The document discusses several acne related syndromes: 1) SAPHO syndrome presents with sudden onset haemorrhagic acne on the face and chest along with palmoplantar pustulosis and pain over the chest wall. Its etiology is unknown but may involve an autoimmune reaction. 2) PAPA syndrome involves pyogenic artheritis, pyoderma gangrenosum, and acne conglobata. It is an autoinflammatory disorder caused by a gene mutation leading to increased interleukin-1beta. 3) Endocrine acne is associated with conditions like PCOS, Cushing's disease, and congenital adrenal hyperplasia which alter hormone levels.

1. Acne Related
Syndromes
-Dr.Gurjot Singh Marwah
Junior resident
(Dept. Of Dermatology)
M.G.M hospital

3. 1)SAPHO SYNDROME
• S : SYNOVITIS
• A : ACNE
• P :PUSTULOSIS
• H : HYPEROSTOSIS
• O : OSTEITIS

4. CLINICAL
PRESENTATION
Sudden onset
haemorrhagic acne on face
and chest ,palmoplantar
pustulosis and pain over
chest wall
ETIOPATHOGENSIS
Unknow, it is
postulated to be an
autoimmune reaction
to some skin or
bacterial antigen

5. • SKIN LESIONS : Palmoplantar Pustulosis
Plaque Psoriasis
Pustular Psoriasis
Acne Conglobata
Acne Fulminans
Hidradenitis Suppurativa

6. 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)

7. TREATMENT
• Mainly Towards The Symptoms Involved
• NSAID’s ,Sulfasalazine
• Biologicals: Infliximab ( Few Case Reports)

8. 2)PAPA Syndrome
• PA : PYOGENIC ARTHERITIS
• P : PYODERMA GANGRENOSUM
• A: ACNE ( CONGLOBATA TYPE )

9. • 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

10. TREATMENT : Reports of successful treatment with
infliximab and anakinra

11. 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

12. Pathogenesis
of pcos
1) 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

13. 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

14. HAIR-AN SYNDROME
H: Hyperandrogenism
A: ACNE
IR: Insulin resistance
AN:Acanthosis nigricans

15. • 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

16. SAHA SYNDROME
S : Sebhorrea
A : Acne
H : Hirsturism
A : Androgenic Alopecia

17. Other causes of saha
syndrome
(1) idiopathic,
(2) ovarian,
(3) adrenal, and
(4) hyperprolactinemia
Other clinical features :
cystic mastitis,
infertility and obesity

18. Treatment of acne associated with
PCOS

19. 4)FOLLICULAR OCCLUSION TRIAD
• ACNE CONGLOBATA
• HIDRADENITIS SUPPURATIVA
• DISSECTING FOLLICULITIS OF SCALP

21. PATHOGENESIS
FOLLICULAR
HYPERKERATOSIS
SECONDARY
BACTERIA
SUPERINFECTIN
FOLLICULAR
OCCLUSION

22. • 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

23. 5)Acne Fulminans
Definition :
most severe
form of
nodular acne
Associated
with systemic
symptoms

24. Pathogenesis
Elevated levels of testosterone
Increased production of sebum and
proliferation of p.acnes
Triggers immunological reaction

25. Clinical features
• Sudden appearance of massive inflammatory
tender oozing friable plaque with haemorrhagic
crust
Sites: 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

26. 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

27. ACNE CONGLOBATA VS ACNE FULMINANS

28. 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

29. 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

30. • 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

31. • Treatment
Topical 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

32. 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

33. Clinical Features
a) Facial : Flattened
Occiput , Proptosis,
Prognathism , Parrot
Beak Nose
b) Extremities : Fused
Shortened
Extremities
c) Respiratory: Cleft
Palate And Bifid Uvula

34. ACNE
ACNE : 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

35. Treatment of acne in Apert’s
Syndrome
• Acne Responds Well To Topical Medications
And Oral Antibiotics
• In Severe Cases Oral Isotretinoin

36. 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