📚 Question Bank

Diabetic dermopathy is common and is a marker for complications of diabetes such as retinopathy, nephropathy, and neuropathy.

It presents as asymptomatic, oval, dullred papules 0.5–1 cm in diameter evolve slowly on the shins, forearms, thighs, and over bony prominences, producing a superficial scale and, ultimately, atrophic brown scars.

Pathologically, there is hyperpigmentation of the epidermal basal layer, deposition of haemosiderin and melanin in the dermis, and arteriolar basement membrane thickening.

The image given below shows Binkley's spots (diabetic dermopathy).

The given image shows an erythematous rash with ulceration and healing over the shin. This clinical presentation in a diabetic patient is indicative of necrobiosis lipoidica.

Necrobiosis lipoidica diabeticorum is a rare chronic and granulomatous skin disorder that affects only 0.3 of diabetic patients. The average age of onset is 30 years, with females being affected more commonly. Its postulated etiology is microangiopathy.

Most rashes are located on the legs, especially above the tibiae. It may present as red papules that enlarge to form patches or plaques with an atrophic yellowish-brown and slightly depressed center.The lesions may resolve spontaneously or become persistent chronic lesions, that can ulcerate.

Option A: Granuloma annular is most commonly found in children and females lt;30 years. It manifests as groups of 1-2mm papules that range from skin colored to violaceous, in an annular arrangement over distal extremities. There is no confirmed association with diabetes.

Option C: Reactive perforating collagenosis (folliculitis) has been reported in patients with diabetes. It is attributed to collagen glycation.

Option D: Rubeosis is a peculiar rosy reddening of the face, and sometimes hands and feet. In longstanding diabetes, it is attributed to microangiopathy or decreased vascular tone.

The image shows Binkley's spots or diabetic shin spots which is another name for diabetic dermopathy. The phrase diabetic dermopathy was coined by Binkley in 1965.

It is characterized by asymptomatic, oval, dullred papules 0.5–1 cm in diameter evolve slowly on the shins, forearms, thighs, and over bony prominences, producing a superficial scale

and atrophic brown scars.

Lupus pernio (cutaneous sarcoidosis) is the most likely diagnosis.

Cutaneous sarcoidosis or presents as redbrown or red violaceous nodules or plaques, generally multiple. It is usually asymptomatic. Diascopy reveals the subtle brownyellow or ‘apple jelly’ color characteristic of granulomatous diseases but usually more opaque than in lupus vulgaris. At diagnosis, at least 60 of patients have an increased serum angiotensinconverting enzyme level.

Lupus pernio is the most distinctive manifestation of cutaneous sarcoidosis. Infiltrated erythematoviolaceous plaques involve the nose, cheeks, ears, lips, forehead, and fingers. On the cheeks, a prominent telangiectatic component is characteristic. The clinical appearance is due to the presence of epithelioid cell granulomas in the dermis.

Lupus pernio is usually painless and, as it does not tend to ulcerate, is not as mutilating as lupus vulgaris.In more than half of cases, lupus pernio is associated with sarcoidosis of the upper respiratory tract, especially in patients with involvement of the nasal rims.

It is also frequently associated with pulmonary fibrosis, chronic uveitis, and bony cysts, particularly affecting the terminal phalanges.

The image given shows erythematous shiny plaques over the shin, indicative of erythema nodosum. The characteristic triad association of erythema nodosum with bilateral hilar and right paratracheal adenopathies, with or without pulmonary infiltrates, and polyarthritis is known as Löfgren syndrome. (it is a type of acute sarcoidosis).

Arthritis in sarcoidosis is usually symmetrical; the ankles (gt;90 cases), knees, small joints of the hands or feet, wrists, and elbows are involved.

Erythema nodosum is the most common nonspecific lesion of sarcoidosis and the first manifestation of the disease but may not be present in all patients. It may be accompanied by fever, polyarthralgia, and uveitis.

Inflammatory markers are elevated in gt;80 of patients with acute sarcoid polyarthritis. Serum ACE level is usually increased, and rheumatoid factor is negative.

It usually resolves spontaneously. Some require treatment is with oral corticosteroids. The prognosis is very good.

Option A: Heerfordt Syndrome is a rare presentation of sarcoidosis and is characterized by the presence of parotid gland enlargement, facial palsy, anterior uveitis, and fever.

Option B: Tuberculosis generally does not show bilateral hilar lymphadenopathy.

Option D: Rheumatoid arthritis also presents with polyarthralgia and erythema nodosum. Rheumatoid factor is often positive without chest involvement.

