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The image shows a psoriasiform papulosquamous lesion on the sun-exposed area(back). Along with the history and the positive lupus band test only in the involved skin, a diagnosis of subacute cutaneous lupus erythematosus can be made. Antibodies to the Ro/SSA antigen are almost universally present in this subgroup.

Subacute cutaneous lupus erythematosus is a specific ‘subset’ of lupus in which patients exhibit mainly cutaneous disease and usually have a good prognosis.

The lupus band test (LBT) is a direct immunofluorescence technique for demonstrating a band of localized immunoglobulins at the dermal-epidermal junction in the skin of patients with lupus erythematosus (LE).

The given clinical details are suggestive of systemic lupus erythematosus (SLE). Salmon rash is seen in adult-onset of Still's disease. It is a rare systemic inflammatory disease characterized by the classic triad of:

• Persistent high spiking fever
• Joint pain
• Salmon rash

SLE is more common in females (7:1 to 15:1). It is associated with classical erythematous rash, typically sparing the nasolabial folds called malar/butterfly rash.

Discoid rashes resembling discoid lupus erythematosus may also be seen. Clinical photosensitivity in the form of polymorphous light reaction and pruritus is associated with more severe systemic disease.

The image given below shows a malar rash.

The given clinical details and histopathology are suggestive of systemic lupus erythematosus (SLE). AntiSmith (antiSm) antibodies are present in only approximately 30 of patients with SLE, but are considered highly specific for the disease and are included in the ACR criteria.

Most sensitive antibody: ANA

In the image below, the glomerulus is hypercellular (endocapillary proliferation). Glomerular capillary loops appear circumferentially thickened, forming a "wire loop" lesion, which

is classically seen in class IV or diffuse proliferative lupus nephritis.

AntiSmith (antiSm) antibodies are present in approximately 30 of patients with SLE, but are considered highly specific for the disease and are included in the ACR criteria, however, they are not of use in monitoring overall lupus disease activity. Anti-ds DNA levels often correlate with disease activity.

The image shows the characteristic malar rash.

AntiRo antibody occurs in approximately 30–40 of patients, who have an increased tendency to photosensitivity, secondary Sjögren syndrome, interstitial pneumonitis, shrinking lung syndrome, or deforming arthropathy, as well as being a marker for neonatal lupus.

Rim pattern of ANA positivity is specific for SLE.

The most common finding in neonatal lupus erythematosus (NLE) is an erythematous, slightly scaly eruption on the face and periorbital skin (raccoon sign/owl eye/eye mask).

NLE is a wellrecognized subtype of lupus erythematosus, thought to be caused by the transplacental passage of maternal antibodies.

The most frequent clinical manifestations are cutaneous lesions and congenital heart block (CHB). Approximately 50 of infants manifest skin lesions, which may be present at birth or occur in the first few weeks of life. The eruption can be exacerbated by UV exposure.

The rash improves over the first few months of life and has usually resolved without scarring by 12 months of age. Skin disease is often mild and often requires no treatment.

The image given below shows typical raccoon eyelid lesions in neonatal lupus erythematosus.

The above clinical scenario is suggestive of dermatomyositis. Muscle tenderness is not common in this disorder.

It is an autoimmune disorder affecting, predominantly, the skin and skeletal muscle and usually presents initially with a facial rash especially around eyelids which can be pruritic and

sore. Myositis symptoms are usually weakness and fatigue especially while climbing stairs, raising arms, and standing from sitting due to proximal muscle weakness.

The presence of dysphagia points to a poorer prognosis. The clinical signs seen in dermatomyositis are as follows:

• Heliotrope rash around eyelids with a lilac hue.
• Gottron papules - flat-topped lichenoid eruption on the proximal and distal interphalangeal joint
• Shawl sign or V sign - fixed erythematous macular erythema)
• Nail fold changes with periungal erythema with visible dilated capillary loops in the proximal nail fold
• Proximal myopathy
• Poikiloderma atrophicans vasculare
• Calcinosis cutis

Approximately 20 to 40 of patients have an underlying malignancy (eg, lung, ovarian, breast) and hence screening should be done at the time of diagnosis.

The image below shows ragged cuticle and periungueal erythema in a patient with dermatomyositis.

The image shows a maculopapular rash on the lateral thigh which is also known as the holster sign, seen in dermatomyositis.

The buttocks are often involved and confluent macular violaceous erythema over the hips and lateral thighs has been termed the ‘holster sign’ corresponding to the site of hanging a holster.

