CTD

Dermatology Dr. Ahmed Abdulhussein Alhuchami
2020

Connective Tissue diseases:

Are groups of clinico pathological conditions involve connective tissue of most systems of the body, include mainly Lupus Erythematosus LE, scleroderma, systemic sclerosis, dermatomyositis, MCTD &others…

Lupus Erythematosus:

is an autoimmune disorder resulting from an interplay of genetic, environmental &hormonal elements with a heterogeneous clinical expression extending from a localized cutaneous form to a life threatening systemic form.
LE is a spectrum of diseases, in one end of spectrum: DLE is purely cutaneous LE, at the other end :SLE. In the middle of spectrum: sub acute LE, neonatal LE, complement deficiency LE& drug-induced LE .

Exacerbating factors:

1. Ultraviolet light
2. Sex hormones
3. Stress
Creteria for classification of SLE: SLE criteria ( require 4 out of 11):
1.Malar rash :fixed erythema over malar eminences, sparing nasolabial folds.
2.Discoid rash :erythematous raised patches with adherent keratotic scaling &follicular plugging .
3.Photosensitivity :skin rash as a result of unusual reaction to sunlight .
4.Oral ulcer: usually painless.
5.Arthritis:non erosive arthritis involving 2 or more peripheral joints ,characterized by tenderness, swelling or effusion .
6.Serositis:pleurisy or pricarditis.
7.Renal disorder: persistent proteinuria >0.5g/day or cellular casts.
8.Neurological disorder: seizure or psychosis .
9.Hematological disorder: Hemolytic anemia ,leucopenia<4000/mm3, lymphopenia <1500/mm3, or thrombocytopenia less than 100.000/mm3.
10.Immunological disorder: anti-DNA, anti-Sm, antiphospholipid antibodies
11.Abnormal antinuclear Ab titer


Drug-induced SLE:
It is different from idiopathic SLE by presence of anti-histone antibodies instead of ANA
Most commonly implicated drugs:
Procainamide, hydralazine, minocycline, INH, penicillamine and TNF-alpha inhibitors
Diagnosis:
Hx e.g joint pain and swelling, worsening or appearance of skin lesions with sun exposure, weight loss, fever( which indicates systemic involvement), Hx of drugs
Physical exam: scar, follicular plug, dyspigmentation , oral ulcer, pleural or pericardial rub,
Investigations: CBC ( decreased WBC, RBC or platelets), ESR(high in SLE), urinalysis ( cast or protein), ANA (sensitive but not specific)
If ANA is positive go to specific autoantibodies e.g. anti-dsDNA for SLE
Treatment:
Topical: Sun protection, topical and intralesional steroids
Systemic: Antimalarial e.g. hydroxychloroquine, chloroquine

Morphea

Affect female more than male
Does not affect survival but can cause a disability especially the linear type
Fibroblast isolated from morphea lesion produce increased amount of collagen and this is thought to be due to production of IL-4 and TGF-β by T-cells
Some believes that Borrelia plays a role
Clinical types:
1. Plaque-type : present as shiny indurated plaque surrounded by lilac border
2. Deep morphea: invlove deep dermis, subcutis +/- fascia
3. Generalized morphea: plaques coaleasce affecting the entire trunk except nipple, can involves the extremities, it is disabiling and causing difficulty in breathing. Distinguished from systemic sclerosis by :
(a) Absence of Raynaud's phenomenon,
(b) Absence of internal organ involvement and
(c) Asymmetry of involvement
4. Linear morphea: different from plaque morphea by:
(a) Childhood onset,
(b) High ANA titer and
(c) Disabling especially when involve joint or cause atrophy of the whole limb
Variants of linear morphea:
En coup de sabre type (sword hit): linear morphea of head, can involve muscle, bone and rarely brain causing seizures
Parry-Romberg syndrome: hemi facial atrophy including eyes and tongue (the most severe form of linear morphea)


Diagnosis:
Hx and physical examination e.g. hardenening of skin lesion
Investigations e.g. autoantibodies: ANA and anti-ssDNA are commonly seen in linear and generalized types
Biopsy: hyalinized and thick collagen bundles
Treatment:
Topical :
Corticosteroid is ineffective
Vit D analogues e.g. calcipotriol may be of benefit
Systemic :
Glucocorticoids, methotrexate , PUVA (psoralen plus UVA)

Cutaneous manifestations of scleroderma:

1. Hardening of skin (hard to pinch)
2. Microstomia (hard to open mouth) with furrowing around mouth
3. Beaking of nose
4. Loss of facial expression
5. Telangiectasia of skin, lip and tongue
6. Ulcers and necrosis of finger tips
7. Calcinosis cutis (deposition of calcium in skin, subcutaneous tissue and muscle)
8. Nail fold telangiectasia


Thank you and good luck