The given image shows keratoderma or thickening of the soles. When this condition is present in both palms and soles, it is called palmoplantar keratoderma or tylosis. It is associated with a very high lifetime risk of developing squamous cell carcinoma of the esophagus.

Pressure points of the sole are predominantly affected, and the palms, less affected.

Tylosis esophageal cancer syndrome, also called as Howel-Evans syndrome is inherited as an autosomal dominant trait with complete penetrance of the cutaneous features.

The cutaneous features usually present by 7 to 8 years of age but can present as late as puberty. Esophageal cancer associated with tylosis usually presents in middle to late life (from mid-fifties onwards) and shows no earlier development than the sporadic form of the disease.

The condition in the given image is acanthosis nigricans. It is characterized by thickened, gray-brown velvety plaques seen in flexural areas such as the back of the neck, axillae, inframammary creases, waist, and groin.

The causal mechanism is due to the secretion of epidermal growth factors in the region involved. It can be benign or malignancy-associated.

• Benign - commonly associated with obesity or insulin resistance, usually mild.
• Malignancy - much less common, associated with gastric, lung, and uterine cancers.
• Acanthosis nigricans can also be seen in the following:
• Drugs such as nicotinic acid used to treat hyperlipidemia
• Sudden onset of multiple seborrhoeic keratoses

This image shows velvety warty hyperpigmentation on the front of the neck called acanthosis nigricans. By far, the commonest site of underlying neoplasm is the gastrointestinal tract (70–90), and gastric adenocarcinoma is the most frequent.

Production by tumor cells of either transforming growth factor ° (TGF-° ) or cytokines that activate insulinlike growth factors or their cutaneous receptors has been proposed as the pathogenetic mechanism.

The image shows enhanced dermatoglyphics with thickened velvety palms that have the appearance of tripe, the stomach lining of beef. Tripe palms or acanthosis palmaris occurring alone is more often associated with bronchial carcinoma.

Other sites of tumors are:

• Genitourinary tract
• Carcinoma breast

Acanthosis nigricans or sign of Leser-Trelat is most commonly associated with gastric carcinoma

The appearance or exacerbation of tripe palms in a known cancer patient may be a sign of recurrence of the malignancy.

The image below shows tripe palms.

Erythema gyratum repens is commonly associated with lung cancer.

Necrolytic migratory erythema is commonly associated with glucagon secreting pancreatic islet cell adenoma.

The image given below shows erythema gyratum repens of the arm secondary to carcinoma of the bronchus.

The given image shows multiple eruptive seborrheic keratoses over the back, abdomen, and neck. This acute onset of an increase in size and number of seborrheic keratoses is a sign of internal malignancy called the sign of Leser-Trélat.

When it is associated with pruritus or acanthosis nigricans, especially in younger patients, it warrants investigation for internal malignancy.

About 1/3rd of associated tumors are in the gastrointestinal tract, and about half of these tumors are adenocarcinomas.

Leukemia cutis is most common in patients with myeloid neoplasms, especially acute myeloid leukemia (AML). The risk is higher with translocation t(8,21).

Leukemia cutis is an infiltration of the skin by myeloid or lymphoid neoplastic leukocytes resulting in clinically identifiable cutaneous lesions. When made up of malignant granulocytic precursor cells, leukemia cutis lesions are also called myeloid sarcoma, granulocytic sarcoma, or chloroma.

Leukemia cutis typically presents as single or multiple monomorphic violaceous, darkred or hemorrhagic skin nodules and plaques, especially on the legs, arms, and face.

The image below shows violaceous infiltrated nodules and plaques on the trunk suggestive of leukemia cutis.

The given scenario is suggestive of pyoderma gangrenosum (PG). It is an autoinflammatory disease (excessive response to an internal antigen) due to neutrophil dysfunction and hence antibiotics have no role in its management.

It commonly presents on the anterior aspect of the lower limbs. The disease starts as a papule/pustule/bulla and rapidly progresses to an ulcerative lesion. The undermined edges of the ulcer show neutrophilic infiltration. Deep ulcers heal with a characteristic cribriform or criss-cross pattern of scarring.

The pustular form of PG is associated mainly with inflammatory bowel disease (ulcerative colitis

gt; Crohn's disease), the bullous form with hematological malignancies.

Pathergy is the phenomenon whereby skin trauma provokes lesions at the site of injury. It is seen in pyoderma gangrenosum, Behcet's disease.

The image below shows a large purulent ulcer with violaceous undermined borders which is a feature of pyoderma gangrenosum.

Neutrophilic infiltration of the dermis indicates one of the diseases under neutrophilic dermatosis. Sweet's Syndrome presents as an acute febrile neutrophilic dermatosis which is characterized by nonitchy, sometimes tender, erythematous plaques and papules.

