of Dunnigan variety

Question 41

Can be recognized at birth or soon thereafter due to near total loss of body fat, marked muscularity, prominent subcutaneous veins, acromegaloid features, acanthosis nigricans, hepatomegaly, and umbilical prominence or hernia.

Answer 41

Congenital generalized lipodystrophy

Question 42

Autosomal dominant disorder characterized by fat loss from the limbs with variable fat loss from the trunk and increased sc fat deposition in nonlipodystrophic regions. Has normal body fat distribution during early childhood, but around the time of puberty, subcutaneous fat from the extremities and trunk is progressively lost.

Answer 42

Familial Partial Lipodystrophy

Question 43

Have characteristic skeletal abnormalities including hypoplasia of the mandible and clavicles, acroosteolysis, cutaneous atrophy, progeroid features such as thin beaked nose, hair loss, thin skin with prominent superficial vasculature and mottled hyperpigmentation, delayed dentition and closure of cranial sutures, joint stiffness, and lipodystrophy. Those with ZMPSTE24 mutations develop clinical manifestations earlier in life, are premature at birth, and can develop focal segmental glomerulosclerosis and calcified skin nodules.

Answer 43

Mandibuloacral Dysplasia

Question 44

SHORT Syndrome

Answer 44

Short stature
Hyperextensibility or inguinal hernia
Ocular depression
Rieger anomaly
Teething delay

Question 45

Presents as a spectrum of clinical manifestations ranging from onset during the first months of life to later during childhood. Early features are recurrent fevers, annular violaceous plaques, poor weight and height gain, persistent violaceous eyelid swelling, hepatomegaly, arthralgias variable muscle atrophy, and progressive lipodystrophy. Histopathologic examination of lesional skin shows atypical mononuclear infiltrates of myeloid lineage and mature neutrophils, and laboratory abnormalities include chronic anemia, elevated acute-phase reactants, and raised liver enzymes with a cytokine profile showing high levels of IP-1, MCP-1, IL-6, and IL-1Ra, consistent with an IFN signaling signature.

Answer 45

CANDLE syndrome (Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated temperature)

Question 46

The autosomal recessive, syndrome presents with generalized loss of body fat and muscle mass and progeroid appearance at birth.

Answer 46

Neonatal progeroid (Weidemann–Rautenstrauch) syndrome

Question 47

Develops in most patients before age 15. Patients lose subcutaneous fat gradually in a symmetric fashion starting with the face and then spreading downwards. Most of them present with fat loss from the face, neck, upper extremities, and trunk with sparing of sc abdominal fat and lower extremities. Approximately, 20% of the patients develop mesangiocapillary (membranoproliferative) glomerulonephritis, and some develop drusen. No metabolic complications.

Answer 47

Acquired partial lipodystrophy (Barraquer-Simons Syndrome)

Question 48

Present with variable amount of sc fat loss usually during childhood. Although many patients have generalized loss of fat, some areas are spared usually, intra-abdominal or bone marrow fat. However, patients develop extremely severe hepatic steatosis and fibrosis, diabetes, and hypertriglyceridemia.

Answer 48

Acquired generalized lipodystrophy (Lawrence Syndrome)

Question 49

Patients usually lose sc fat from the face, trunk, and extremities 2 years or more after receiving PI-containing HAART. Fat loss from the face can be so severe as to result in an emaciated appearance. Some of them develop buffalo hump, double chin, and also gain intra-abdominal fat. The fat loss progressively gets worse with ongoing HAART therapy and does not reverse on discontinuation of PIs.

Answer 49

Highly Active Antiretroviral Therapy-Induced Lipodystrophy in Human Immunodeficiency Virus-Infected Patients

Question 50

Most common cutaneous lesion seen in patients with lipodystrophy

Answer 50

Acanthosis nigricans

Question 51

Mainstay of treatment of Lipodystrophy

Answer 51

Cosmetic surgery

Management of complications