The image shows pathognomonic involvement of eyelids in dermatomyositis in which the upper eyelids are preferentially affected with erythema that is lilac in color called heliotrope rash. This is associated with anti-Mi-2 antibodies.

Gottron’s papule, heliotrope rash, and shawl sign are associated with anti-Mi2 antibodies.

Mechanic hand, Myositis, and ILD (Interstitial Lung Disease) are associated with antihistidyl tRNA synthetase (anti-Jo-1) antibodies.

The image shows a confluent macular violaceous erythema called the V sign seen in patients of dermatomyositis.

It is an autoimmune disorder affecting, predominantly, the skin and skeletal muscle and usually presents initially with a facial rash, especially around eyelids, which can be pruritic and

sore. Myositis symptoms are usually weakness and fatigue especially while climbing stairs, raising arms, and standing from sitting due to proximal muscle weakness.

The presence of dysphagia points to a poorer prognosis. The clinical signs seen in dermatomyositis are as follows:

• Heliotrope rash around eyelids with a lilac hue.
• Gottron papules - flat-topped lichenoid eruption on the proximal and distal interphalangeal joint
• Shawl sign or V sign - fixed erythematous macular erythema
• Nail fold changes with periungual erythema with visible dilated capillary loops in the proximal nail fold
• Proximal myopathy
• Poikiloderma atrophicans vasculare
• Calcinosis cutis

The given clinical scenario featuring Raynaud's phenomenon, arthritis, GERD, and calcinosis cutis is suggestive of systemic sclerosis. Mat-like telangiectases on the face and upper trunk and limbs,

and cutaneous calcinosis, most often on the fingers over joints and at pressure points, develop in the later stages, particularly in the limited systemic form of systemic sclerosis, or CREST syndrome.

Image A is a classical fish mouth appearance (microstomia). Image B shows the nucleolar pattern of IF(some spots of fluorescence within the nucleus and it represents antibodies to RNA). This is seen in systemic sclerosis.

The majority of cases are adult-onset with Raynaud's phenomenon as the first symptom.

The earliest cutaneous manifestations are nonpitting edema and puffiness, which tend to be seen first in the fingers, hands, and face. The skin becomes taut, indurated, thickened, and then fixed to deeper structures on the fingers resulting in sclerodactyly.

Pruritus is described in 40 of patients and may be intense during the early or active stages of the disease. It correlates with the extent of skin sclerosis and gastrointestinal symptoms.

Anti-scl-70 positivity in this disease is useful in predicting those at higher risk for interstitial lung disease. Anti centromere antibody is most specific for CREST syndrome.

The image shows a young woman with mask-like facies with stretched, shiny skin, and loss of normal facial lines, microstomia, and pitted scarring on the right index finger which is suggestive of scleroderma or systemic sclerosis, an autoimmune rheumatic disease in which there is a 4: 1 female predominance.

Cutaneous features:

• Hands and feet - Raynaud's phenomenon, non-pitting edema, painful ulcerations which heal with pitted scars, sclerodactyly, contractures, loss of sweat glands, bone resorption.
• Face - periorbital edema, mask-like facies, telangiectasia, and diffuse hyperpigmentation
• Trunk - firm, shiny, and thickened skin

The extent of skin sclerosis in systemic sclerosis can be measured, most commonly employing a modified Rodnan skin score (MRSS).

Systemic features:

• GIT- dysphagia, GERD, diminished peristalsis, malabsorption
• Lungs- pulmonary fibrosis and alveolitis
• Heart- cardiac conduction defects, heart failure
• Kidney- uremia, malignant hypertension
• Musculoskeletal- weakness, carpal tunnel syndrome

The image below shows typical facial features: note the expressionless facies, matlike telangiectases, microstomia, perioral furrowing, and beaklike nose.

The image below shows digital ulceration

The image below shows advancedstage sclerodactyly with contractures and vasculopathic ulcers over the bony prominences

In systemic sclerosis, progressive loss of fingerprints is seen in patients. These altered fingerprint ridges are seen due to changes in skin elasticity, hardness, and thickness and eventually make a patient with scleroderma a “fingerprintless person”.

Autoantibody patterns seen in systemic sclerosis:

• Reactivity to autoantibodies such as anticentromere (ACA) is commonly seen in limited skin sclerosis.
• Reactivity to autoantibodies such as antitopoisomerase 1 antibody (antiScl70 or ATA), and antiRNA polymerase III antibody (ARA or RNAP) is commonly seen in SSc.

The given image shows nail-fold capillaroscopy findings. The image shows giant capillaries, hemorrhages, and reducing capillary density suggesting an active pattern in systemic sclerosis.