The plaques are often oedematous and as the process develops they may become studded with pseudovesicles or pseudopustules (no fluid collection present).

The lesions in Sweet's Syndrome are most commonly distributed on the arms, upper body, head, and neck. An associated fever and peripheral leucocytosis are commonly seen and females are more frequently affected.

Histological findings include a dense dermal neutrophilic infiltrate with edema. Three subgroups are recognized:

• Classical
• Malignancy-associated
• Drug-induced

Option A: Behcet's syndrome is also a neutrophilic dermatosis but presents as recurrent oral and genital aphthous ulcers with an erythematous halo.

Option B: Pyoderma gangrenosum, another neutrophilic dermatosis presents as painful ulcerative erythematous plaques or papules with undermined violaceous borders.

Option C: Kasabach-Merritt's syndrome shows hemangiomas with thrombocytopenia.

The image given below shows Sweet syndrome- pseudovesicles may occur within the inflammatory plaques.

The given clinical scenario is suggestive of hypothyroidism. Palmar erythema is seen in hyperthyroidism.

Graves disease may also cause thyroid dermopathy resulting in pretibial myxoedema and exophthalmos, which are associated with the presence of thyroid antibodies.

Hypothyroidism

Cold peripheries with pale an ddry coarse skin

Aurantiasis cutis,yellowish ti nged skin secondary to beta-c arotene accumulation

Sparse and brittle hair

Sign of Hertoghe -loss of hair in the outer third of eyebrow s

Myxoedema - puffy face, eyel ids, hands, and feet

Delayed wound healing

Hyperthyroidism

Smooth, moist, and warm ski n

Facial andpalmar erythema

Fine, soft, and thinned scalp hair

Distorted and overgrown nail s (thyroid acropachy) that ma y lift off the nail bed (onychol ysis)

Generalized pruritis Urticaria

Localized (trunk, head) or generalized pruritus occurs in 90 of patients with endstage kidney disease due to irritation of free nerve endings caused by uremia.

Other cutaneous manifestations of end-stage renal disease are:

• Xerosis
• Anemic pallor
• Hyperpigmentation
• 'Half and half' nails
• Pseudoporphyria (following hemodialysis)
• Nephrogenic fibrosing dermopathy
• Calciphylaxis

The image given below shows calciphylaxis- also known as calcific uremic arteriolopathy (CUA) or “Grey Scale”, is a rare painful syndrome of calcification of the small blood vessels located within the fatty tissue and deeper layers of the skin, blood clots, and the death of skin cells due to too little flow.

The most likely cause of the above picture is COVID-19, an entity called COVID toes.

Pernio (chilblains) present as erythematous-violaceous or purpuric macules on fingers, elbows, toes, and lateral aspect of the feet, with or without accompanying edema and pruritus. The underlying mechanism is thought to be an inflammatory process. They can be:

• Idiopathic, due to exposure to cold that present within 12-24 hours.
• Secondary to hematological disorder (paraproteinemia), autoimmune disease, viral hepatitis or malignancy.

The latest guidelines suggest patients presenting with new-onset, pernio-like lesions that have no other clear cause should be tested for severe acute respiratory syndrome coronavirus 2

(SARS-CoV-2) by polymerase chain reaction (PCR). There are no treatment guidelines for COVID-19-associated, pernio-like lesions of the feet or hands. However, high-potency topical corticosteroids may be helpful if the lesions are causing discomfort.

The finding seen in the case scenario provided is seborrheic keratosis and this is highly suggestive of visceral malignancy.

A sudden appearance of multiple seborrheic keratoses in association with skin tags and acanthosis nigricans has been associated with a variety of malignancies, including gastrointestinal and lung cancers; it is also known as the Leser-Trélat sign.

Seborrheic keratoses are common epidermal tumors consisting of a benign proliferation of immature keratinocytes. They present as well-demarcated, round or oval lesions with a dull, verrucous surface and a typical stuck-on appearance.

The image above along with positive serology for syphilis suggests a diagnosis of anogenital warts or condylomata. It is seen in the stage of secondary syphilis.

Syphilis is caused by Treponema pallidum. It is usually acquired through sexual contact and co-exists with other sexually transmitted diseases except for congenital syphilis.

Primary syphilis commonly presents as a papule on external genitalia that rapidly ulcerates to form a primary chancre.

Secondary syphilis is the stage when generalized manifestations of syphilis appear on the skin and mucous membranes. Serological tests in immunocompetent patients are always positive.

