TABLE 1: DIFFERENTIAL DIAGNOSIS OF THE RETICULATED PIGMENTARY DISORDERS

 

 

 

Dowling Degos Disease (DDD)

Galli-Galli Disease*

(GGD)

Reticulated

Acropigmentation

of  Kitamura (RAPK)

Dyschromatosis

Symmetrica

Hereditaria **  (DSH)

Dyschromatosis

Universalis

Hereditaria (DUH)

Pigmentary

morphology

 

Brown-black macules

Same as DDD; but some lesions may be palpable

Brown-black macules but some may be atrophic and depressed

Intermingled hyper- and hypo-pigmented macules

Intermingled hyper- and hypopigmented macules

Distribution

Flexural: axillae, neck,

antecubital & popliteal fossae

Same as DDD

Dorsal hands and feet; may slowly become generalized in adult life

Dorsal hands and feet; occasionally on the face

Widely disseminated on trunk, limbs and face

Histology

Elongation and branching of rete ridges; lower third of rete tips are darkly pigmented; no increase in melanocytes

Same as DDD; but  acantho-

lysis is present

Epidermal atrophy; little or no elongation of the rete ridges; increased basal layer pigmentation but no increase in number of melanocytes

Increased pigment in basal layer of hyper-pigmented spots; decreased pigment and sometimes decreased melanocytes in basal layer of hypopigmented spots

Similar to DSH but pigment incontinence may be present in the hyperpigmented macules

Other abnormalities

that may be associated

Acne-like facial pits; prominent comedones; follicular papules; hidradenitis suppurativa

Likely similar to DDD but not well documented in the few cases of GGD so far reported

Small pits and a broken rete ridge pattern on palms, soles and digits; pits may be present on the dorsal surface of digits

None definitively documented

None definitively documented

Gene Defect

Keratin 5

Keratin 5

Unknown

ADAR1***

KITLG****

 

*  Galli-Galli disease and DDD are variants of the same disease.

**  Synonym: reticulated acropigmentation of Dohi

***  ADAR1,  gene for adenosine deaminase acting on RNA 1; also known as DSRAD

****  KITLG, c-Kit ligand