Acne Related Syndromes

-Dr.Gurjot Singh MarwahJunior resident

(Dept. Of Dermatology)M.G.M hospital

1)SAPHO SYNDROME

• S : SYNOVITIS

• A : ACNE

• P :PUSTULOSIS

• H : HYPEROSTOSIS

• O : OSTEITIS

CLINICAL PRESENTATION Sudden onset haemorrhagic acne on face and chest ,palmoplantarpustulosis and pain over chest wall

ETIOPATHOGENSIS

Unknow, it is postulated to be an autoimmune reaction to some skin or bacterial antigen

• SKIN LESIONS : Palmoplantar Pustulosis

Plaque Psoriasis

Pustular Psoriasis

Acne Conglobata

Acne Fulminans

Hidradenitis Suppurativa

Inclusion Criteria For Sapho Syndrome

• Osteoarticular Syndromes Associated With Acne

• Osteoarticular Syndromes Associated With Palmoplantar Psoiasis

• CHRONIC MULTIFOCAL Osteomyelitis(with Or Without Dermatosis)

• Hyperostosis Of Anterior Chest (With Or Without Dermatosis)

TREATMENT

• Mainly Towards The Symptoms Involved

• NSAID’s ,Sulfasalazine

• Biologicals: Infliximab ( Few Case Reports)

2)PAPA Syndrome

• PA : PYOGENIC ARTHERITIS

• P : PYODERMA GANGRENOSUM

• A: ACNE ( CONGLOBATA TYPE )

• PATHOGENESIS : Autoinflammatory disorder resulting from mutation in CD2 BINDING PROTEIN 1 GENE and increased INTERLEUKIN -1beta

• OTHER CLINICAL FEATURES : Sterile cutaneous abcess ,inflammatory bowel disease , psoriasis and pancytopenia

TREATMENT : Reports of successful treatment with infliximab and anakinra

3)ENDOCRINE ACNE

• Although endocrine system plays a pivotal role in pathogenesis of acne

• The term “endocrine acne” is reserved for acne associated with clear signs and symptoms of endocrine disease Eg. A)PCOS

B)Cushing’s disease

C)Congenital androgenital

syndrome

D) SAHA syndrome

Pathogenesis of pcos1) An alteration in

gonadotropin releasing hormone resulting in increased LH secretion

2) Alteration in insulin secreation and insulin action results in hyperinsulinemia and insulin resistance

3) Defect in androgen synthesis resulting in increased androgen secretion

PCOS

Clinical features : irregular menstrual cycle, obesity , hirsturism and acne ( flaring up of acne prior to menses)

Lab finding :

total serum testosterone: 150-200ng/ Dl

LH:FSH = 2:1

HAIR-AN SYNDROME

H: Hyperandrogenism

A: ACNE

IR: Insulin resistance

AN:Acanthosis nigricans

• Other clinical features : Increased libido , large clitoris , deep voice , infertility , irregular menstruation

• These patients have an increased risk of hypertension and type 2 DM. have to be monitored regularly

SAHA SYNDROME

S : Sebhorrea

A : Acne

H : Hirsturism

A : Androgenic Alopecia

Other causes of sahasyndrome

(1) idiopathic,

(2) ovarian,

(3) adrenal, and

(4) hyperprolactinemia

Other clinical features :

cystic mastitis, infertility and obesity

Treatment of acne associated with PCOS

4)FOLLICULAR OCCLUSION TRIAD

• ACNE CONGLOBATA

• HIDRADENITIS SUPPURATIVA

• DISSECTING FOLLICULITIS OF SCALP

PATHOGENESIS

FOLLICULAR HYPERKERATOSIS

SECONDARY BACTERIA

SUPERINFECTIN

FOLLICULAR OCCLUSION

• QUITE OFTEN PRESENTS WITH A FOURTH COMPONENT PILONIDAL CYST ( OCCLUSION TETRAD)

• TREATMENT : a)High dose systemic antibiotics (normally used in acne )

b)Zinc sulphate 135mg/twice daily

c)Oral isotretinoin

d)Oral , topical and systemic steroids( controversial )

e)Wide spread surgical excission with grafting

5)Acne Fulminans

Definition : most severe form of nodular acne Associated with systemic symptoms

Pathogenesis

Elevated levels of testosterone

Increased production of sebum and proliferation of p.acnes

Triggers immunological reaction

Clinical features

• Sudden appearance of massive inflammatory tender oozing friable plaque with haemorrhagic crustSites: predominantly back and chest

Other features : fever prior to lesion , leukocytosis(10,000 – 30,000 / cubic millimetre)Polyarthralgia , myalgia , hepatospleenomegaly , anaemia and Bone pain

Erythema nodosum present occasionally

Severe forms of acne

• ACNE CONGLOBATA

1)Onset : less explosive

2)Polymorphic

3)Face is commonly involved

4)No systemic complaints

• ACNE FULMINANS

1)Onset : more explosive

2)Mostly monomorphic

3)Face and neck usually spared

4)Systemic complaints are usually present

ACNE CONGLOBATA VS ACNE FULMINANS

Treatment of acne fulminans

• To start of –systemic glucocorticoids, systemic antibiotics and intralesional steroids

• Oral isotretinoin to be added once inflammation is controlled (to avoid excessive initial flare)

• Dapsone to be used concomitantly with isotretinoin in cases or erythema nodosum

6)ACNE EXCORIEE

• Commonly called ‘PICKERS ACNE’

• Seen as a subset of neurotic excoriations

• Usually seen in adolescent girls with an underlying personal or psychological problem

•Causes a) Caused in girls with a fragile self-image in which any imperfection perceived, real or not, is blown out of proportion

b) Finding satisfaction, as if they are picking away at their troubles or their emotional pain

Differentiated from acne vulgaris by the presence of post inflammatory hyperpigmented spots on where acne eruptions usually occur

*disease can emerge into dermatitis artefacta

• TreatmentTopical treatment usually not helpful, tendency to irritate the skin

Olanzapine 2.5-5mg daily (D.O.C)

Trifluperazine hydrochloride(5-30mg/day)

Pimozide (2mg twice daily)

*ALONG WITH PSYCHOTHERAPY

7)Apert’s Syndrome• Definition – Type 1

acrocephalosyndactyly,charecterised by craniosynstosis and early epiphyseal closure

• Pathogenesis : Genetic defect leading to amino acid substitution in exon 7 of chromosome 10

Clinical Featuresa) Facial : Flattened

Occiput , Proptosis, Prognathism , Parrot Beak Nose

b) Extremities : Fused Shortened Extremities

c) Respiratory: Cleft Palate And Bifid Uvula

ACNEACNE : usual sites face and trunk along with unusual sites forearm and buttock .

Pustular type of acne

CAUSE : Abnormal sensitivity of androgen receptors rather than excess circulating androgen

Treatment of acne in Apert’sSyndrome

• Acne Responds Well To Topical Medications And Oral Antibiotics

• In Severe Cases Oral Isotretinoin

TAKE HOME MESSAGE

• ALTHOUGH 99.9% OF THE CASES OF ACNE ARE ROUTINE EVERY CASE NEEDS TO BE LOOKED AT DIFFERENTLY , AS ACNE MAY BE THE ONLY OVERLYING SYMPTOM OF A SERIOUS UNDERLYING DISORDER

• Watch for other signs like sebhorrea , hirsturism , artheritis and irregular menses , also along with the regular sites uncommon sites should also be looked into which are equally important