The most important tests for systemic sclerosis are ANA reactivity and nail fold capillaroscopy. Investigations to exclude or confirm other autoimmune rheumatic diseases including arthritis or other forms of connective tissue disease are also done.

Nail-fold capillaroscopy is a non-invasive, easy, and safe diagnostic technique designed to evaluate the general architecture of capillary rows and fine details of small vessels of the microcirculation in the nail fold.

The most important indications for performing capillaroscopy include differential diagnosis of primary and secondary Raynaud's phenomenon, as well as the assessment of scleroderma spectrum disorders.

In systemic sclerosis, capillary abnormalities appear and evolve in a clearly defined sequence called the scleroderma pattern, which correlates with internal organ involvement.

Nail fold capillaroscopy is also listed as a systemic sclerosis classification criterion recognized by the European League Against Rheumatism (EULAR).

The history along with the image showing calcinosis cutis and sclerodactyly are suggestive of CREST syndrome.

In limited cutaneous systemic sclerosis or CREST syndrome, anti-centromere antibodies are the most commonly found antibodies.

All other options are true for diffuse cutaneous systemic sclerosis, not CREST syndrome.

Autoantibodies such as ANA, anti-histone, and antissDNA may be present in morphea, but the systemic sclerosis specific autoantibodies such as anti-topoisomerase, anti-centromere and antiRNA polymerase are rarely found.

Morphoea, a term used in preference to ‘localized scleroderma’, encompasses a group of related conditions characterized by varying degrees of sclerosis, fibrosis, and atrophy in the skin and subcutaneous tissues.

It is distinguished from systemic sclerosis by the absence of sclerodactyly and nail-fold capillary changes.

Overall, plaque morphea is the commonest subtype.

Morphoea en coup de sabre: Blaschkoid linear induration affecting the face and scalp, may involve underlying muscle, bone, eye, and the brain.

Salt-and-pepper appearance is characterized by the presence of patchy hypopigmentation and hyperpigmentation of the skin manifesting as depigmentation with perifollicular pigmentary retention. This along with lung fibrosis is seen in systemic sclerosis.

This appearance may also be seen in areas of repigmentation in lesions of vitiligo.

The image below shows salt and pepper appearance of skin over the abdomen seen in systemic sclerosis.

The above image shows multiple plaque-like lesions coalescing with each other which is suggestive of Kaposi sarcoma. All forms of Kaposi sarcoma are associated with HHV 8 infection as an aetiological agent.

It is a multifocal, endothelial proliferation most often with cutaneous involvement and with or without visceral extension. This virus is transmitted through saliva and blood products.

It has a low-grade malignant potential. The most important cofactor associated is HIV co-infection which increases the risk up to 20,000 fold.

The above image showing purple-colored plaques on the ala and tip of the nose in an immunocompromised patient is suggestive of Kaposi sarcoma. It is mostly seen in extremities, commonly on feet and occasionally on hands, ears, and nose, and has

a low-grade malignant potential.

Skin lesions are usually purple and multifocal which eventually evolve into plaques and tumors. Lymph nodes, mucosae, and viscera may be involved as the disease progresses, although this can occur without skin involvement.

The most common type of primary cutaneous T cell lymphoma is Mycosis fungoides.

Option A: Sézary syndrome may develop either ab initio or rarely as a progression from classic mycosis fungoides. It is a severe form of cutaneous T cell lymphoma with a poor prognosis.

Option B: Follicular mucinosis is a type of cutaneous T cell lymphoma that consists of boggy cutaneous plaques showing follicular prominence and histological evidence of mucinous degeneration of the hair follicles.

Option C: Pagetoid reticulosis is a localized, solitary variant of cutaneous T cell lymphoma, which histologically shows intense epidermotropism. It is also called Woringer–Kolopp disease.

Note: Mycosis fungoides is derived from skinresident effector memory T cells, whereas Sézary syndrome is derived from central memory T cells.

Seborrhoeic keratosis is not a part of the clinical triad of Sézary syndrome The clinical triad of Sézary syndrome consists of the following:

• Erythroderma - As a result of diffuse infiltration of the skin by neoplastic cells
• Peripheral lymphadenopathy
• Atypical mononuclear cells (Sézary cells) comprising more than 20 of total lymphocyte count or total Sézary count of more than 1000 × 109/L

The given clinical features along with the characteristic investigation of T-cell receptor analysis showing clonal rearrangement of the T-cell receptor (TCR) genes is diagnostic of Mycosis fungoides.

