Thursday, August 3, 2023

Tutorial 20

 Topics covered in this Tutorial

Hypothyroidism in different Age Groups

Macroglossia causes

Amyloidosis

Localised red papules

Hand granulomas

Cutaneous TB

Gardner's syndrome

Bechet's disease

What are some of the features of hypothyroidism at different age groups?

Answer

In infants it causes a big tongue. In younger children it causes big lips and livedo and in juveniles it causes hypertrichosis on the shoulders. In adults you can get punctate telangiectasia on the extensor arms, leg ulcers and carotenemia.
In practical terms you consider hypothyroidism when you have a patient with sudden onset of dry skin, pruritus, weight gain, dry brittle hair and hair loss. They have a typical facies. There is infiltration of tissues with mucopolysaccharides giving the large tongue and carpal tunnel syndrome. Infiltration of the dermis gives non pitting oedema of the hands, feet and eyelids.

The Differential Diagnosis of Macroglossia

Congenital, - Down’s syndrome, Hurler’s syndrome and cretinism.

Tumours-Namely neurofibromatosis, lymphangioma and haemangioma.

Infiltrates--Namely amyloid, lipoid proteinosis and rarely sarcoid.

Angioedema--From superior vena cava obstruction, congestive heart failure or renal oedema.

Hormonal--Due to acromegaly, cretinism

Granulomatous--From the Melkerson Rosenthal syndrome,

Infections-- mainly syphilis, leprosy and Actinomycosis.

Also see Riga Fede disease

Image below is amyloid macroglossia in myeloma


Regarding Amyloidosis

Nodular amyloidosis on the legs is like hypertrophic lichen planus. Primary systemic amyloidosis involves the face, the fingers and the flexures.
View this case of systemic amyloidosis

View these other images of amyloidosis for some other clinical presentations
Nodular amyloidosis is also commonly seen on the scalp as a red brown nodule with central thinning revealing the underlying fat. Some patients have isolated clinical disease while in others it is part of systemic amyloidosis.

Image below is lichen amyloidosis



Vertical lines in the face causing leonine faces can be due to leprosy, mycosis fungoides, Sezary’s syndrome, leukemia, systemic amyloidosis and scleromyxedema.

Question

What is the differential diagnosis of granulomas of the hand?

Answer

They include atypical mycobacterial infection, Leishmaniasis, sporotrichosis and the scarring types of sarcoidosis which tend to be annular and papular. Always check the eyes in early suspected sarcoidosis.

  • Dorsal hand nodules + trauma → foreign‑body granuloma

  • Sporotrichoid pattern → M. marinum or sporotrichosis

  • Palisading necrobiotic granulomas → GA or rheumatoid nodule

  • Non‑caseating granulomas → sarcoidosis

  • Chalky deposits → gouty tophus

  • Bone involvement → LCH

  • Question

    What types of cutaneous tuberculosis do you know?

    Answer

    There is primary inoculation TB, there is tuberculosis cutis verrucosum, there is tuberculosis cutis orificialis, lupus vulgaris, scrofuloderma and miliary TB.

    Id Reactions

    Include papulonecrotic tuberculid, erythema induratum and lichen scrofulosorum.

    Image below is cutaneous TB


    Lichen scrofulosorum

    The differential diagnoses include lichen nitidus (more shiny), lichen spinulosus, keratosis pilaris (keratotic projections- antenna sign), phrynoderma, secondary syphilis, papular sarcoidosis, pityriasis rubra pilaris, and folliculitis. Histopathology is diagnostic and demonstrates superficial granulomas around hair follicles and sweat ducts, with little or no caseation necrosis.


    What is the differential diagnosis of localised red papules?

    Answer

    Generally the diagnosis will come into the categories of embolic, bites , adnexal particularly miliaria and Grover’s disease, folliculitis, vasculitis or drugs. Look out for frictional lichenoid papules on the elbows.

    The image below is Grover's




    Gardner’s Syndrome

    This is an inherited autosomal dominant disorder with large bowel polyps and osteomas. They also develop desmoids, epidermoid and pilar cysts. Osteomas are often in the skull and facial bones. Polyps develop in early childhood or early adult life. 100% subsequently develop malignancy. There can be a need for colectomy and surgery in a case like this. They can also develop fibrosarcomas, dental cysts and have supernumary and un-erupted teeth.

    Compare Gardner’s syndrome to Steatocystoma multiplex and keratinised cysts.

    In Gardner’s the cysts are of early onset. There are multiple different lesions and they have osteomas. Steatocystoma multiplex tends to develop at puberty and may have associated vellus hair cysts. They leak an oily fluid if burst. Keratin cysts such as epidermoid or pilar cysts can develop at any age. They are usually in the scalp.

    The image below is eruptive vellus hair cysts



    In Bechet’s disease ulcers never start as vesicles or bullae. Aphthae when they occur are larger and last longer and note that aphthae do not affect the gingivae.

    Image below is of Sutton's apthous ulcers