Tertiary syphilis commonly presents as superficial or nodular punched out ulcers or gummas(granulomas appearing as cutaneous plaques or nodules with central ulceration)

Late syphilis is the stage when there are no clinical stigmata of active disease but serological testing for the disease is positive

Clinical features:

• Primary genital sores appear 3 weeks after exposure
• Enlarged inguinal lymph nodes are palpable 5 weeks after exposure
• Macular rash appears 8 weeks after exposure
• Condylomata appears 6 months after exposure

Serological testing is done using a standard non-treponemal antibody test (VDRL, RPR) and a specific treponemal antibody test(TPHA, FTA-ABS).

Microscopically, Treponema pallidum is identified with a characteristic wave-like appearance on darkfield microscopy.

Parenteral penicillin G is the drug of choice for all stages of syphilis.

If the patient has a history of drug allergies, it is advisable to keep the patient under observation for 15 to 20 mins after injection. An emergency kit for the management of anaphylaxis should always be available.

Treatment of recent sexual contacts is recommended.

The given clinical scenario is suggestive of secondary syphilis. The absence of chancre is suggestive of a rare form of syphilis called syphilis d'emblee, where syphilis is transmitted through blood transfusion.

In this condition, there are no primary lesions and the disease directly presents in the secondary stage. It is treated as per usual guidelines with benzathine penicillin G 2.4 mega units IM single dose or 2 doses on days 1 and 8.

Option A: Sexual contact would present initially with a chancre at the primary site of contact.

Option C and D: Endemic treponemal infections such as yaws can be transmitted by direct skin-to-skin contact. It causes skin and bone deformities. Painless, indurated ulcers are seen, which are common over the legs and ankles.

Pseudochancre redux is seen in tertiary syphilis and is not infectious. It must not be confused with chancre redux.

Pseudochancre redux Tertiary syphilis

Gummatous lesion at the site of a previously healed chanc re

No lymphadenopathy Not infectious

Chancre redux Primary syphilis

Recurring chancre at the sam e site in the first 2 years. Also calledrelapsing syphilis

Painless lymphadenopathy Highly infectious

A positive VDRL test with the above rash indicates secondary syphilis in this woman. Syphilitic rhinitis, generally described as ‘snuffles’, is often the earliest and most frequent sign of early

congenital syphilis.

It manifests as a profuse, serous, nasal discharge that contains a high concentration of Treponema pallidum. In later stages, saddle nose may occur due to bone and cartilage destruction in the nose.

Congenital syphilis occurs due to transplacental spread from an infected mother or through intrapartum spread through maternal genital lesions. The risk is highest during primary and secondary stages of the disease. If left untreated, it can lead to fetal death or congenital syphilis in a live birth.

Congenital syphilis can present early or late. The early disease presents lt; 2 years and late disease presents gt; 3 years.

Elevated RPR titer of the cord blood is indicative of congenital syphilis in the infant. Seventh nerve palsy is not a feature of congenital syphilis. It is associated with 8th nerve deafness, along with tinnitus and vertigo. It is a part of Hutchinson's triad which includes the following:

• Interstitial keratitis
• Eighth nerve deafness
• Hutchinson's teeth

One of the features of congenital syphilis is periostitis of the skull which results in parrot's nodes or hot-cross bun appearance.

Most newborns with congenital syphilis are asymptomatic at birth. However, a vesiculobullous rash called syphilitic pemphigus may be present at delivery. After 2 weeks, a maculopapular rash is more common. Clinical signs usually appear between weeks 3 to 8 of life, usually within 3 months.

The X-ray shows Wimberger's sign, which is the localized bilateral metaphyseal destruction of the medial proximal tibia. It is seen in early congenital syphilis.

Option A: Clutton's joints refer to painless symmetrical swelling of the knees. It is seen in late congenital syphilis.

Option B: Higoumenaki's sign is the unilateral enlargement of the sternal end of the clavicle, seen in late congenital syphilis.

Option D: Wimberger ring sign refers to a circular calcification surrounding the osteoporotic epiphyseal center of ossification in scurvy, which may result from bleeding.

Anti-treponemal IgG is not useful for the initial diagnosis of congenital syphilis. The given image shows Hutchinson's teeth, which is a deformity of the upper, central incisor teeth with a notched or pegged appearance. It is a presentation of late congenital syphilis.

The following investigations must be done in children born to seropositive mothers with no documented treatment at least 4 weeks before delivery.

• Examination for stigmata of congenital syphilis
• Xray of long bones for evidence of periostitis
• CSF examination (VDRL test) to rule out neurosyphilis
• Darkfield microscopy and/or PCR from exudates of suspicious lesions or fluids.
• Serology - Total serum treponemal antibody titer that is gt; 4 times the mother’s, or if specific
• IgM treponemal antibody tests are positive.