Mycosis fungoides is characterized by erythematous, polymorphic patches and plaques which

are fine, scaly, and slightly atrophic (wrinkled) usually involving limb/girdle, breast, and especially the buttock area.

Analysis of TCR genes is now a standard approach that has diagnostic, prognostic, and therapeutic implications. It consists of the analysis of DNA from tissue samples for the detection of clonal rearrangements of the TCR genes as a marker of a monoclonal Tcell population. Tcell clones can be detected in a proportion (70 overall) of skin biopsies from patients with earlystage disease and are almost invariable in patients with later stages of the disease.

Option A: Follicular mucinosis consists of boggy cutaneous plaques showing follicular prominence and histological evidence of mucinous degeneration of the hair follicles. It is often associated with an atypical pilotropic Tcell infiltrate.

Option B: Bowen's Disease is a type of squamous cell carcinoma in situ that presents as an erythematous scaly plaque.

Option C: Psoriasis is a papulosquamous disorder presenting with erythematous plaques with silver coloured scales.

Note: Follicular mucinosis is the only other cutaneous T-cell lymphoma given in the options.

The image shows a characteristic cerebriform appearance of T-lymphocytes as seen in Mycosis fungoides.

It is a type of cutaneous T cell lymphoma with an indolent (slowly progressive) clinical course. Most accepted theory is that human Tcell leukemia virus 1 (HTLV-1) is the causative organism.

Histopathology findings:

• Moderate lymphocytic infiltrate in the papillary dermis.
• Epidermotropism - presence of lymphocytes in the epidermal cells.
• Pautriers’s microabscesses consisting of clusters of atypical lymphocytes.
• Atypical lymphocytes have a cerebriform appearance with an irregular nuclear outline and halo around cells.

Note - Cerebriform appearance is also seen in Sézary syndrome.

Extracorporeal photopheresis is a systemic therapy used for mycosis fungoides. The procedure consists of three stages:

• Leukapheresis
• Photoactivation (with psoralen and UVA)
• Reinfusion of buffy coat

After exposure to UVA, psoralen binds covalently to DNA in separated leukocytes leading to cell cycle arrest and apoptosis. These apoptotic leukocytes when reintroduced into the peripheral circulation are phagocytosed by antigen-presenting cells (APCs). These APCs then produce tumor suppressor cells against the malignant cells responsible for mycosis fungoides.

Skin-directed therapy is preferred in the early stages whereas advanced stages require systemic therapy.

Skin-directed therapies:

• Topical corticosteroids
• Topical chemotherapy: nitrogen mustard (mechlorethamine) and carmustine (BCNU)
• Topical rexinoids
• Phototherapy and photochemotherapy (PUVA)
• Radiotherapy and electron beam therapy: Mycosis fungoides and variants are highly radiosensitive

Systemic therapies:

• Immunotherapy: Pegylated IFN°
• Oral Retinoids and rexinoids
• Combination therapy
• Systemic chemotherapy
• Extracorporeal photopheresis (ECP)
• Toxin therapies: Denileukin diftitox
• Monoclonal antibody therapy
• Histone deacetylase inhibitors.

The above image shows an eczematous ring surrounding a melanocytic naevus (birthmark), suggestive of halo dermatitis.

It is also known as Meyerson nevus. Histology shows a benign naevus surrounded by a dermal lymphocytic and eosinophilic infiltrate, with acanthosis, spongiosis, and parakeratosis.

The condition usually resolves spontaneously within few months without involution of the nevus.

The above image showing dry, scaly eczematous lesions with a characteristic crazy-paving pattern (eczéma craquelé) is suggestive of asteatotic eczema. It usually develops in the elderly, not in children.

It affects the legs, arms, and hands of elderly people in the context of very dry skin. The condition is usually chronic, relapsing each winter and clearing in the summer, but eventually becoming permanent.

It may be a presenting sign of myxoedema. It can also be due to zinc deficiency. Drugs implicated include:

• Diuretics
• Cimetidine
• Topical corticosteroids

The above image is suggestive of erythroderma which is an inflammatory skin disease affecting

gt;90 of body surface.

The most common causes of erythroderma in decreasing order of frequency are:

• Eczema (40)
• Psoriasis
• Lymphoma and leukemias
• Drugs - phenylbutazone, phenytoin, carbamazepine, gold salts, lithium, cimetidine

Note: If the option was between atopic dermatitis and psoriasis always choose psoriasis because the latter is the single most common cause of erythroderma whereas atopic dermatitis is one of the subtypes of eczema.